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928 Cards in this Set
- Front
- Back
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Generalized seizures
Define, types |
Whole brain is electrically irritable
Convulsive (gran mal), tonic, atonic, absence |
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Mutations and generalized seizure disorders
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Ion channels
K+ channel implicated in benign neonatal familial convulsions |
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Acquired generalized epilepsy
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Head trauma
Birth anoxia Other diffuse brain injuries |
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Focal seizures
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Part of the brain is hyperexcitable
Seizure activity starts there but can become secondarily generalized |
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Focal epilepsy causes
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Focal brain lesions
Can be disorganized or grossly normal appearing |
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intraaxial
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In the brain parenchyma
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Intraaxial lesions on MRI
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Expand brain/gyrus
Displace subarachnoid veins laterally Decreased CSF space overlying Vasoactive edema |
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Extraaxial lesions on MRi
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Compression of brain
Medial displacement of subarachnoid veins |
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Acute vs Old stroke on MRI
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Acute -- sulci crowded, midline shift, ventricle smaller
Chronic- sulci wider, midline normal, ventricle larger |
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cytotoxic vs vasogenic edema
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Caused by cortical cell death vs caused by leaky capillaries
Cytoxic -- lose gray/white difference Vasogenic -- enhance gray white difference |
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Etiologies of cytotoxic edema
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Stroke!
could be encephalitis |
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Etiologies of vasogenic edema
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Tumor, inflammation, hypertension
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How is the diagnosis of stroke made?
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Clinically
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What diagnostic test is necessary in an acute stroke? why?
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Noncontrast Head CT
Helps make treatment decisions/rule out hemorrhage |
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CT signs of a stroke
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Can be none for 6-24 hours
Dense MCA Cytotoxic edema (loss of gray white, loss of insular ribbon, effacement of sulci) |
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Ischemic Penumbra
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Neurons that have ceased to function because of low blood flow (20 ml/min/100g) but are not dead yet
Over time this will cause infarct This is the tissue that stroke treatment is trying to save |
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Stroke treatment
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tPA if w/in 4.5 hours
unless contraindication (hemorrhage) |
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Diffusion imaging in stroke
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Gold standard for IDing dead brain tissue
Bright show restricted diffusion Becomes positive in minutes stays positive for two weeks Near 100% sensitivity, false positives with abscess, stroke, infarct |
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Epidural hematoma
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Arterial bleed peels dura off bone
Biconvex Coup to a site of injury (usually fracture) Obeys suture lines Often a disruption of the middle meningeal artery Good prognosis with interventin |
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Managing epidural hematoma
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> 30 ccs, surgery
< 30 ccs with little displacement, good glascow coma score, may be managed nonoperatively If acute epidural hematoma, with reduced glascow score, and aniscoria, evacuate ASAP |
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Subdural hematoma
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Venous blood in between arachnoid and dura
Crescent shaped From the tearing of bridging vein, often counter coup, but can also be coup Worse prognosis than epidural |
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Who gets subdural hematomas
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Young/old
more room for the brain to slosh |
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Which subdurals need to be evacuated?
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> 10 mm thickness
> 5 mm shift with dropping glascow score, increased intracranial pressure, fixation of pupils |
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Midline shift assessment
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Conducted at level of the foramen of Monro
Total/2 - one side from midline >5mm is time for surgery |
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Subarachnoid hemorrhage
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Tearing of veins in subarachoid space
Trauma is most common cause -may worsen prognosis in head trauma >5mm is worse No specific treatment |
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Contusions
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Hemorrhaggic or not
often get bigger over several days May need to be drained if hematoma forms Coup or countercoup |
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Most likely sites of brain contusion
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Anterior/inferior frontal
Anterior temporal |
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Diffuse axonal injury
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shear injury results in axonal death
CT normal early but patient often comatose Gray-white jnc, corpus callosum, dorsolateral midbrain |
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CT or MRI in the ER?
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CT first -- shows surgically important lesions
MRI if there are continued unexplained deficits --more sensitive |
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Who needs a CT scan with loss of consciousness or post traumatic amnesia?
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Headache
Vomitting >60 years short term memory deficits evidence of trauma to neck or head decrease in GCS focal deficit coagulopathy |
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Who should get a CT scan after head injury w/o loss of consciousness or amnesia
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Severe headache
Vomiting >65 years Physical evidence of basilar skull fracture Decreased GCS Dangerous manner of injury |
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Dementia
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Deterioration of intellectual/cognitive abilities without altered altertness or perception
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Amnesia
Antero? Retro? |
Loss of memory without any other deficits
Antero-after injury Retro - before injury |
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When to focus on cognitive testing
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Brain lesion
Psychiatric disease Behavior complaints |
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Aphasia/dysphasia
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Language impairment
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Dysprosodia
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impairment in non verbal communication
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Agnosia
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Inability to understand sensory input despite intake sensation
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Apraxia
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Inability to perform learned tasks despite intake motor functions
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Somnolent
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Mostly awake with a tendency to drift towards sleep
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Stuporous
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Mostly asleep but able to maintain alertness for short periods of time
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Paraphasia
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production of well articulated but incorrect words
Semantic -- substitute wrong word Phonemic -- substitute wrong syllable Neologism -- non-existant word |
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Brain regions important for communication
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Dominant
Broca's - frontal lobe anterior to the motor strip --production Wernicke's - superior temporal gyrus --understanding Arcuate fasiculus - white matter deep to the anterior parietal lobe connecting the two Homologous non-dominant side -- inflection and nonverbal communication |
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Broca's aphasia is often seen with?
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Right hemiparesis
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Aphemia
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'cortical dumbness'
extreme broca's with complete inability to speak |
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Wernicke's is often seen with?
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Right superior quadrantanopsia
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Transcortical aphasias
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Sensory/motor/global
with intact repetition |
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Cortical dumbness/word dumbness
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More extreme Broca/Weirnicke
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Subcortical aphasias that end to mimic Wernicke's
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Head of the caudate -poor comprehension
Thalamic - logorrheic, good repetition |
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Types of long term memory
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Declarative
Episodic -- memory of events, medial temporal Semantic -- knowledge of concepts - cortical association areas Non-declarative Procedural --Basal ganglia Conditioning/priming -- amygdala |
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Where is working memory located?
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Frontoparietal
Several seconds of storage |
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Deficits in calculation from lesion where?
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Angular or marginal gyri
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Visual agnosia lesion
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Bilateral parieto-occipital
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Propagnosia
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Inability to recognize faces/differentiate things in a class
Typically a bilateral occipital lesion |
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Alexia
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Inability to read
Often associated with right hemianopsia and agraphia Lesion to left occipital |
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Alexia without agraphia
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Inability to read with intact writing
Lesion usually in left anterior occipital (splenium of the corpus callosum) must detach both visual cortices from wernicke's |
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Agraphesthesia
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Inability to recognize numbers traced on hand
Lesion in nondominant parietal |
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Asomatognosia
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Inability to recognize own body part
Lesion in nondominanet parietal |
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Auditory agnosia
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Inability to recognize sounds despite intact hearing
Lesion in contralateral temporal |
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Neglect
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Underusing or under-recognizing one side
Visual -- ignore what you see Motor -- don't use that side Sensory -- ignore afferent information Lesion in nondominant parietal, if includes visual usually involves the occipital region also |
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Agraphia lesion
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posterior frontal cortex
Also temporal-parietal parietal This is an apraxia |
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Gerstmann syndrome
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Agraphia
Acalculia Finger agnosia L/R disorientation Lesion to dominant angular gyrus and corpus callosum |
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Neglect can also be caused by a lesion to
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ventral thalamus
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Constructional apraxia
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Not a true apraxia
Damage to the nondominant parietal lobe gives visual interpretation defect |
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Ideomotor apraxia
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Cannot perform a task if directed
Can perform task if not prompted Not localizing, although often involving a disconnection, often seen in dementia |
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Ideational apraxia
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Inability to perform a task that has several steps
Not localizing, often seen in dementia |
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Dressing apraxia
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Not a true apraxia
Really a visuospatial processing defect Nondominant parietal |
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Gait apraxia
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Not a true apraxia because gait is instinctual not learned
Bilateral frontal lesion, widened base with heavy, magnetic steps |
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Orbitofrontal lobe lesion results in
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Social inappropriateness
Disinhibition Euphoria Abnormal sexual behavior |
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Medial frontal lobe lesion results in
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Apathy
Akinesis Abulia |
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Dorsolateral frontal lobe lesion results in
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Irritability
Inflexibility Reduced sexual interest Loss of executive functioning |
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Frontal release reflexes
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Rooting
Sucking Snout Grasp Palmomental |
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Causes of loss of olfaction
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Mechanical -- septal deviation, occipital trauma, olfactory grooven neoplasia
Infection-- common cold, hsv encephalitis Metabolic -- DM, B12/vit A/zinc deficiency |
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Central vision losses
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Refractive losses (try pinhole to rule this out)
Optic atrophy Macular lesions |
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What characterizes diplopia in patient with extraocular muscle weakness?
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Object is always further off to the side on the weak side
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Horner's syndrome
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Sympathetic deficit
Miosis Slight ptosis Sight elevation of lower eyelid Ipsilateral decreased sweating |
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Pupillary light reflex
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Fully bilateral from retina to brain
Afferent - CNII Efferent - CNIII parasympathetics |
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Weakness in a pterygoid muscle is reflected as?
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Protusion toward weak side on jaw protrusion
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5 functions of the CNVII
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Facial movement
Taste Sensation of auditory canal Stapedius Lacrimaton |
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Facial nerve lesion
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Total facial weakness
Loss of taste (sugar water test) Hyperacusis Some depression of tearing |
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Lesion to corticobulbar fibers innervating the facial nucleus
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Lower facial weakness only
Extensive damage may give hyperimia -- excessive expression of emotions in face |
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What to rule out with progressive unilateral deafness in adult?
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VIII tumor at cerabellopotine angle
(acoustic neuroma) |
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Use of caloric testing
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Localize labyrinthine abnormality
Assess brainstem function in a comatose patient |
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When do you use oculocephalic testing?
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Comatose patient
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Postural testing or Hall Pike or Nylen-Barany maneuver utility
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Diagnosing benign positional vertigo
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Curtain sign
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Palate deviates away from weak CNX side
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Nasal speech indicates what cranial nerve weakness?
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Palate weakness -CNX
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Hoarseness indicates what cranial nerve weakness?
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Laryngeal weakness - CNX
Could also be inflammation, mass |
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Carotid sinus reflex
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CNIX afferent, CNX efferent
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Spinal accessory nerve damage
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Nucleus -- commonly damaged in strokes
Nerve -- most common site of damage is passage through jugular foramen |
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Hypoglossal lesions
Corticobulbar? Nucleus nerve? Early signs? |
Upper - little deficit, bilateral
Nucleus/nerve -- dysarthric, protruding tongue to side of weakness Fasiculations are early signs |
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Vertigo definition
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Sense of movement of self or objects around self
Generally accompanied by nausea, dysphoria, malaise |
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Vertigo mechanism
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Vestibular system dysfunction
Inappropriate vestibular input to cortex = false perception of head rotation |
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"Dizzy" possibilities
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Vertigo
Dysequillibrium (unsteadiness) Lightheadness Visual dysfunction Disorientation |
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Dysequillibrium causes
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Proprioceptive deficits
B12 deficiency Peripheral neuropathy Cerebellar deficits |
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Lightheadness cause
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Lack of bloodflow to brain
cardiac arrythmias, hypotension (orthostatic, medication induced) |
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Path of vestibular sensation
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Hair cell - bipolar neuron with nucleus in vestibular (scarpa's) ganglion - vestibular nerve -> acoustic nerve
through internal acoustic meatus, traverse cerebellopontine angle, enter brain at pontomedullar jnc -- 4 vestibular nuclei |
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Organs of sensation of angular and linear acceleration
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Angular - ampullae of semicircular canals
Linear - utricle and saccule |
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Vestibular nuclei connect with
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Cerebellar systems
Cortex 3,4,6 |
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Lesions in vertigo
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Labyrinth
CNVIII Vestibular nuceli Cerebellum Cortical centers (temporal) |
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Nausea in vertigo, why?
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Sensory conflict?
--also cause diaphoresis, lightheadness Abberant input to nausea cener |
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Oscillopsia
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Jumping vision
Due to nystagmus Often absent |
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Other symptoms associated with vertigo?
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Hearing loss
Tinnitus |
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Benign position vertigo
Define Treat |
Recurrent (usually brief) episodes of vertigo positionally induced
Sometimes after trauma, bed rest, prolonged time in an odd position From canaliths (calcified granules) stimulating hair cells. Repeated head maneuvers can treat |
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Medications that can cause vertigo
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Aspirin
Aminoglycosides Phenytoin NSAIDs OTC cold medications |
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Vestibular neuronitis/Vestibular labyrinthitis
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Acute onset of severe vertigo
Lasting 48-72 hours Steroid can help sometimes Inflammation of vestibular nerve or labyrinth, unclear etiology |
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Bacterial otitis symptoms
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Ear pain
Vertigo Fever Hearing loss Systemic illness |
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Meniere's disease
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Recurrent attacks of vertigo, n/v, fluctuating hearing loss, tinnitus
Sensation of fullness in ear, sudden falls Caused by too much endolymph |
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Meniere's disease treatment
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Salt restriction, diuretics, weight loss
Extreme -- endolymph shunting, labyrinthectomy, vestibular neurectomy |
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Post traumatic vertigo
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Can lag after injury
A lot like BPH ? otolithiasis |
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Psychogenic/phobic vertigo
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Usually situational
May experience vertigo or vertigo-like without nystagmus May be very debilitating May follow a different vestibular insult Treatment must include therapy, may also use SSRIs, benzos, etc |
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Perilymphatic fistula
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Trauma induced fistula between labyrinth and outside world (usually through round or oval windows
Sudden onset vertigo, hearing loss, n/v Often after sneeze Valsalve can worsen Surgery sometimes helps |
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Weird thing about Meniere's hearing loss?
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Low frequencies go first
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Ischemic causes of vertigo
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Vertebral artery
Basilar PICA AICA Internal auditory artery (branch of AICA) |
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Infectious causes of vertigo
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meningitis
otitis media syph sarcoid -- this is not really an infection |
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Wallenberg's syndrome
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PICA blockage causing ischemia results in:
Vertigo, ipsilateral ataxia, dysarthria, ptosis, miosis |
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Ramsay Hunt syndrome
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Herpes Zoster oticus
vertigo, hearing loss, bell's palsy, ear pain Vesicles on tympanic membrane, in ear Can track in brainstem --encephalitis Treat with acyclovir |
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Cerebellopontine angle (CPA) tumor symptoms
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Vertigo
Hearing loss Facial paralysis Masses compressing CNVIII |
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Migraine and vertigo
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Vertigo can occur without headpain in patients with history of migraine
Can be treated with TCAs, triptans, beta blockers, calcium channel blockers, valproate |
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Nystagmus and timing to localize lesion in vertiginous patient
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Vertical, non fatiguing, non latent -- central problem
down - craniocervical jnc up - pontomedullary jnc Pure horizontal or rotary -- peripheral usually Pendulous -- not usually vestibular, associated with oscillopsia |
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Rapid head turning diagnostic in vertiginous patient
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Normal patient will be able to fixate on target with rapid head turn
With vestibular dysfunction, there will be cortical jumps to catch up |
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Dix-Hallpike manuever
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Lay down with head turned and hanging further down
Usually ear down when vertigo is provoked is the injured side (patients also tend to fall toward this side, and fast nystagmus beat is away from this side) |
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Treatment of vertigo
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Epley canalith repositioning for BPV
Anticholinergic (scopalamine) Antihistamine (meclizine) Neurleptic (promethazine) Benzodiazapine |
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Typical description of vertigo
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Spinning
Tilting Listing Rolling Falling |
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Wakefullness in brain
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Reticular system of rostral brainstem
and thalamic/forebrain projections |
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Awareness in brain
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Thamocortical
Cortiocortical Precuneus is an important area Wakefullness is needed for awareness |
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Obtundation
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Impairment of consciousness, not indicative of death
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Coma
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No wakefullness, nor awareness
Not arousable even with vigorous stimuli |
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Stupr
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Not awake, not aware
Arousable for brief periods |
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Vegetative state
how do you get this? |
Wakefullness without awareness
Global injury to brain may affect just the more metabolically active, phylogenetically younger cortex/thalamus involved in awareness rather than the older, less demanding reticular system of wakefullness |
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Types of injury that can result in coma
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Structural: Trauma, edema, inflammation, ischemia
Metabolic: toxic metabolic encephalopathy (effects all neurons) |
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Herniations
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Structural pertubations cause lateral or caudal displacement of brain because of increased pressure
Central tentorial Uncal Falx cerebri Foramen magnum |
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Uncal herniation
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Most common with rapidly expanding lateral mass
Ipsi occulomotor nucleus damaged |
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How does acute meningitis produce coma?
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Vascular and inflammatory changes
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Metabolic encephalopaties
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Alterned conditions
Altered blood flow, oxygen, glucose, meningitis, seizures, organ failure |
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Toxic encephalopaties
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Endogenous or exogenous poisoning
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Severity of metabolic/toxic enephalopathy can be determined by
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Rapidity of onset
Slow onset may on give stupor while rapid can lead to seizures and coma |
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Major causes of stupor and coma
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TBI
CVA Mass lesion Infections Seizures Metabolic/toxic encephalopathies |
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What Comas Assessment do you use?
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TBI - glascow
FOUR score - everything else |
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Stimuli to test awareness/arousal
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Shout patients name
As to move eyes up and down (test locked in syndrome) Nose tickle Sternal rub |
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Initial coma work up
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Stabilize vital signs
Immediate lumbar puncture in no localizing signs or nuchal rigidity Emergent CT |
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Post hyperventilation apnea
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Often early sign
"upper diencephalon" |
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Cheyne Stokes
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Hyperpnia punctuated by apnea
"lower diencephalon" Often seen with metabolic encephalopaties, like cardiac failure |
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Central neurogenic hyperventilation
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Continuous hyperpnea and tachypenia
Results in a respiratory alkalosis Damage to rostral brainstem tegement at midbrain |
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Apneustic breathing
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Prolonged inspiratory pause
Pontine lesion or pons-medulla |
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Ataxic breathing
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Irregular, shallow breathing
Medullary lesion |
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Pupils remain reactive until near the end of disease?
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Metabolic cause
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Pupil dilated and fixed?
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Uncal herniation
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Pupil midposition, fixed?
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Midbrain lesion
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Pinpoint pupils?
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Pontine lesion with preservation of midbrain
Pontine hemorrhage, infarction |
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Conjugate eye deviation, where is the lesion?
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Cortical lesions -- eyes go towards the lesion
Brainstem lesion -- eyes go away from lesion |
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Ocular skew deviation, where is the lesion?
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One eye is slightly higher than the other
Midbrain or pons |
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Ocular bobbing, where is the lesion
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Pons
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VOR in coma
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Lesion at diencephalic level -- only tonic (slow) part, never jumps back
Lesion in brainstem - no response |
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Decorticate posturing to noxious stimulus
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Arm flexed and leg extended
Midbrain lesion |
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Decerebrate posturing to noxious stimulus
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Arm and leg extended (though wrist flexed)
Pontine lesion |
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No response to noxious stimulus
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Lesion at level of medulla or spinal cord level
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Early treatments for pt in coma
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Stabilize breathing, circulation
Prevent vitamin deficiency (thiamine) Give glucose Antibiotics if indicated Block opiods and benzos (naloxone, flumazenil) Hyperventilate/mannitol in ICP |
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Early third nerve lesion
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Drowsy
Pupil dilated and sluggish May have dysconjugate VOR Babinski |
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Late third nerve presentation
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Sustained hyperventilation
Dilated unreactive pupil Ipsilateral eye does not move medially on VOR testing, contralateral does move laterally Decordicate or decerebrate posture |
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Prognosis in coma after cardiac event
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Negatives
Day1 : myclonic status epilepticus Day1-3: biltateral absence of n2o response, increased serum enolase Day3: absent pupillary responses, exstensor or absent motor responses |
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Coma course
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Usually only a few weeks
Die, wake up, or go into persistent vegetative state |
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Most common etiologies of vegetative state
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Hypoxia (from cardiac event)
Diffuse axonal injury (torque injury) |
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Prognosis in chronic vegetative state
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Non-traumatic, less than 1% have any recovery after 3 months
Traumatic, can't really call until a year |
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Minimally conscious state
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Profound lack of responsiveness with intermittent evidence of awareness
More common and sometimes mistaken for vegetative state Somewhat more responsive to stimulants, levadopa than vegetative state |
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Locked-in syndrome
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Profound paralysis without a deficit in consciousness
Large infarct/hemorrhage at pontine tegmentum -- quadripelgia, pseudobulbar palsy, paralysis of horizontal eyemovements Only left with vertical eye and eyelid movements |
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Locked-in syndrome prognosis
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Most patient die in months
Some survive for years A few recover Communicate with eyes and software |
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Important prenatal factors
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Maternal age, medical condition, medications, drugs
Preterm labor, GDM, HTN, group B strep, bleeding, prior pregnancy losses, HIV, herpes, etc infections Birth trauma, perinatal problems |
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Neurologic development at birth check
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Tracks objects 45 degrees
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Developmental milestones
3-4 months |
Tracks fully
Minimal head lag Bears weight Coos |
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Developmental milestones
5-8 months |
Rolls
Sits when placed Peek-a-boo Babbles |
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Developmental milestones
9-11 months |
Pulls to stand
Cruises Pincer grab Babbles +/- words Stranger anxiety |
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Developmental milestones
11-18 months |
walks
3-15 words |
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Developmental milestones
18 months |
Points to body parts
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Developmental milestones
24 months |
2-3 word phrases
scribbles |
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Developmental milestones
3 years |
draw circle
gender identify |
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Developmental milestones
4 years |
count to 5
tell stories copy cross and square |
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Developmental milestones
5 years |
counts to 10+
knows colors copies triangle may write name |
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Developmental milestones
6 years |
Read grey scale
write alphabet do simple sums |
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When do pupils become reactive to light
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30 weeks gestation
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Optic nerve pallor on fundoscopic exam
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Chronic pressure
Demylination |
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Infantile spinal musclular dystropy pathognomonic sign
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fasiculations in lateral tongue
looks like "worms in a bag" |
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Ankle clonus in newborn?
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A couple of beats is ok
Sustained or asymmetric is not |
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Babinski in newborn?
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Normal until 18 months
Asymmetry is not |
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Moro reflex
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Startle reflex
Arm extension, finger flaring, eyes open followed by Arm adduction, finger grasping, leg flexion Disappears at 4 months |
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Persistance of newborn reflexes
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Indication of upper motor neuron lesion
Often first indicator of cerebreal palsy |
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Fencer's posture reflex
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Ipsi extension
Contra flexion with head turn 6 weeks - 6 months |
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Muscle tone definition
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Resisance to passive movement
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Hypertension in mother is a risk of?
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Ischemia in fetus
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Recurrent miscarriages can be a symptom of
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mitochondrial disease
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Maternal clotting disorder history indicates which risk in neonate?
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Inherited clotting disorders increase risk of stroke
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Hyperbilirubinemia leads to a risk of
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Hearing loss
cerebral palsy in extreme cases |
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Inherited myotonic dystrophy in fetus
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Much worse if mother has myotonia because of womb environment processes
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Cafe au lait spots
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Neurofirbomatosis
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Ash leaf spots
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Tuberous sclerosis
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Port wine stain
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Sturge Weber
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|
|
Motor strength grading
|
0 - no contraction
1 - contraction but no movement 2- movement but not against gravity 3 - movement against gravity as max 4 - movement against gravity + 5 - normal |
|
|
Pronator drift
|
With diffuse upper limb weakness
Outstretched arms w/ palms up and eyes closed Weak arm drifts down and pronates Do not usually see just drift down with organic disease |
|
|
Break away or collapsing weakness
|
Good initial buildup of strength and then suddenly letting go
Not a neurologic problem Can be pain, fake |
|
|
Upper motor neuron damage
|
Damage of corticospinal tract somewhere between cortex and spinal cord
Increased reflexes, spasticity |
|
|
Lower motor neuron damage
|
Damage to anterior horn cells or their axons
Decreased reflexes, decreased tone, lots of atrophy |
|
|
dysdiadochokinesia
|
difficulty with rapid alternating movements
suggestive of ipsi cerebellar injury |
|
|
Undershoot or overshoot on voluntary movement
|
Intention tremor
|
|
|
Over rebounding or not correcting after excursion from fixed position
|
Cerebellar injury, ipsi
|
|
|
Rapid tremor ( 7 cps) causes
|
Too much sympathetic activity
Anxiety Pheochromocytoma Thyrotoxicosis |
|
|
Tremor of Parkinsonism
|
Slow
Primarily at rest and decreased with movement Asymmetric |
|
|
Essential tremor
|
Increased with sustain postures
Worsen with intentional movement Often affect eating/writing Worsen with anxiety |
|
|
Cerebellar tremor
|
Pronounced tremor during voluntary movements
|
|
|
Fasiculations
|
Random contractions of motor units that do not result in joint movement
Lower motor neruons are damaged/irritated |
|
|
Chorea
|
Random, non sterotyped movements, rapid and fleeting
Can be briefly suppressed consciously |
|
|
Tic
|
Stereotyped, repetitive movements
Can affect voice |
|
|
Athetosis
|
slow, writhing, snake-like movements in one or more body parts
|
|
|
Dystonia
|
Sustained twisting of a body part
Neck - torticollis |
|
|
Hemibalism
|
Flinging of one side of the body
|
|
|
Movement symptoms that are basal ganglia related
|
Hemibalism
Athetosis Chorea |
|
|
Swaying from station with eyes open
|
Vestibular or cerebellar
Vestibular is usually to one side Cerebellar (esp midline lesions like alcohol) is to either side |
|
|
Circumducting gait
|
Swinging stiff leg around
Upper motor neuron damage |
|
|
Reeling or staggering gait
|
Cerebellar dysfunction
|
|
|
Falling to ground especially with eyes closed
|
Vertigo
|
|
|
High stepping gait
|
Foot drop
Fibular nerve palsy |
|
|
Parkinson's disease gait
|
Shuffling, difficulty with initiation
|
|
|
Glue-footed walk
|
Sliding rather than stepping
Bilateral frontal lobe degeneration or midline cerebellar damage If really bad, can result in falling backwards (retropulsive) |
|
|
Waddle walk
|
Hip girdle weakness
Hips shift toward weak side when leg off group |
|
|
Walk with dorsal column injury
|
Foot stamping
Need to look at the feet while walking |
|
|
Walk of spinal stenosis
|
"Simian"
Stooped posture |
|
|
Lateralization of cortical control of muscle
|
Distally - contralateral cortex
Axially -- bilaterally |
|
|
Do caudal or rostral upper motor neuron lesions have greater effect on spasticity/pathologic reflexes
|
Caudal -- tracts are more consolidated, hit more of them with a lesion
|
|
|
Evolution of a spinal injury
|
Acute transection leads to spinal shock
Flaccid paralysis - lasts longer the more caudal the lesion Spastic paralysis |
|
|
Lesion to cerebellar hemisphere
|
Ipsilateral intention tremor
Ataxic gait Hypotonia on passive testing |
|
|
Lesion to cerebellar vermis
|
Vestibular syndrome
Dysequilibrium, nystagmus Often seen with medulloblastoma, childhood tumor of the vermis |
|
|
Lesions to get decordicate/decerebrate posturing
|
Above red nucelus in brainstem -- decordicate
Between red nucleus and vestibular nucleus -- decerebrate |
|
|
Nerve roots that abduct shoulder
|
C5-6
|
|
|
Shoulder external rotation nerve root
|
C5
|
|
|
Elbow flexion nerve roots
|
C5-6
|
|
|
Elbox extension nerve roots
|
C7
|
|
|
Wrist flexion
|
C7-C8
|
|
|
Wrist extension
|
C7
|
|
|
Intrinsic hand muscles nerve roots
|
C8-T1
|
|
|
Hip flexion
|
L2-3
|
|
|
Hip extension
|
L4-5
|
|
|
Knee flexion
|
L5-S1
|
|
|
Knee extension
|
L3-4
|
|
|
Ankle plantar flexion
|
S1-S2
|
|
|
Ankle dorsiflexion
|
L4-5
|
|
|
Ankle inversion/eversion
|
L5-S1
|
|
|
Weakness from NMJ problem is characterized by
|
Fatiguibility
|
|
|
Crossed adduction on reflex testing
|
Sign of hyperreflexia
Opposite leg pulls in with reflex testing |
|
|
Cremaster reflex
|
Rapid brief elevation of testicle ipsi to medial thigh scratch
L1-L2 |
|
|
Abdominal reflex
|
Contraction of quadrant of muscle 2/2 scratch
|
|
|
Bulbocavernosus reflex
|
Anal contraction in response to glans penis squeezing or Foley manipulation
Absence used to evaluate presence of spinal shock in the short term or damage to conus medularis in long term S1-S3 |
|
|
Hoffman's reflex
|
Flick finger -- thumb flexion
Hyperreflexia |
|
|
Tromner's reflex
|
Tapping on voler part of finger results in fingers and thumb flexion
|
|
|
Jaw jerk test
|
Tap on open relaxed jaw
Jerk if there is a lesion in brain above brainstem |
|
|
Blepharospasm
|
Eyelid dystonia
Can be bad enough to render blind |
|
|
Myoclonus
|
Sudden activation of muscles
|
|
|
Asterixis
|
Sudden erratic loss of muscle tone
encephalopathy, toxins. |
|
|
Astasia
|
Inability to maintain station unassisted
|
|
|
Abasia
|
Lack of coordinator when walking
|
|
|
Instability of station with eyes closed only
|
Lesion of conscious proprioception
|
|
|
Hyperesthesia
|
Abnormally increased sensation
Usually pain related not nerve related |
|
|
Hyperalgesia/hyperpathia
|
Perception of innocuous stimuli as painful
Usually pain related and not nerve related |
|
|
Sensation vibration lost distally but preserved proximally
|
Suggests polyneuropathy
|
|
|
Loss of vibration is a lesion of
|
Larger diameter fibers in peripheral nerves
or dorsal columns or medial lemniscus |
|
|
Sensory loss over a large part of the body
|
CNS lesion
|
|
|
Sensory loss with a level
|
Spinal cord injury
|
|
|
"Suspended" sensory loss
|
Upper limb lesion with lower limb preservation
Suggests lesion in spinal cord like syringomyelia |
|
|
Joint position sense
|
Toe hold and move up and down
Deficiency could be from large fiber, DC-ML injuries |
|
|
Hysterical anesthesia
|
Reported loss of sensation
Usually sharply demarcated and not associated with a nerve pattern Usually do not loose vibratory sense |
|
|
Thalamic lesions presentation
|
Hemihypesthesia
Hyperpathia |
|
|
Brown Sequard syndrome
|
Hemisection of spinal cord
Ipsi loss of motor, touch, vibration Contra loss of pain, temp (several segments below lesion) |
|
|
Dorsal big toe is autononmous zone for
|
L5
|
|
|
Lateral heel autononmous zone for
|
S1
|
|
|
Below knee medially autononmous zone for
|
L4
|
|
|
Near thumb autononmous zone for
|
C6
|
|
|
Middle finger autononmous zone for
|
C7
|
|
|
Small digit autononmous zone for
|
C8
|
|
|
Sensation loss with damage to nerve root?
|
Usually none, they are completely overalapping
In distal limbs you can find some spots |
|
|
Epilepsy
Define Prevalence |
Habitual provoked recurrence of seizures
0.5% of population "electrically irritable brain" |
|
|
Acute seizure
Prevalence Antecedents |
9% of population will have seizure over lifetime
Injury, trauma, anoxia, fever |
|
|
Seizures on neuronal level
|
Repetitive firing
Excitatory post synaptic potential changes vastly more common than inhibitory, can result in rapid firing |
|
|
Epilepsy causes that present later
|
Brain tumor
Vascular disease Some hereditary |
|
|
Generalize epilepsy with convulsive seizures
|
Tonic-clonic seizure
Idiopathic or genetic Usually respond well if there is not other neurologic problem Typically begins in childhood or adolescence |
|
|
Tonic-clonic seizure symptoms
|
Loss of consciousness
Fall Muscle rigidity -- muscle jerking Respiratory depression Tongue biting common Bladder/bowel control loss common Usually 1-5 minutes Post ictal confusion |
|
|
Juvenile myoclonic epilepsy
|
Generalized seizure disorder
Convulsive seizures Diffusely abnormal EEG Prominent motor cortex involvement Often 2/2 mutated GABA receptor |
|
|
Absence seizures
|
Generalized epilepsy with non-convulsive seizures --childhood petit mal
Staring spells Brief loss of consciousness Subtle myoclonic movement -- simple automations No post ictal period |
|
|
Absence seizures course
|
Present in school age children
Usually good prognosis Although some kids develop convulsive seizures |
|
|
Atonic seizures
|
Sudden loss of muscle tone
--often association tonic EEG with diffuse flattening Problem with thalamic/cortex interaction Often seen in context of diffuse brain damage, poor progosis |
|
|
Focal epilepsy is caused by
|
Focal brain lesions
|
|
|
Complex partial seizure
|
Impaired consciousness
Last about a minute Blank stare Simple or reactive automatisms Ictal amnesia Focally abnormal EEG |
|
|
Relation of head trauma to seizures and epilepsy
|
Can cause seizures
Damage is cause of subsequent epilepsy Hemoglobin breakdown products are very epileptogenic |
|
|
Mesial temporal sclerosis
|
Common cause of epilepsy
Caused by trauma, infection, congenital malformation, prolonged febrile convulsion |
|
|
Auras and localizaiton
|
Gastric, olfactory, gustatory, deja vu - temporal
Auditory - lateral temporal Lateralized paresthesias - frontal, parietal Visual - occipital Vague -- frontal or generalize |
|
|
Nonepileptic seizurs
|
Psychogenic
Caused by unbearable stresses LOC and shaking |
|
|
Vagus nerve stimulator
|
Use on left
Can stop seizures if given at right time In humans, give 30 second/5 minutes 50% reduction in seizurs |
|
|
Best drugs in generalized epilepsy
|
Valproate
Lamotrigine (esp in women) |
|
|
Best drugs in focal
|
Carbmazepine
Lamotrigine |
|
|
Focal vs general
More common? More treatable |
Focal is more common (7:3)
And less treatable |
|
|
Cerebral stimulators in epilepsy
|
Probably work if you can get enough electrodes in
|
|
|
Epileptic surgery
|
Can help 50% of medically refractory patients
Mesial temporal sclerosis is common cause of surgically fixable epilepsy Can decrease surgery AND raise IQ No brain is better than bad brain |
|
|
Localizing epileptic focus for surgery
|
Perfusion imaging (hypo normally hyper ictally)
FDG imaging (normally hypo active) Intracranial EEG |
|
|
Corpus callosumectomies
|
Palliative
Stop secondary generalization of focal seizures |
|
|
CNS lymph system
|
CNS does not have lymphatics
CSF serves this purpose |
|
|
Nissl stain
|
Stains Nissl bodies -- collections of RER in neurons
|
|
|
Staining axons
|
Silver stains
IHC for neurofilaments |
|
|
IHC for GFAP
|
Stains glial intermediate filament
An astrocyte stain |
|
|
LFB - Luxol fast blue stain
|
Stain myelin blue
|
|
|
Ischemia/hypoxia of a neuron results in histologically
|
Red neurons
Hypereosinophilia and pyknosis of nuclei Severely damaged nuclei will eventually disappear |
|
|
Neuronal atrophy
|
Gradual shrinkage of cell body and dendritic tree
From disuse atrophy, neurodegenerative diseases, transsynaptic atrophy |
|
|
Chromolysis
|
Loss of Nissl staining
Sign of retrograde degeneration from axonal transection Also of some metabolic disturbances like pellagra |
|
|
Ferrugination
|
Deposition of Ca, Fe, minerals in damaged neurons
|
|
|
Neurophagia
|
Neurons eaten by microglia
|
|
|
Lipofuscin
|
Wear and tear pigment
not usually seen purkinje cells until later in life Early appearance of lipofuscin (really ceroid) seen in early neurons-- storage disease |
|
|
Lipid storage diseases appearance in brain
|
Block in catabolic pathway
Sphingolipids or gangliosides build up in perikaryon -- take globular shape |
|
|
Wallerian degeneration
|
Degeneration of axol distal to injury
Myelin will fragment if present |
|
|
Dying-back of axon
|
Degeration of most distal parts of axon because of inadequate axoplasmic flow or nutrients
Seen in some degenerative, toxic neuropathies |
|
|
Axonal spheriods
|
Bulbous swellings of axons
Usually represent sublethal injury |
|
|
Loss or abnormal dendrites
|
Often see in developmental disorders
Congenital or acquired |
|
|
What makes scars in the CNS
|
Astrocytes
Fibroblasts only get involved if there is abscess or breaking of the meninges |
|
|
Alzheimer's type 2 glial
|
Astrocyte reaction to high ammonia in blood CSF (as with hepatic enecephalopathy)
Clear karyoplasma and chromatin around nuclear envelop |
|
|
Progressive multifocal leukoencephalopathy
|
Viral infection that localizes to oligodendrocytes
Destruction of oligos results in demylination in the CNS |
|
|
Ependymal cells
|
Line ventricles
Maintain equilbrium between CSF and intrastitial fluids of brain |
|
|
Rod cell morphology of microglia
|
Indicative a chronic disease
Like CNS syphillis |
|
|
Resting microglia
|
Small cells with almost no visible cytoplams
Projections set up to make non-overlapping areas for each microglia |
|
|
Schwann cells fncs
|
Increase conduction speed
Contribute to NMJ Ingest tissue debris Promote regeneration |
|
|
CNS regions most vulnerable to anoxia
|
Pyramidal cells of hippocampus (CA1 esp)
Pyramidal cells of 3rd lamina of cortex Purkinje cells of cerebellum Mediated by energy requirements, suseptiblity to vascular compromise with swelling and glutamate toxicity (NMDA receptors) |
|
|
Glutamate toxicity in anoxia
|
Astrocytes cannot take of glutamate
Great increase in glutamate concentration NMDA receptors can be opened |
|
|
Methyl alcohol poisoning results in
|
Bilateral putamen hemorrhage
|
|
|
Minamata disease
|
Organic mercury poisoning
Granular cells of cerebellum are most in danger |
|
|
B1 deficiency cellular changes
|
Destruction of mamillary bodies, wall fo 3rd ventricle, and floor of 4th ventricle
|
|
|
B12 deficiency
|
Colbalamin deficiency
Destruction of white matter in dorsal and lateral columns of spinal cord |
|
|
Increased intracranial pressure
Threshold, mechanism |
>200 cm H2O with recumbent patient
Increased brain volume prevents venous outflow Can be generalized edema or focal (tumor, abscess, etc) |
|
|
Hydrocephalus
|
Excess accumulation of CSF in ventricular system
Caused by impaired flow or resorption |
|
|
Non-communicating hydrocephalus
|
Area of CSF accumulation from blockage
|
|
|
Communicating hydrocephalus
|
Enlargement of entire ventricular system
|
|
|
Hydrocephalus ex vacuo
|
Dilated ventricular system 2/2 brain loss
|
|
|
Causes of vasogenic edema
|
Trauma to vessels
Tumor Infection Anoxia |
|
|
Causes of generalized brain edema
|
Anoxia
Hepatic failure Reye's syndrome (in kids, also see fatty infiltration of kidney/liver) Diabetic ketoacidosis |
|
|
Questions to ask with a biopsy of mass?
|
Is this normal tissue?
Is this neoplastic? Metastatic or primary? |
|
|
Metastatic or neuroglial?
|
Neuroglial have infiltrative borders and neuropil background
Metastatic have well demarcated borders and |
|
|
Medulloblastoma family of tumors arise from
|
Neuroglial stem cells
|
|
|
Astrocytoma histologic appearance
|
Large irregular nuclei
Production of GFAP (oligos and ependys can also make this) |
|
|
Astrocytoma grading
|
I - pilocytic astrocytoma
II - astrocytoma III - anaplastic astrocytoma IV - glioblastoma |
|
|
Defining feature of a glioblastoma
|
Pleimorphic, hypercellular glioma
Areas of necrosis and/or extensive endothelial proliferation |
|
|
Primary vs Secondary glioblastoma
|
Primary -- arise as a glioblastoma, often have EGFR amplification/mutation, older pts
Secondary -- arise from background of lower grade astrocytoma, often have p53 mutations, younger pts |
|
|
Pilocytic astrocytoma (juvenile)
|
Benign astrocytoma arising in cerebellum or hypothalamus
Cystic lesions with small nodule Cerebellar can regress or be completely cured by surgery Hypothalamic not as good prognosis - don't regress, hard to get to surgicaly |
|
|
Oligodendroma
|
Fried egg appearance on H and E (artifact but consistent)
Often see calcium deposits 1p/19q loss is good prognostic |
|
|
Ependynoma
|
Regular small nuclei (looks a lot like oligo)
Gliovascular rosettes with nuclear clearing around capillary Sometimes can see basal body of cilia |
|
|
Medulloblastoma family tumor
Histologic appearance Types |
Sheets of small blue cells
Medulloblastoma (usually in posterior fossa, arising from cerebellar external granular layer) Pineoblastoma Retinoblastoma |
|
|
RB mutations lead to increased risk of
|
Retinoblastoma
Pineoblastoma (not medulloblastoma) FYI, its on chromosome 13 |
|
|
Gangliocytoma
|
Neuron like cells only
Slow growing Probably more like a hartoma |
|
|
Ganglioglioma
|
Glial tumor with significant neuron component
Prognosis is completely based on glial component |
|
|
Meningioma
|
Tumors arising from arachnoid
(often look like big granulations) Slow growing Surgically cured or treated with repeated excisions |
|
|
Schwanomma
|
Tumor arising from schwann cells of peripheral nerve
In cranium -- acoustic neuroma or arising from CNV Cells have streaming, spindle shape, cigar shaped nuclei, Verocay bodies |
|
|
Differentiating Schwanoma and Meningioma
|
Reticulin stain
Basement membrane -- around periphery of each Schwann cells, in course fibers around meningioma |
|
|
Pituitary adenoma
|
Small round nuclei
Presentation with endocrine symptoms (often ammenorhea first in women), bitemporal hemianopsia Rarely progress to malignancy |
|
|
Medulloblastoma classic feature
|
Rosettes
w/ lumen (Flexner-Wintersteiner) or without (Homer-Wright) |
|
|
Papillary meningioma
Hemangiopericytoma |
Two patterns of meningioma growth that make it worse prognositically
Bump it up to grade 2 |
|
|
Grade 2 or atypical meningiomas
|
Necrosis
Brain invasion Increased cellularity/pleiomorphism |
|
|
Contact cranial injuries
|
Kinetic force transmitted directly to skull/brain
--skull fractures --epidural hematomas --gunshot wounds --blast wounds |
|
|
Open skull fractures
define, risk, treatment |
Skin over fracture broken
Often require elevation and debridement to avoid infection |
|
|
Membraneous bones fractures
|
Often heal only with fibrous scar
|
|
|
Why is there depressed consciousness with epidural hematoma
|
Due to hematoma pressure
Not brain injury Dramatic improvement with treatment |
|
|
Gunshot head wound management and prognostics
|
Debride to prevent infection
Level of consciousness in impt progonsis Bihemispheric trajectory is worse |
|
|
Mechanism of decelleration injuries
|
Different densities of white and gray matter give different decelleration, results in rotation injuries at the gray/white jnct
Tears axons, causes contusions |
|
|
Depressed consciousness in subdural hematoma caused by
|
Both hematoma and rotational injury to brain
Does not always recover with treatmetn |
|
|
Mortality of an acute subdural hematoma
|
50-70%
May be rising because of increased coumadin use |
|
|
Chronic subdural hematoma
|
Occurs in elderly (more space in cranium)
HA, confusion, focal deficits--weeks after a relatively minor trauma Collection in cerebral convexity |
|
|
Elevated intracranial pressure
|
Not good prognostically
unclear if its a marker or variable Normal is <12 mmHg |
|
|
Management of elevated intracranial pressure
|
Cranial Perfusion Pressure management is mostly used
CPP = MAP - ICP Cerebral blood flow is what needs to be maintained Increase systemic pressure This maintains flow and allows for the blood vessels in the brain to constrict Leading to a decrease in the volume of the brain and tf ICP |
|
|
Spinal cord injuries 1940s advances
Ludwig Guttman |
Supportive care and reintroduction into society
-- drops mortality in first year from 90% to 5% -- rehab and reintroduction is born as medical concept |
|
|
Complete spinal cord injury
|
No motor or sensory function below lesion
5% will walk again |
|
|
Partial spinal cord injury
|
Some preservation of motor/sensory below lesion
Prognosis variable Tend to get better with time (difficult to study population) |
|
|
Steroids in spinal cord injury
|
Complicated history
Some positive studies, but now does not look like it helps Not currently a favored treatment |
|
|
Surgery in spinal cord injury
|
Spinal cord decompression
not proven how much compression is related to deficiencies or correction helps Correction of deformity hopes to help with pain |
|
|
Leading cause of death in people <40
|
Head trauma
|
|
|
Death from head trauma (%) and cause
|
1-2%
50% are vehicular accident |
|
|
Number of people in US per year with severe head trauma
|
700,000
Mortality with injuries increase 3x if one of them is a head injury |
|
|
Common mechanisms for head traumas
|
Falls
Motor vehicle accidents Bike accidents Assaults |
|
|
Concussion
|
Totally reversible cerebral dysfunction
May be associated with brief loss of consciousness, postural tone No known underlying pathologic changes ?focal axonal injury ?transient BBB damage |
|
|
Blunt head trauma
Initial injuries Delayed injuries |
Initial - lacerations, diffuse axonal injury, skull fracture, hemorrhage
Delay -- increased ICP, intracranial hematoma, ischemia, infection |
|
|
Extracranial hematoma with blunt head trauma forms where?
|
Under galea
Over/in loose connective tissue overlying periosteum |
|
|
Contusion and lacerations
|
Damage to brain right under skull fracture
Tops of gyri are injured in contusions Lacerate small blood vessels, arterioles in white matter |
|
|
Areas susceptible to contusions
|
Inferior anterior frontal lobe
Anterior temporal lobe Occiput (slightly less) |
|
|
Resolution of contusions
|
If survive
Cystic area from reabsorbed brain Gliotic scar |
|
|
Difference in injuries between getting hit with something and falling on head
|
Hit -- more coup than countercoup injury
-- on coup side weapon exerts positive force, then decelerating brain exerts positive force -- counter has a +/- Fall Counter coup more than coup Counter coup has brain acceleration and skull deceleration (2 + forces) Coup has +/- |
|
|
Diffuse axonal injury
|
Widespread damage to axons
Resulting from severe deceleration or acceleration of head Pts usually comatose immediately and do not recover consciousness |
|
|
Areas often damaged with diffuse axonal injury
|
Corpus callosum
Walls of 3rd ventricle Dorsolateral brainstem -- esp mesencephalon Periaqueductal gray |
|
|
Microscopic time course of diffuse axonal injury
|
4-5 hours -- focal accumulations of beta amyloid precursor protein (B-APP) at sites of shear injury
12-24 hours -- axononal varicosities 24-2 months -- active microglial, spheroids Long term -- Wallerian degeneration, atrophy |
|
|
Diffuse brain edema in kids
|
Bihemispheric
After injury (sometimes trivial) Extreme swelling 2/2 problem with vasoregulation Death 2/2 herniation in hours |
|
|
Causes of diffuse brain hypoxia
|
Brain shifts occluding major vessels
Increased ICP tampenading Systemic hypoxia Arterial spasm Often see effects at boundary zones |
|
|
truism
|
n. self-evident truth, platitude. Many a truism is summed up in a proverb; for example, "Marry in haste, repent at leisure."
|
|
|
Prevalence of child abuse
|
Difficult to know
USA: 1 in 100 50% are <3 25% are <1 |
|
|
Shaken baby syndrome
|
Somewhat controversial
Present in coma May have little external damage Sub and epi hematomas with contusion to cervical spine --- telling combo |
|
|
Meningitis symptoms
|
Headache, diffuse and pulsatile
Fever Stiff neck Mental status change N/V Development of focal neurologic deficits or seizures |
|
|
Brain tumor headache
|
Unremitting
Sometimes with focal neurologic deficit Sometimes worse when laying down Focal, hemicranial, holocranial Eventually increased intracranial pressure |
|
|
Symptoms of increased pressure
|
Loss of venous pulsations on fundoscopic
Papilledema N/V double vision |
|
|
Is this headache a stroke?
|
Maybe
Is the patient older and with risk factors for stroke? |
|
|
Acute subarachnoid hemorrhage
|
Worst headache of life
Sudden onset, possibly preceeded by exertion (including coitus) Usually with n/v, neck stiffness, mental status change |
|
|
Diagnosing a subarachnoid
|
Most seen on CT
Lumbar puncture containing blood, increased protein, increased pressure Can also use MRA, CT angiography |
|
|
Temporal arteritis
|
Can present with any kind of headache
Most patients are over 50 and have an ESR >50 Immediate treatment with steroids to avoid blindness and stroke |
|
|
Idiopathic intracranial hypertension
Pseudotumor cerebri |
Headaches resemble migranes, pulsitile with blurry vision
Usually have papilledema May have a CNVI palsy No intracranial mass, although may have venous thrombosis High opening pressure of LP Needs to be treated to avoid loss of vision |
|
|
Status migranosus
|
Migraine headache lasting longer than 72 hours
|
|
|
Migraine headaches
|
Can be focal, hemicranial, or holocranial
Anorexia, n/v, photo/phonophobia Normal neurologic exam More common in women than men |
|
|
Many women suffer migraines around
|
Beginning of menstrual period
Presumably 2/2 falling estrogen levels Birth control/estrogen therapy typically worsens |
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|
Cluster headaches
|
Periorbital, unilateral headaches
Come in groups or chronic Lasting 30 to 90 minutes Associated with congestion and lacrimation Often awaken patient 90 minutes into sleep |
|
|
Cluster headache triggers during sensitive period
|
Histamine
Alcohol Nitroglycerin |
|
|
Trigeminal autonomic cephlaplegia
|
Like clusters but shorter in duration
|
|
|
Analgesic rebound headache
|
From the chronic use of short acting, immediate relief medications
Such as acetaminophen, aspirin, butalbital, opiods Refractory to treatment until washout May be mediated by upregulation of serotonin |
|
|
How often can short acting medications be used safely in migraines
|
Twice a week
|
|
|
Post concussive headaches
|
Can be of any time
Treated as the type of headache 70% gone in one year and 85% at 3 years |
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|
Drug that induce headache
|
Cocaine
Vasodilators (hydralazine, isosoribide, and nitroglycerin) minodixil, nifidepine, rantidine, cimetidine, piroxicam, trimethoprim, sulfa, nalaxic acid, griseofulvin, diclofenac, danazol, estrogen, progesterone, reserpine, proton pump inhibitors and SSRI medications |
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|
TMJ
|
temporalmandibular joint dysfunction
Can mimic migraines with anterior ear pain Frequently with jaw clicking, popping Exacerbation with chewing, jaw opening Bite splints, massage, NSAIDs |
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|
Sinus headaches
|
Bifrontal or bimaxillary
May throb Temporally related to sinus disease Most sinus headache complaints are actually migraines |
|
|
Occipital neuralgia
|
Pain, nerves and tingling over greater occipital nerve distribution
Tinel's sign may be positive May treat with local anesthetic |
|
|
Trigeminal neuralgia
Tic douloureaux |
Paroxysms of severe, lancinating pain in a CN V division, often V2
Either single jolts or trains Trigger point can reproduce (although refractory after pain) |
|
|
Trigeminal neuralgia
Causes and treatment |
Idiopathic
Compressive lesions Demyelinating disease Carbmazepine Microvascular decompression (Jannetta procedure) |
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|
Stroke
|
Sudden neurologic deficits caused by a blood vessel problem
Hemorrhagic or ischemic |
|
|
3rd leading cause of death in US
|
Stroke
|
|
|
Stroke warning signs
|
Sudden weakness or numbness
esp on one side Sudden confusion, trouble speaking Sudden trouble seeing Sudden trouble walking, loss of coordination, dizziness |
|
|
Difficulty with stroke diagnosis
|
No test
Not painful Poor recognition of symptoms |
|
|
Special radiology for strokes
|
Diffusion weighted imaging
Perfusion imaging Difference between the two -- penumbra |
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|
Causes of ischemic stroke
|
Cardiomyopathies
Afib Atherosclerosis Verterbal artery disease Intracranial disease Arterial dissection Hypercoaguable states Drugs Cerebral venous thrombosis |
|
|
Options for treating an ischemic stroke
|
tPA (within 3 hours)
Intra-arterial thrombolysis (6 hours) Corkscrew cath to pull out clot (8 hours) |
|
|
Best way to treat a stroke?
|
Prevent it
|
|
|
Preventative treatments of stroke in descending order of magnitude of effect
|
Warfarin in afib
Carotid enderectomy (if sympt) Smoking Cessation HTN treatment Statins for high cholesterol Aspirin |
|
|
Subarachnoid hemorrhage causes
|
Aneurysms
Trauma Drugs |
|
|
Intracerebral hemorrhage causes
|
Small vessel disease/HTN
Anticoagulants Trauma Bleeding disorders Tumors Aneurysms/AVMs |
|
|
Amyloid angiopathy
|
Proteins deposited in blood vessel walls increase chance of rupture
Associated with increasing age, AD |
|
|
Preventing hemorrhagic strokes
|
Prevent inappropriate coagulation
Smoking cessation HTN treatment Avoid trauma Avoid drugs |
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|
Why is smoking bad for strokes?
|
Promotes aneurysm formation and growth
Can lead to subarachnoid bleeds |
|
|
Myelin components
|
Myelin basic protein
Cholesterol Proteolipid apoprotein Myelin-oligo specific protein, glycoprotein Gangliosides GM1, GM4 Galactocerebroside Wolfram proteins, chlorophorm-methanol insoluble |
|
|
Difference between myelin and regular plasma membrane
|
More lipids
Half-life of components last much longer |
|
|
When can you see myelin antigens by immunofloresence?
|
During active mitogenesis
|
|
|
Difference between central and peripheral myelin?
|
Origin (oligo vs schwann)
Stain w/ LFB-PAS (sky vs purple) Antigenic (disease specificity) Nodes (bare vs interdigitated) |
|
|
Dys vs de myelination
|
Dys -- improperly formed myelin
-genetic or metabolic =leukodystrophies De -- destruction of myelin -- immunlogically mediated |
|
|
Viral induced demyelination
|
Viruses that infect oligos
Either kill them or render them unable to make myelin |
|
|
Subacute sclerosing panencephalitis
|
SSPE
Late complication of measles Widespread demyelination of cerebrum High anti-measles titers in blood and CSF Presents years after measles, often in kids who were <2 when infected boys>girls ? viability of the measles virions isolated in oligos |
|
|
Progressive multifocal leukoencephalopathy
|
JC virus (papova virus) in immunocompromised patients
Irregular demyelination |
|
|
Diptheria and demyelination
|
Of cranial and spinal nerves at nerve roots
Caused by toxin Usually no damage to axon and can by remyelinated |
|
|
Experimental allergic encephalomyelitis
Experimental allergic neuritis |
Injection of neurologic material induces immune response
CD4s, macros Selective phagocytosis of myelin with sparing of axons and oligos |
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|
Acute disseminated encephalitis
Post-infectious encephalomyelitis Acute hemorrhagic leukoencephalitis |
Similar entities
Demyelination shortly following immunologic challenge (infection or immunization) May reflect viral infection and likely involves immunologic reaction like experimental |
|
|
Guillian-Barre
Idiopathic polyneuropathy |
Rapidly progressing bilateral ascending paralysis
After a non-specific viral infection Immunologic (autoimmune) destruction specific to PNS myelin, sparing axons and Schwanns. Usually regenerates |
|
|
Chronic Inflammatory Demyelinating Polyneuropathy
|
CIDP
Chronic, deteriorating, or recurrent Guillian Barre |
|
|
Instigating event preceeding CIDP
|
Infection
Operation Immunization |
|
|
Who is more at risk for MS?
|
Women
Northern European > Mediterranean > Africans/Asias Northern climates > Equatorial Before 15 is what matters |
|
|
Viral etiology of MS
|
Prior to age 15 climate is what is important
Suggestion that a slow virus may be involved |
|
|
MLA type associated with MS?
|
HLA-DR2
|
|
|
MS lesions must be
|
Separated in space and time
|
|
|
Typical age of MS onset
|
20-40
|
|
|
Areas with MS vulnerability
|
Angles of lateral ventricles
Optic nerves and optic chiasm Floor of fourth ventricle Adjacent to pial surface in spinal cord and brain stem |
|
|
MS plaque
|
Irregular
Sharply demarcated Area of demyelination Loss of myelin, loss of oligos Early preservation of axons |
|
|
Early vs late plaque
|
Early -- lots of macros with myelin products in them
Late -- gliosis |
|
|
Long term disability in MS is from
|
Axonal loss
|
|
|
Demyelination secondary to edema
|
Changes is brain extracellular fluid affect myelin
Demeylination can occur after tumors, abcesses, infarcts, etc secondary to prolonged edema |
|
|
Hypertrophic or onion skin neropathies
|
Peripheral neuropathies from repeated cycles of demyelination and remyelination
Results in layers of schwann cells processes Associated with Charot-Marie-Tooth, Sottas, Refsum's |
|
|
Leukdystrophies
|
Demyelinating enzymatic defect disorders
Progressive and fatal |
|
|
Adrenoleukodystrophy (ALD)
|
X-linked recessive peroxisomal deficiency
Accumulation of very long chain FAs Extensive demyelination (cerebrum, cerebellum, brain stem) Adrenal insufficiency --presenting sign |
|
|
Treatment of adrenoleukodystrophy
|
Nothing if disease is established
Early treatment with Lorenzo's oil can cessate |
|
|
Krabbe's disease
Globoid cell leukodystrophy |
AR deficiency of glactocerobroside-B-galactidase
PNS and CNS Presents in infancy with spasticity -->total decerebrate posturing Seizures, optic atrophy, exaggerated startle response Fatal by 2 Gloiboid cells are large, multinucleate, macrophage like cells found in gliotic white matter |
|
|
Metachromatic leukodystrophy
|
AR deficiency of arylsulfatase-A
Any age, mostly infants Hypotonia --> spasticity, dementia, quadiparesis Fatal in 5 years Build up of galactolipids, mostly sulfides. Macros in the white matter |
|
|
Canavan disease
|
Spongiform leukodystrophy
Aspartoacylcase deficiency MR, megacephaly, hypotonia Fatal by age 10 |
|
|
Location of plaques in MS
|
Perivascular -- that's where the inflammatory cells are coming from
|
|
|
Schilder's Disease
|
Large bilateral demyelinating lesions occurring in a short period of time
Progressive or relapsing remitting ?MS variant |
|
|
Balo's concentric sclerosis
|
T-cell/macro mediated demylination of alternating layers
Destroys oligos in demyelinated layers Severe and rapidly progressive Cause unknown |
|
|
Acute disseminated enecphalomyelitis
Inciting incidents |
Rabies vaccine
Snake bite Malaria Liver transplant Leptospirosis Enteroviral infections Anit-tetanus Hep A Lyme Campylobacter jejuni Mumps |
|
|
Weston Hurst Disease
Acute Hemorrhagic Encephalomyelitis |
Abrupt onset, progressing to seizures, motor signs, coma and death in 1-5 days
Infiltrate includes PMNs in addition to Tcells/macros TypeIII + ADEM? |
|
|
Central Pontine Myelinolysis
|
Rapid onset of motor and sensory symptoms, locked in syndrome, coma and death
Seen in the already sick. Diseases that cause rapid electrolyte shifts (liver failure, HIV, etc). Can be iatrogenic |
|
|
What is different about central pontine myelinolysis from other demyelinating diseases?
|
There is no inflammation
Oligos die and axons in the area are transected |
|
|
Metachromatic leukodystrophy, what is spared?
|
Subcortical U fibers
|
|
|
Leukodystrophies pathogenesis
|
Some enzyme defect
Oligos need that enzyme Heterogenous materials build up Oligos die Myelin lost Usually without inflammation |
|
|
Pelizaeus-Merzbacher Disease
|
Neurologic degeneration with blindess, seizures, spasticity --> death
CNS only PLP gene mutation Onset can be until 50 |
|
|
PLP knockout mice?
|
Pretty much fine
Worse to have a bad protein than no protein Thought is that misfolded proteins buildup in ER and cause apoptosis |
|
|
Typical presentations of MS
|
Gait disorder
Optic neuritis/retrobulbar neuritis Trigeminal neuralgia Intranuclear opthalmoplegia Transvere myelitis Lhermitt's phenomenon |
|
|
Symptoms of MS
|
Decrease vision
Eye pain Diploplia Weakness Paresthesias Vertigo Bowl/bladder dys Sex dys Fatigue Cognitive disturbances Vertigo Ataxia |
|
|
Important mediators in MS
|
NO
TNFalpha |
|
|
Demyelinated axons
|
Cannot perform saltatory conduction
Na channels regenerate along length Can result in nerve block, or inappropriate signalling |
|
|
Diagnosing MS
|
Objective lesions separated in space and time
90% have abnormal MRI CSF with polyclonal IgG, slightly increased protein, mononuclear Elevated MBP |
|
|
Must rule out for MS diagnosis
|
SLE
Lyme Burcellosis Neurosarcoid Neurosyphillis AIDS HTLV-1 Paraneoplastic |
|
|
Treatment of MS attack
|
Steroids
|
|
|
ACTH/cytoxan in MS
|
Can be used in chronic progressive
Benefits about 1/3 Hurts about 1/3 Can be very toxic |
|
|
ABC treatment in MS
|
Immunomodulatory
Beta interferons (Avonex, Betaseron) Galtiramer acetate (Copaxone) Lessen flares, improves MRIs in relapsing remitting, ?change course |
|
|
Most common symptom in MS?
|
Fatigue
|
|
|
Supportive treatment in MS
|
Adequate rest and rapid treatment of infections
Amantadine, modafenil, pemoline, methlyphenidate -- fatigue Amytriptyline -- urine retention and depression Baclofen, tizandine, diazapem - spasticity Gabapentin, valproate, carbmazepine - pain and sensory symptoms |
|
|
Guillan Barre
What's the danger to life? Treatable? |
Respiratory failure
Treatable |
|
|
Guillan Barre
Reflexes Sensory |
Deep tendon reflexes lost
More motor than sesnory |
|
|
Fisher variant of GB
|
Presents with CN symptoms
These usually come on late (like respiratory symptoms) |
|
|
Labs in GB
|
CSF -- high protein, few WBCs
Inflammation of nerve roots Decreased conduction velocities, proximal conduction blocks |
|
|
Treatment of ??
|
Respiratory monitoring/support
Plasmapheresis Most recover (20% with disability) young do better |
|
|
CIDP from GB
|
3% progress to chronic
|
|
|
Guillan Barre AKA
|
Acute Inflammatory Demyelinating Polyradicular Neuropathy
Treat with plasmphoresis, immunosuppression |
|
|
Course of relapsing remitting MS
|
Attacks
Returns to baseline for a while Develops increasing disability -often with decreased attacks |
|
|
Course of secondary progressive MS
|
Attacks with return to increasingly disabled baseline
|
|
|
Course of primary progressive MS
|
Chronic progression
No attacks |
|
|
Course of progressive relapsing MS
|
Progessive MS with attacks
|
|
|
Median delay between symptoms and diagnosis of MS
|
3.5 year
|
|
|
What is an MS attack
|
Neurogloic disturbance lasting at least a day
30 days since previous onset of symptom |
|
|
Diagnosing MS
|
Objective abnormalities of CNS
Predominant white matter involvement Exam/history show 2 distinct CNS lesions Two or more events or gradual progression over at least 6 months |
|
|
Probable MS
|
Only one lesion or only one episode
|
|
|
Clinically isolated syndrome
|
Only one symptom, episode
Not MS yet |
|
|
MRIs in MS show you?
|
T1 - look for black holes, atrophy (pretty far gone MS)
T2/Flair - disease burden T1 + gadolinium -- defects in BBB - active MS |
|
|
VER?
|
Visual evoked responses
Delayed in MS (not always) |
|
|
Neuroprotective factors
|
Th2 cytokines
Regulatory T cells TGFbeta Il-10 Neurotrophic factors |
|
|
Beta interferon effects
|
Decreases T cell activation
Rapidly decreases Gd lesions Neuroprotective by anti-inflam Decreased T cell adhesion |
|
|
Glatiramer acetate effect
|
Increases Th2s in CNS
Bystander suppression Protects from black holes, BDNF, axonal protection |
|
|
Natalizuab action (Tysabri)
|
Antibody to alpha integrins
Keeps T cells out of CNS Limited use because of SEs |
|
|
Fingolimod action
|
Keeps T cells in lymph nodes
|
|
|
When to treat MS?
|
Early as diagnosed
May improve outcome Reduce accumulation of disability May prevent irreversible damage to brain |
|
|
Sensory fibers by size
|
Largest - Muscle stretch
Intermediate - Fine touch, proprio Small - Pain, temp, autonomic fnc |
|
|
Sensory nerve conduction studies
|
Stimulate nerve near surface
Measure amplitude and time it takes to another spot Decreased amplitude -- loss of axons Decreased speed -- loss of myelin |
|
|
Motor nerve conduction studies
|
Stimulate nerve and measure muscle response
Small responses -- loss of axons or muscle fibers Slow responses -- loss of meylin |
|
|
Fibrillation
|
Brief electrical activity in a deinnervated muscle
|
|
|
Giant motor unit potentials
|
An axon has taken on additional muscle fibers because they lost their own axon
|
|
|
Tiny/myopathic motor unit potential
|
Extensive damage leaves few fibers for an axons
Need to recruit other units with minimal effort |
|
|
Entrapment neuropathy
|
Mechanical damage to nerve results is dysfunction
Damage by compression or demylination |
|
|
Common sites of entrapment neuropathy
|
Median
Ulnar around in wrist Radial in spiral groove Fibular nerve (foot drop) Latral femoral cutaneous |
|
|
Meralgia paresthetica
|
Entrapment neuropathy of lateral femoral cutaneous
Burning on lateral thigh |
|
|
Tinel's sign
|
Light tapping on damaged nerve produces tingling in area of distribution or peripheral nerve
Remyelinating neurons are more susceptible to mechanical stimulus |
|
|
How fast do axons regenerate in peripheral nerve
|
1 inch/month
|
|
|
What helps peripheral nerves regenerate?
|
Axonal damage only
Intact connective tissue |
|
|
Neurapraxia
|
Stunned peripheral nerves
Non-transecting trauma leads to inability maintain RMP and transmit signals Resolves in hours |
|
|
Symptoms helpful at localizing a lesion to a peripheral nerve?
|
area of sensation loss
area of motor deficit Pain is less helpful as it may be more widesprea |
|
|
Saturday night palsy
|
Neuropraxia of the radial nerve
Often from sleeping with arm over shoulder |
|
|
Diseases of the anterior horn cells
|
ALS
Polio |
|
|
Amyotrophic Lateral Sclerosis
|
Upper and lower motor symptoms
No significant sensory/sphincter symptoms Onset 50-70 Progressive with respiratory problems and swallowing problems (3 years) Some cases are familial |
|
|
Diagnosis and treatment of ALS
|
Diagnosis by EMG showing widespread deinnervation
No effective treatment |
|
|
Peripheral neuropathy
|
Longest peripheral nerves are damaged (axons, myelin, both)
Systemic disease affect feet first Paresthesias, dyesthesias Vibratory and proprioception can be lost Can have distal weakness |
|
|
Causes of peripheral neuropathy
|
25% idiopathic
Diabetes B vitamin deficiency Toxins (including chemos) Lupus, RA, PAN Tertiary syph, lyme, leprosy Sarcoidosis Demyelinating diseases Inherited conditions CMT |
|
|
Myopathies
|
Inflammatory
Congenital Metabolic disorders |
|
|
Inflammatory myopathies
|
Polymyocitis
Dermatomyocitis Inclusion body myocitis |
|
|
Polymyocitis
Which muscles first? Symptoms |
Proximal
Muscle soreness Constitutional symptoms |
|
|
What reflects the degree of damage in polymyocitis
|
CK and sedimentation rate
|
|
|
Dermatomyocitis
|
Inflammatory disease of muscles
Rash (often heliotrope around the eyes) |
|
|
Dermatomyocitis
Polymyocitis Treatment |
Immunosuppresion
|
|
|
Inclusion body myositis
|
Slowly progressive, less proximal
Symmetrical Involving flexors of wrist and fingers CK elevated, but ESR may not be No effective treatment |
|
|
Metabolic myopathies
|
Many metabolic disorders show up as myopathies because muscle is so metabolically active
|
|
|
Appearance of muscle biopsy of mitochondrial disease
|
Ragged red fibers
Mitochondria along outside |
|
|
Duchenne's muscular dystrophy
|
Absent dystrophin gene
X-linked Presents before 5, progressive weakness, death in 20s Cardiac abnormalities lead to death |
|
|
Presentation of muscular dystrophy
|
Gait abnormalities
Proximal muscle weakness Pseudohypertrophy (replacement with fat) |
|
|
Becker's dystrophy
|
Abnormal dystrophin
Presentation before 10 and slower progression that Duchenne's |
|
|
Other dystrophies
|
Facioscapulohumoral dystrophy
Shoulder girdle Oculopharyngeal dystrophy --ptosis, late onset, french canadians, latinos |
|
|
Myotonic dystrophies
|
Genetic -- repeats
Predominantly distal weakness Myotonic component -- inability to relax after contraction Delayed relaxation after tapping on thenar muscles May be temporal wasting Cardiac conduction abnormalities |
|
|
Pregnancies in myotonic dystrophy
|
Kids of myotonic moms are more severely effected
Womb effect |
|
|
Myastenia gravis
|
Autoimmune condition
Antibodies against AcH receptors -- blocks and mediates destruction Fatigue is the hallmark Early onset is more women, later it evens out |
|
|
Myastenia gravis course
|
Ptosis, diploplia, swallowing, speech problems
Sometimes remains in eyes only (ocular myastenia) Or progresses within the year Respiratory failure is the problem |
|
|
Myastenia gravis treatment
|
anti-cholinesterases
immunosuppresion plasmophoresis |
|
|
Thymus and myastenia
|
Many patients with MG have thymus tumors
Antibodies against a thymic surface antigen and the AchR is a bystander effect |
|
|
Lambert-Eton myastenic syndrome (LEMS)
|
Antibodies against voltage gated Ca channels at motor neuron terminals
Weakness (hip girdle), diminished reflexes, autonomic dysfunction Muscles become stronger with use |
|
|
LEMS often seen in patients with
|
Cancer
Usually small cell lung cancer |
|
|
LEMS treatment
|
Anticholinesterases
Immune modulation |
|
|
Myofilaments
|
Contractile parts of myofibrils
|
|
|
What determines if a muscle fiber will be type one or type two?
|
Nerve that innervates it
|
|
|
What do deinnervated muscle fibers look like?
|
Atrophic
More angular |
|
|
Fiber type grouping
|
Typically fibers within a muscle are scatters of Type 1 and Type 2
If axonal loss and reinnervation happens through sprouting from nearby axons, then there will be bunch of the same type together |
|
|
Leprosy and nerves
|
Mycobacterium leprosum
Segmental demeylination and remyelination Granulomatous inflammation |
|
|
Shingles
|
Varicella zoster reactivation
Vesicular rash along dermatome Ganglia show neuronal destruction and axonal damage Abundant mononuclear infiltrates Often brought on by decreased cellular immunity |
|
|
Tetanus
|
Clostridium tetani
Tense paralysis Toxin (tetanaospasm) is retrogradely transported up axon and spreads |
|
|
Biopsy of nerve in DM neuropathy shows
|
Loss of small fibers
Preservation of larger fibers Arteriole thickening |
|
|
Toxins causing peripheral neuropathy
|
Uremic
Heavy metals Amiodorone Gold Ciplatinum Metronidazole Thalidomide Taxanes |
|
|
Deficiencies causing peripheral neuropath
|
Thiamine
B12 B6 VitE |
|
|
Spinal muscular atrophy
|
Autosomal recessive
Survival gene of motor neuron disrupted Progressive loss of lower motor neurons Infantile/pediatric disease |
|
|
ALS gene
|
20% of familial cases are superoxide dismutase
|
|
|
How many Duchenne's muscular dystrophy cases are de novo mutations?
|
1/3
|
|
|
Dermatomyositis patients are at increased risk of
|
Cancer
|
|
|
Dystrophic muscle histologic appearance
|
Increase in heterogenetity of fiber size
Connective tissue and fat infiltration |
|
|
Dystrophin
|
Large gene/protein
Holds cytoskeletal apparatus to extracellular matrix |
|
|
Which stairs direction is problematic in peripheral neuropathy
|
Going down
|
|
|
Treatment of AIDP and CIDP
|
IVIG
Plasmaphoresis Corticosteroids, Rituzin in chronic |
|
|
Prognosis in AIDP and CIDP
|
AIDP fatal 3-8%
degree of axonal loss is predictive involves autonomics CIDP Rarely fatal |
|
|
Atrophy of thenar eminence`
|
Early ALS symtom
|
|
|
Emotional lability in ALS
|
Overreact with facial emtions
Loss of repressing cortical input |
|
|
Tongue atrophy and weakness
|
Rare
Seen with ALS |
|
|
Facioscupulohumeral muscular dystropy symptoms
|
Sleeping with eyes open
Drooling |
|
|
Muscular dystrophys usually result in weakness
|
Proximate
Myotonic Type I is exception |
|
|
Myotonic dystrophy genetics
|
Autosomal dominant
CTG repeat affecting protein kinase needed for muscle cell maturation Worse with generations--repeat expands |
|
|
Myelopathy
|
Change to the spinal cord of any cause
|
|
|
Myelopathy symptoms
|
Weakness below lesion
-usually spastic Loss of sensation below lesion -pain sensation loss will start below level because of dorsal tract of Lissaur Bladder and bowel problems -spasticity with urgency and volume incontinence Level symptoms |
|
|
Myelopathic symptoms at level of injury
|
Radicular symptoms
Lower motor weakness with flaccid paralysis |
|
|
CN problems from myelopathy
|
Only potential ones
--decreased pain&temp in face from craniocervical jct lesions --SCM, and trapezius |
|
|
DDx for patient with bilateral lower limb symptoms
|
Myelopathy
Polyneuropathy |
|
|
Localizing spinal cord lesions
|
Back pain to percussion
Radicular pain or LMN weakness Sensory loss helps with lowest possible lesion |
|
|
Causes of myelopathy
|
Spondylosis
Trauma Tumor Infection Hematoma Metabolic Demeylination Degeneration Syrinx Infarct |
|
|
Spondylosis
|
Degeneration of spine
Often with spinal stenosis and disc protrusion Usually slowly progressive, can have overlying trauma |
|
|
Myelopathy tumors
|
Usually spinal metastasis
Lung, breast, prostate, kidney, lymphoma 20% cervical, 60% thoracic, 20% lumbar (conus symptoms) |
|
|
Symptoms spinal tumors
|
Pain 90% first
Weakness 80% Numbness/paresthesias 55% Bowel bladder 40% |
|
|
Signs of spinal tumor
|
Weakness 85%
Sensory level 70% Abnormal reflex 65% Babinski 50% Local tenderness 70% Decreased rectal tone 60% |
|
|
Treatment of spinal cord tumors
|
Corticosteroids -- can help preserve fnc
Decompression may be useful if there is no known primary, spine in unstable, not responsive |
|
|
Extramedullary spinal cord tumors
|
Mutiple myeloma
Lymphoma |
|
|
Intradural extramedullary spinal cord tumors
|
Meningioma, neurofibroma, ependynoma
|
|
|
Intramedullary spinal cord tumors
|
Astrocytoma, Ependynoma
|
|
|
Epidural abcess
|
Medical emergency
Acute, severe, focal back pain ESR elevated, +/- fever May result from vertebral osteomyelitis, discitis |
|
|
Infections that target the spinal cord
|
Lyme
Syph CMV (usually with AIDS) HTLV-1 |
|
|
Hematomas of the spinal cord
|
Usually epidural
Usually with trauma, on anticoagulants, with bleeding disorders |
|
|
Subacute combined degeneration
|
B12 deficiency
Usually absorption rather than intake Loss of vibratory, position sense with preservation of pain Bladder control preserved |
|
|
Lhermitte's sign
|
Forced neck flexion results in tingling up and down back of legs
Sign of dorsal column irritation Can be positive in MS |
|
|
Does ALS affect the bladder?
|
No
|
|
|
Infarction in the spinal cord
|
Rare
Usually with severe vascular disease, aortic disease Usually painless unless subarachnoid hemorrhage |
|
|
Transverse myelitis
|
Unknown cause
Acute myelopathy Often days to weeks Typically painful |
|
|
Radiculopathy
|
Something is taking up the space of the nerve root
--mostly happens at the intravertebral foramen (disc, osteophytes) |
|
|
Symptoms of radiculopathy
|
Sharp shooting pain
--- increased with movements that compress or stretch nerve Weakness common, loss of reflexes Sensory loss unlikely unless in autonomous areas |
|
|
Intravertebral disc disease
|
Fissures in annulous fibrosis leads to nucleus pulposus escpang
If free in spinal canal can irritate as well as compress cord Usually damages nerve of higher number |
|
|
Joint degeneration and radiculopathy
|
In cervical/lumbar spine facet joint degenerations and osteophyte regrowth can impinge on intravertebral foramen
|
|
|
Lumbar spinal stenosis
|
Neurogenic claudication
Due to vertebral column that is too small cutting of blood supply to cauda equina With standing, the disc bulging reduces diameter further Weakness, paresthesias, fatigue in legs Improves with spine flexion |
|
|
Cauda equina syndrome
|
Compressive lesions of cauda equina
Bilateral lower leg symptoms Flaccid weakness, bladder disturbance, loss of sensations Bilateral sciatica Medical emergency |
|
|
Red flags with back pain
|
Significant trauma
Immunosuppression, anticoagulated New back pain over 50 Cancer Fever raised ESR IV drug abuse Back pain is worse at rest Deteriorating neurologic fnc |
|
|
Yellow flags in back pain
|
Bilateral sciatica
Perianal anesthesia Bladder dysfunction Multiple nerve root involvement Lack of improvement with conservative management |
|
|
Spondolithises
|
Anterior slipage of one vertebra on the one below
Can result in stenosis or reticulopathy |
|
|
Kyphosis
|
Most often occurs with osteoporosis, often with compression fractures
Can be symptomatic of ankylosis spondylitis |
|
|
Spinal dysraphism
|
Neural tube fails to close
Defect occurs in first 4 weeks of pregnancy |
|
|
Spinal dysraphism symptoms
|
Sensation and movement
Bowel/bladder Sexual function Hydrocephalus (Arnold chiari) |
|
|
Prevalence of spinal dysraphism
|
1/1250 births
3000 kids/year Higher among Irish, English, Hispanic, Chinese Lowest in African Americans |
|
|
Occult spinal dysraphism diagnosis
|
Post natal
Skin over deformity can have hair, hyperpigmented/hypopigmented, lump or mass, asymmetric gluteal crease |
|
|
Spinal dysraphism prevention
|
Folic acid
Hyperdoses for women with kids that have spinal dysraphism |
|
|
Kids with spinal dysraphism have normal intelligence?
|
Usually
Some learning difficulties with at and attention |
|
|
Role of neurosurgery in spinal dysraphism immediately
|
Close placode, dura, muscle, skin
Evaluate for hydrocephalus, surgery increases chance of Arnold Chiari |
|
|
Arnold Chiari
|
Brainstem, cerebellum, 4th ventricle pushed through foramen magnum
Fluid may enter syrinx Treat with vetriculoperitoneal shunt |
|
|
Bladder in spinal dysraphism
|
Bladder is stiff and unyielding
Sphincter may or may not work Urine may leak, overflow back up to kidneys (hydronephrosis) Need to be catheterized UTI susceptible |
|
|
Appendovescicostomy
|
Use appendix as conduit from bladder to umbilicus
|
|
|
Spinal dysraphism and club foot
|
20% of patients
Can affect walking |
|
|
Spinal dysraphism and fractures
|
Osteoporosis from decreased use
-important to stand |
|
|
Meningismus
|
Nuchal rigidity
|
|
|
Brudzinski's sign
|
Spontaneous knee flexion with passive neck flexion
Meningeal irritation sign |
|
|
Kernig's sign
|
Resistance to passive leg extension
|
|
|
Chronic meningitis signs
|
Intermittent headaches
Mental status changes |
|
|
Define meningitis
|
CSF > 7-10 cells/mm3
|
|
|
Define encephalitis
|
Infectious invasion of brain parenchyma with focal neurologic symptoms
|
|
|
Define abcess
Empyema |
Mass caused by infection
Abcess is subdural or subarachoid space |
|
|
Bacterial meningitis organisms in babies
|
Group B strep
E coli Listeria monocytogenes |
|
|
Bacterial meningitis organisms in kids
|
Neisseria meningitis
Streptococcus pneumonia H. influenza (prevaccine) |
|
|
Bacterial meningitis organisms in seniors
|
Streptococcus pneumonia
Listeria monocytogenes |
|
|
Bacterial meningitis in neurosurgical patients
|
Stap aureus
Opportunistics |
|
|
Path of bacterial meningitis
|
Nasopharyngeal colonization
Epithelial cell invasion Blood stream Blood brain barrier Enters CSF through cribiform plate or via leak Exudate in subarachnoid space |
|
|
Bacterial meningitis symptoms
|
Fever, headache, meningismus
Kernig, Brudzinski positive Photophobia Vomiting Toxic appearance Increase ICP |
|
|
Diagnosis of bacterial meningitis
|
Start antibiotics immediately
LP (have 6 hours after treatment started) blood cultures ?imaging |
|
|
Normal CSF
appearance pressure RBC, WBC Protein Glucose |
Clear
<180 mmH2O <5 RBCs, WBCs < 40 mg/dl protein 60-80 mg/dl glucose |
|
|
CSF in bacterial meningitis
|
Cloudy
>180 mmHg >1000 WBC/mm3 variable RBCs Protein >40 Glucose <40 |
|
|
Treatment of bacterial meningitis
|
Ceftriaxone (ceph that penetrates BBB)
Vanco for resistances/staph +Ampicillin for Listeria Corticosteriods for swelling Intraventricular drain if needed Adjust to cultures |
|
|
Prognosis of bacterial meningitis
|
3-20%
25% sequellae (normal pressure hydrocephalus, seizures, CN defects) |
|
|
Viral meningitis symptoms and treatment
|
Fever, headache, meningismus
Not as severe Bedrest, fluids, analgesia |
|
|
Viruses causing meningitis?
|
Many
Echovirus, enterovirus, influenza |
|
|
CSF in viral meningitis
|
Clear
Normal pressure WBC <500, mostly lymphocytes Protein <40 Glucose normal May see oligoclonal bands Early will see granulocytes |
|
|
Herpes simplex encephalitis
|
Usually reactivation of dormant HSV in ganglia
Impairment of consciousness, confusion Seizures Headache, fever, meningismus Aphasia, hemiparesis |
|
|
Viral encephalitis CSF
|
Clear, normal pressure, normal glucose
WBC, lymphocytes <500 Protein <200 Herpes simplex PCR positive |
|
|
Treatment of HSV encephalitis
|
Acyclovir
Watch out for renal insufficency |
|
|
Normal CSF in toxic, meningeal patient?
|
HSV encephalitis
|
|
|
Herpes zoster encephalitis and myelitis
|
Shingles in immunsuppresion can lead to CNS infections
Reactivation from doromancy in dorsal root ganglia Treat with antivirals (acyclovir, etc) Can develop post herpetic pain |
|
|
Herpes zoster in CNV1 complication
|
Corneal scarring
|
|
|
Fungal meningitis presentation
|
Chronic meningeal symptoms
Fatigue, malaise, dementia Low grade fever Sometimes CN symptoms Often in an immunocompromised patient |
|
|
Fungal meningitis organisms
|
Cryptococcus neoformans
Aspergillus Candida neoformans |
|
|
CSF in fungal meningitis
|
Hazy
<200 WBCs Protein high Glucose <20 Eosinophila |
|
|
Cysticerosis
|
Tissue invasion with larval Taenia solium (tape worm)
Invades liver, muscles, brain, eye Enhancing cystic, calcifed lesions Treat only if focal symptoms Endemic in Latin America |
|
|
Tuberculous meningitis
|
Chronic meningeal presentation: headache, confusion
Seen in kids, HIV, immunosuppressed CN palsies, obstructive hydrocephalus Treat TB |
|
|
CSF in tuberculous meningitis
|
Hazy
variable pressure WBC, lymphcytes <200 Protein >300 Glucose very low Acid fast bacilli |
|
|
Neurosyphillus
|
Treponema pallidum
Early in syph infection can get meningitis, CN neuritis, cerebrovascular disease Late Sensory ataxia from dorsal column damage (tabes dorsalis) Dementia with psychosis |
|
|
Lyme disease neuro symptoms
|
Polyradiculitis
Chronic meningitis |
|
|
Chronic meningitis causes
|
Fungal
TB Lyme Syphilis Sarcoid Neoplastic |
|
|
Pathogenesis of brain abcesses
|
From otitis media, teeth, chronic pyogenic lung disease, trauma, surgery
|
|
|
Symptoms and course of brain abcesses
|
Encapsulated at first
HA, normal CSF No fever ICP Focal deficits/seizures Rupture Meningitis, death |
|
|
Appearance of brain abcess
|
Ring enhancing lesion
|
|
|
Treatment of brain abcesses
|
Antibiotics
Aspiration Anticonvulsants |
|
|
Treatment of subdural empeyema
|
Surgery
|
|
|
Common cause of epidural abcess?
|
Spinal anesthesia
|
|
|
Neural opportunistic infections in HIV/AIDS
|
Toxoplasmosis
TB Cryptococcal meningitis Syphilis CMV encephalitis Progressive multifocal encephalopathy |
|
|
Toxoplasmosis susceptibility in HIV? treatment?
DDx |
Increases with low CD4
Pyrimethamin, sulfadiazin Looks at lot like CNS lymphoma (but antibiotics do not cure that) |
|
|
PML
|
Progressive multifocal leukoencephalopathy
JC virus White matter lesions Treat HIV to treat this |
|
|
AIDS dementia
|
Direct effect of virus on brain
Disorientation and confusion Occurs with increasing viral load Treat with HIV antivirals |
|
|
Five neuro complications of AIDS
|
AIDS dementia
vaculoar myelopathy HAART associated neuropathy Myopathy with zidovidudine Meningitis with seroconversion |
|
|
Creutzfelt Jacob Disease
|
Spongiform encephalopathy
Prion transmitted Rapid dementia and death in months Normal CSF, imaging (except diffusion) |
|
|
Pachymeningitis
|
Inflammation of the dura
|
|
|
Leptomeningitis
|
Inflammation of the arachnoid and pia
|
|
|
Routes of infection into CNS
|
Defects in protective coverings: developmental, iatrogenic, traumatic
Direct spread from sinusitis, otitis, mastoiditis, spinal abcess Hematogenous, most often from lung Retrograde neural transport Opportunistic penetration along cribiform plate |
|
|
Prion diseases
|
Slowly progressive dementia
Spongiform encephalopathy Kuru, Scrapie, CJD. Gershmann-Straussler, Fatal Familial Insomnia, Bonvine spongiform encephalopathy |
|
|
Prions are resistant to? sensitive to?
|
Sensitive to proteases
Resistant to nucleases, radiation, fixation, ether, dehydration |
|
|
Histologic features of prior disease
|
Spongiform encephaolopathy
Extensive neuronal loss Gliosis Kuru-like plaques White matter changes |
|
|
Histologic features of viral encephalitis
|
Lymphocytic infilitrate
Death of neurons/glia Viral inclusions Perivascular lymphocytic cuffs Microglial clumps for neuronophagia |
|
|
Direct CNS effects of HIV
|
AIDS dementia - HIV infection of microglia results in toxin production and neuron/oligo death
Vaculor myelopathy -- degeration of dorsal and lateral columns, similar to b12 deficiency |
|
|
HIV/AIDS immunosuppression effects on CNS
|
PML
Toxo Candida CMV Cryptococcus Herpes viruses Mycobacterium (TB and avium) CNS lymphomas |
|
|
Quality of exudate in bacteria meningitis
|
Pus tracking along vessels
Different based on organism: staph and pneumococcus - creamy, listeria is thin and watery |
|
|
Sequellae of treated bacteria meningitis
|
CN palsies
Hydrocephalus Mental retardation Seizures Learning disabilities |
|
|
Risk factors for brain abcesses
|
COPD
Heart defects |
|
|
Pathologic development of a brain abscess
|
Acute encephalitis with PMN and bacteria in edematous brain
PMNs liquify brain In about 10 days, loose fibrous capsule forms around and plasma cells appear Eventually mature abcess with thick fibrous capsule forms |
|
|
Treatment of abcess
|
Surgical excision
Cannot penetrate enough with antibiotics |
|
|
Daughter abscess process
|
As abscess matures, a daughter abscess extends closer to the ventricle
Acute ventriculitis = rapid death |
|
|
Presentation of brain abscess
|
As mass
Seizures, HA, hydrocephalus CSF will be clean, but really you shouldn't do one anyway, as this can precipitate herniation |
|
|
Neurosyph initial
|
Meningeal infection during secondary syph
Can progress in several ways |
|
|
Meningeal neurosyph
|
Diffuse lymphoplastic meningitis
Can result endarteritis obliterans vasculitis Fibrosis Obstruction of CSF flow |
|
|
Meningovascular neurosyph
|
Includes vessel thrombosis and cortical infarction
HAs and increased ICP |
|
|
Tabes dorsalis
|
Extension of syphilis into dorsal (sensory) root of spinal cord
Shoot pain and then loss of sensation |
|
|
Primary optic atrophy
|
Subacute degeneration of the optic nerves probably from extension of menigeal disease
Results in blidness |
|
|
Paretic neurosyphilis
General paresis of the insane |
Dementia and delusions of grandeur
Opacified meninges over frontal lobe, lymphocytic infiltrate, extensive corticalloss, rod cell microglia |
|
|
Gumma
|
Late consequence of syphilis
Small masses of epitheliod macrophages, fibroblasts, and chronic inflammatory cells |
|
|
CNS TB
2 things |
Subacute meningitis
Tuberculoma |
|
|
Tuberculous meningitis
|
Multiple, frequently confluent necrotizing granulomas of meninges
Often base of brain including over hypothalamus CN palsies, artery occlusions and infarcts Most often seen in kids |
|
|
Tuberculoma
|
Dense fibrotic mass of granulomas
Center is necrotic |
|
|
Pott's disease
|
TB of the vertebrae
Can cause spinal cord damage secondarily |
|
|
Fungal meningitises
|
Cryptococcus
Coccidiodes Blastomycosis Candida Basal meningitis |
|
|
Fungal encephalitises
|
Opportunistic, lethal
Aspergillus, mucormycosis, candida Spread into brain hematogenously, lodge in vessel wall and grow out |
|
|
Lesions of toxoplasmosis
|
Necrotizing and hemorrhagic
In deep structures of the brain |
|
|
Tachyzoite
|
free living infectious to CNS cell toxo particles
|
|
|
Post meningeal infarct
|
Type III hypersensitivity reaction
Blood vessel walls are damaged by immune complexes that are formed with dead bacteria as antigens |
|
|
Most common pathogen of bran abscesss
|
Streptococcus
|
|
|
Cogwheeling
|
Rigidity + tremor
Rigidity then give a little then rigidity then give a little |
|
|
Rigidity
|
Increase in resting muscle tone
|
|
|
Ballismus
|
Flinging, large amplitude movements
Usually of arm or leg |
|
|
Tics
|
Muscle movements or vocalizations that are compulsive and largely involuntary
Can sometimes be briefly suppressed |
|
|
Differential diagnosis for choreoathetosis
|
Very long
Common ones: Neurleptics Levodopa Huntington's diesease Senile Cerebral palsy |
|
|
Huntington's disease
Clinical triad |
Severe chorea, athetosis
Dementia --> vegetative state Behavior changes |
|
|
Huntington's disease
Genetics Onset |
AD, CAG repeats
Genetic anticipation (more repeats and worse disease) Onset 35-40, mean duration 13 years High incidence of suicide |
|
|
Huntington's symptoms worsen with administration of
|
Levodopa
|
|
|
Huntington's disease treatment
|
Neuroleptics that block dopamine
(halperidol, perphenazine) Neurotransmitter depleaters like reserpine (but depression) Genetic counseling Palliative care |
|
|
Essential tremor
Presentation |
Fine and irregular postural or intential tremor
Usually presents as young adult (also senile version) Worsens with age Usually upper limb, may be voice, head |
|
|
Essential tremor genetics
|
Sporadic
AD with incomplete penetrance |
|
|
Essential tremors may be temoporarily suppressed by>
|
EtOH
|
|
|
Essential tremor treatment
|
Beta blockers
Primidone Neurontin, topiramate Thalamotomy, deep brain stim |
|
|
Distinguishing between essential tremor and parkinsons
|
Parkinson's tremor is resting
Essential is postural or intentional Essential is often familial Parkinson's onset is unilateral, essential is symmetric Parkinson's is more rapidly progressive Parkinson's involves legs mroe |
|
|
Parkinsonism
2 of 3 to diagnosis |
Bradykinesia
Rigidity Tremor at rest |
|
|
Parkinsonism other symptoms
|
Masked facies
Hypophonia Micrographia Flexed posture Dysphagia Shuffling, hesitant, freezing gait |
|
|
Onset of parkinsonism
|
Insidious
Unilateral to bilateral |
|
|
Parkinson disease histology
|
Marked depigmentation of substantia nigra
Lewy body inclusions are hallmark (but can be found withouth diagnosis) |
|
|
Dopamine loss in Parkinson's disease
|
Correlates closely with degree of bradykinesia
Need to lose 70% of dopamine to have symptom 90% gone at death |
|
|
Risk factors of parkinson's disease
|
Age
Family history has some predisposition Perhaps toxic exposures: pesticides, herbicides, heavy metals, well water, industrial exposures |
|
|
Mortality in Parkinson's disease
|
15 years after diagnosis
Longer if not demented Longer with levodopa Cause: pulmonary infection, falls, PE, UTI Parkinson's survival is better than the other parkinsonian diseases |
|
|
Symptoms in Parkinson's
|
Progressive
Improved by l-dopa |
|
|
Genetics of parkinson's
|
Rare familal parkinsons
AD - synuclein mutation AR - parkin Environment seems to generally be more important |
|
|
Parkinsonism Classificatons
|
Idiopathic (85%)
Neuroleptic induced (7-9%) not always reversible Vascular - strokes in basal ganglia (3%) Multisystem atrophy (2.5%) Rare causes (toxins) Post encephalitis in past |
|
|
Parkinson plus syndromes
|
Lewy Body dementia
Progressive supranuclear palsy Multisystem atrophy |
|
|
When to think its parkinson's plus
|
Lack of tremor
Poor response to levodopa Symmetry or persistent assymetry Prominent autonomic dysfunction orthostatic hypotension, incontinence Early hallucinations, dementia Upper/lower motor neuron signs Frequent falls early Cerebellar signs |
|
|
Progressive supranuclear palsy radiology
|
Atrophy of midbrain
|
|
|
Dementia with Lewy bodies radiology
|
Relative sparing of hippocampi compared with AD
|
|
|
MSA - SND type radiology
|
Putamen ring on T2
|
|
|
MSA - OPCA type radiology
|
Infratentorial atrophy
|
|
|
MPTP toxicity
|
Just like parkinson's disease
MPTP converted to MPP+ by astrocytes Inhibits NADH-CoQ1 in mitochondria of dopaminergic neurons -- cell death |
|
|
Parkinson's disease treatment
|
Carbodopa/levodopa, other agonists
COMT inhibitors MAO-B inhibitors Anticholingergics because Ach opposes DA often Amantadine increases DA Pallidotomy, thalamotomy Deep brain stimulation |
|
|
DDx resting tremor
|
Parkinsons
Wilsons Mercury poisoning |
|
|
Ddx postural tremor
|
Anxiety, fatigue, physiologic
Essential tremor Lithium intoxication Thyrotoxicosis |
|
|
Dds intentional tremor
|
Multiple sclerosis
Cerebellar degeneration |
|
|
Tardive dyskenisa
|
Involuntary facial, buccal, lingual choreoathethotic movmements
Associated with long term neuroleptic use (protracted dopamine blockade) |
|
|
Syndenham's chorea
|
Autoimmune post-streptoccocal
|
|
|
Drug induced Parkinsonism common cause
|
Metoclopramide
|
|
|
Side effects of levodopa
|
Dyskinesias
Nightmares Hallucinations |
|
|
Alzheimer's prevalence
|
6% of over 65
10-20% of over 80 4 million Americans |
|
|
Alzheimer's disease genetic disease
|
Rarely autosomal dominant
40% ? familial with close relatives but no pattern 60% sporadic Down's related |
|
|
AD presentation
|
Memory loss (esp for recent events)
Loss of initiative Difficult with w/ word finding and calculations Confusion Behavior changes |
|
|
Alzheimer's diagnosis
|
Most a diagnosis of exclusion during life
|
|
|
AD gross pathology
|
Diffuse atrophy of cortex with some sparing of occiptal lobe
Increase in size of sulci and ventricles compared to age-matched controls |
|
|
AD histology
|
Increased numbers of:
Neurofibrillary tangles of tau Senile plaques of beta amyloid Most dense in hippocampus, basal nucleus of Meynert involved often |
|
|
Early onset AD genes
|
Presenillin 1 (chromosome 14)
Presenillin 2 (chromsome 1) Important in processing of B amyloid precursor protein 80% of familial AD APP mutations in B amyloid precursor protein |
|
|
Genetics of late onset AD
|
ApoE4 increases likihood
Many others |
|
|
Tau mutations
|
Lead to FTDP-17
Not Alzheimers |
|
|
Huntington's disease 3 clinical features
|
Dominant inheritance
Choreoasthetosis Dementia |
|
|
Progression of symptoms in Huntington's disease
|
Fidgeting --> grimacing, writhing, chorea
Changes in personality --> emotional liability, indifference, dementia |
|
|
Gross pathology of Huntington's disease
|
Striking loss of caudate and putamen
Secondary expansion of the anterior lateral ventricles May also have cortical (frontal, parietal) atrophy |
|
|
Location and function of Huntingon's disease gene
|
Chromosome 4
Fnc unknown |
|
|
Is sensation affect in Parkinson's disease?
|
No
|
|
|
Dementia rates in Parkinson's disease
|
30%
|
|
|
Gross pathology of Parkinson's disease
|
Loss of pigmentation of substantia nigra and locus ceruleus
Sometimes cerebral atropy |
|
|
Histology of Parkinson's disease
|
Dopaminergic neuronal degradation
Esp in substantia nigra, locus ceruleus, Can also see degeneration in dorsal raphe, basal nucleus meynert Lewy bodies increased in surviving neurons |
|
|
Lewy bodies contain
|
Alpha synuclein
|
|
|
Differentiating MSA from Parkinsons on histology
|
Marked cell loss and gliosis in putamen and caudate in addition to substantia nigra
|
|
|
What diseases are now called MSA
|
Striatonigral degeneration
Olivopontocerebellar atrophy Shy Drager |
|
|
Progressive Supranuclear palsy
|
PD + supranuclear opthalmoplegia
Cell loss, gliosis, tangle formation in substantia nigra, locus ceruleus, periaqueductal gray, tectum, others |
|
|
Parkinsons-Dementia-ALS complex of Guam
|
Widespread degeneration with neurofibrillary tangles
Parkinson's symptoms because SN and LC get tangles too |
|
|
Some drugs that induce Parkinson's
|
phenothiazines
butyrophenones reserpine |
|
|
Toxins that can induce Parkinson's
|
Manganese
CO carbon disulfide MPTP |
|
|
Genetics of familial parkinsons
|
Rare
Synuclein mutations Ubiquination mutations |
|
|
Gamma secretase actions
|
Cleaves amyloid precursor protein after alpha and beta
Lots of other stuff |
|
|
Immunization against beta amyloid protein
|
Could work
Can give encephalitis |
|
|
Clinical presentation of frontotemporal dementia
|
Disinhibition, lack of empathy, lack of self care, blunting, repetitive behaviors
Progressive non-fluent aphasia -problems with word comprehension, impairment of semantic memory Relative preservation of sensation, non-verbal porblem solving, autobiographical memory |
|
|
Tau
|
microtubule biding protein
heavily phosphorylated -- prediliction ot aggregate Different isoforms aggregate in different dementias |
|
|
Tauopathies
|
Mutation - FTDP-17
Sporadic (3R) - Pick's Sporadic (4R)- corticobasal degeneration, progressive supranuclear palsy, argylophilic granule disease Sporadic mixed tauopathies - AD |
|
|
Pick's disease
Presentation and histology |
Dementia with profound loss of neurons and gliosis of frontal lobe
Pick's cells - swollen neurons with Tau aggregates, Pick bodies |
|
|
Corticobasal degeneration
|
Frontotemporal tauopathy with neuronal loss, gliosis and tau aggregates
Presents as movement disorder in one arm --> alien limb and dementia |
|
|
Progressive supranuclear palsy histology
|
Globose tangles in basal ganglia and brain stem
|
|
|
Spinocerebellar atrophy
|
Progressive ataxia of trunk and extremities
Ataxic speech, nystagmus... Loss of reflexes Autosomal dominant, CAG repeat disease |
|
|
Freidrich's ataxia
|
Autosomal recessive
GAA intro repeat make frataxin inactive Frataxin is mitochondrial protein related to iron metabolism Loss of coordination/reflexes, degeneration of spinocerebellar, dorsal columns, cortex, cerebellum Presents preadolesence Cardiomyopathy also |
|
|
Shy Drager syndrome
|
Promiment autonomic failure
A multisystem atrophy |
|
|
Degeneration of ALS
|
Ventral nerve roots thin
Dorsal roots look okay - sensation preserved |
|
|
TDP-43-opathies
|
TDP-43 is a DNA binding protein probably important in RNA processing
Inclusions seen in ALS and frontal lobe dementia with ubiquinated inclusions Dementia a part of ALS? |
|
|
Lewy body dementia symptom?
|
Florid hallucinations without drugs
|
|
|
Brain death characteristics
|
Not awake
Not aware Not moving Not breathing No evoked responses, EEG is flat |
|
|
Pathophysiologic causes of brain death
|
Transtenorial herniation
Loss of intracranial blood flow Diffuse secondary loss of neurons |
|
|
When must brain tests be used over cardiopulmonary to declare death?
|
When ventilation is being used
|
|
|
Primary and secondary etiologies of brain death
|
Head trauma
Intracranial hemorrhage, esp subarachnoid Asphyxiation Cardiac arrest Secondary ICP --> herniation |
|
|
Neuroexam with brain death
|
No response to external stimuli
Cranial nerve reflexes lost Apnea (must test at PCO2 >60) Limb reflexes may persist, including Lazarus sign |
|
|
Lazarus sign
|
Lifting arms
Folding over chest Purely spinal so can be intact with brain death |
|
|
Diagnosing brain death
|
1. Clinical signs present
2. Serial exams 3. Clinical irreversibility -known structural lesion, not hypothermic, toxic Can confirm using brain blood flow test |
|
|
When to use confirmatory tests with determining brain death?
|
Full clinical exam cannot be performed
Speed up process for organ donation Inexperienced examiner Any question of validity of clinical exam |
|
|
Choices for confirming brain death
|
Electrophysiologic
EEG + BAER + SSER EEG- cortex/thalamus BAER/SSER - brain stem Brain blood flow Radiolabelled angiography Transcranial Doppler US SPECT MRA, MR perfusion |
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|
Vegetative state
|
Complete unawareness of self/environment
Sleep wake cycle intact Preservation of brain stem, hypothalamic, autonomic fncs Lasting >1 month |
|
|
Diagnosing vegetative status
|
I: No awareness, No voluntary response to visual, auditory, tactile, noxious stimuli, No language
II: Sleep/wake, CN reflexes, autonomic fncs intact enough to survive without care for extended periods |
|
|
Vegetative state, what does the patient do?
|
Sleep, wake
Yawn Breathe Make sounds but not words Blink and move eyes but don't look/track Grimace Chewing movements Move limbs Startle myoclonus |
|
|
Vegetative state pathology
|
Cortex, thalamus, their connection damaged
Traumatic -- diffuse axonal injury Non traumatic -- diffuse laminar cortical necrosis Thalamus is typically quite involved |
|
|
What should be on differential diagnosis when considering persistant vegetative state?
|
Minimally conscious state where some awareness does exist
|
|
|
What might a PET show in vegetative state?
|
Decreased glucose uptake in region
Auditory/visual responses are limited to primary cortical regions -- not reaching association regions Disconnection of frontoparietal cortices from thalamus |
|
|
fMRI in vegetative state
|
No functioning thalamocortical networks
fMRI and EEG do not have normal homeostatic coupling |
|
|
Vegetative state prognosis
|
Not good if it persists
<1% recovery from nontraumatic VS after 3 months <1% recovery from traumatic VS after a year 70% mortality at 3 years, 85% at 5 years |
|
|
Vegetative state legally
|
Alive in all jurisdictions
Patient/proxies have right to refuse supportive care (including hydration, feeding) |
|
|
Vegetative state treatment
|
Supportive
Trach/PEG needed Nothing proven to shorten or get patients out of VS |
|
|
Minimally conscious state
|
Altered consciousness
Minimal but behaviorally evident |
|
|
Minimally conscious state behaviors
|
Follow simple commands
Gesture yes/no Intelligible verbalization Reach, touch objects Sustain visual pursuit Cry, smile appropriately |
|
|
Minimally conscious state
Prognosis, treatment |
Difficult to predict prognosis
Treat with neurorehab levodopa/dopamine agonists SSRIs, stimulants, zolpidem DMS to intralaminar nucleus expt |
|
|
Minimally conscious vs Vegatative state?
|
Behaviors is the key difference
fMRI to see brain responses even without physical behaviors |
|
|
External ear is set to resonate at
|
2000-5000 Hz
Amplifying human language best |
|
|
Middle ear purpose
|
Ampliflication
Hydraulic effect of tympanic membrane to oval window -- 17x Lever effect of malleus/inkus -- 1.3x Total -- 22x |
|
|
What is oval window connected to
|
Scala tympani
which connects to Scala vestibuli via helicotrema which connects to round window |
|
|
Endolymph
|
In scala media
High in potassium from pumping in via gap jncts in the spiral lamina, connexin |
|
|
Hair cells
|
On basilar membrane
Inner ---contact the vestibular membrane --afferent Outer --connected to the vestibular membrane -- efferent |
|
|
Tonotopy of colchlea
|
Higher frequency near base
-where tympanic membrane is tighter Lower frequency near helicotrema -where tympanic membrane is looser |
|
|
How is tuning accomplished
|
Tonotopy
+ lateral inhibition via efferents |
|
|
Auditory afferent pathway
|
Spiral ganglia
Cochlear nerve Cochlear nucleus Superior olivary complex Nuclei of lateral lemniscus Inferior colliculus Medial geniculate Primary auditory cortex |
|
|
Role of superior olivary complex in hearing
|
Bilateral innervation
Sound localization Relays to inferior colliculus Efferents originate |
|
|
Role of inferior colliculus in sound processing
|
Input from superior olive
Contralateral cochlear nucleus Integrates spectral, temporal aspects Encodes combinations of frequencies Obligatory relay |
|
|
Medial geniculate in hearing
|
Slightly more responsive to contralateral
Necessary for processing sounds |
|
|
How to measure central pathways of hearing?
|
Brain stem auditory evoked response
Give stimulus many times, that way background fades away |
|
|
Right vs left hemisphere auditory processing
|
left
better temporal resolution speech sounds right better spectral resolution tones |
|
|
Control of the ear muscles
|
Cochlear nucleus to trigem/facial nuclei
|
|
|
Conductive hearing loss
|
Air hearing down, bone okay
Infections, tumor, trauma, cyst, congenital |
|
|
Sensory hearing loss
|
Reduced hearing through bone and air
Age, hereditary, congenital |
|
|
Cerumen impaction
Tuning fork, audiograpm, tympanogram |
Fork - air loss
Audiogram -- conductive loss Tympanogram -- occlusion |
|
|
Otitis media
Exam, audiogram, tympanogram |
Exam -- fluid in middle ear
Fork -- BC>AC Audiogram -- conduction loss Tympanogram -- flat, no movement |
|
|
Damage to middle ear bones
Exam, tuning fork, audiogram, tympanogram |
Exam - wnl
tuning for BC>AC Audiogram - conductive loss Tympanogram -- wnl |
|
|
Cholestetoma define
|
Retraction of tympanic membrane by eustachian tube
Ingrowth of keratinzing epi Bone destruction and infection |
|
|
Otosclerosis
Exam, audiogram, tympanogram |
Congenital fixation of ossicles
Exam - normal appearance, absent auditory reflexes Audiogram - conductive hearing loss Tympanogram -- normal |
|
|
Noise/age related hearing loss
--hair cell damage exam, normal, tuning fork, audiogram |
Exam - normal
Tympanogram - normal Fork AC>BC Audiogram -- high frequency loss of bone and air |
|
|
Meniere's disease
Fork, audiogram |
Fork -- AC>BC
Low frequency fluctuating sensory hearing loss |
|
|
Acoustic neuroma
Fork, audiogram |
Fork - AC>BC
Audiogram -- sensory neuronal hearing loss Evoked reflex abnormal |
|
|
Temporal lobe lesion
Exam, tympanogram, fork, audiogram |
Everything is normal
Fork AC>BC Poor speech understanding |
|
|
Superior semicircular canal fistula
|
Inner ear conductive hearing loss
Exam - wnl Tympanogram -- vertigo Fork -- BC> AC Audio - conductive hearing loss "third window" |
|
|
Ventral tegmental area projections
|
Dopaminergic projections to medial forebrain bundle
Medial forebrain bundle projects GABA onto nucleus accumbens Medial prefrontal cortex and amydala also |
|
|
Where will rats self inject cocaine/amphetamines?
nicotine/opiods? |
Cocaine/amphetamines -- nucleus accumbens and ventral pallidium
Nicotine/opiods - ventral tegmental area |
|
|
What blocks the rewards of cocaine/amphetamines?
|
Dopamine blockers
|
|
|
Withdrawal why?
|
Changes in receptor density/specificity
Changes in second messenger levels/phosphorylation status Increased hepatic metabolism |
|
|
Autonomic changes with opiod use
|
Pupillary constriction
Dry mouth Diaphoresis Itching Respiratory suppression Cough suppression Constipation Urinary retention Biliary tract spasm |
|
|
Weird bad complication of opiod OD/naloxone use
|
Non cardiac pulmonary edema
|
|
|
Treatment of combo benzo/opiod OD
|
Flumazenil
Naloxone -- may need to redose |
|
|
Acute treatment of opiod withdrawal
|
supportive, fluids
autonomic stabilization with alpha blockers (clonidine, guanfacine) |
|
|
Opiod medical complications
|
Heroin - cardiac arrythmias, neuropathy (autoimmune?)
Sudden pulmonary edema Immunosuppression (down in Ts) Infections -including neuro, osteomyletis Stroke |
|
|
Chasing the dragon
|
Inhaling smoke from opiod heated on tinfoil
Severe Leukencephalopathy is a rare complication --- may be treated with Coenzyme Q --- ?mitochondrial dysfunction |
|
|
Amphetamine action
|
Dopamine reuptake blocker
Long term potentiation enhanced Changes in receptors -- dyskinetic SEs, toelrance |
|
|
Ampethamine medical uses
|
Narcolepsy - symptomatic relief
ADHD - improves concentration Depression - esp useful in elderly |
|
|
Amphetamine OD
|
Excitement, diaphrosis, pupil dilation, HTN, tachycardia
Psychosis Seizures, pulmonary edema, hyperthermia, muscle necrosis |
|
|
Amphetamine OD treatment
|
Fluids + diuresis
Labetalol for to slow HR and lower BP Sedation with benzos |
|
|
Amphetamine withdrawal
|
Fatigue, depression, somulance
Rebound hunger Craving for months, but not usually years (like opiod users) |
|
|
Amphetamine medical complications
|
MI -- HTN/vasospasm or accelerated atherosclerosis
Liver toxicity with elevated enzymes Infections Vasculitis Rare stroke |
|
|
Amphetamine and sensitization/tolerance
|
Tolerance - euphoria, anorexia, hyperthermia
Sensitization - pyschosis and movement disorders |
|
|
Amphetamines and movement disorders
|
Adults -- dystonia, choreathetosis, tremor
Typically subside rapidly, but may last for years Kids -- potentiate tics |
|
|
What's different about the pyschosis of amphetamines and schizophrenia
|
Lack of negative symptoms
|
|
|
Cocaine mechanism
|
Reuptake blocker for dopamine, NE, 5HT
|
|
|
Cocaine effects
|
Euphoria and excitement
Mild autonomic activation Sterotypic movements Psychosis Illusion of improved memory/mental fnc |
|
|
Cocaine OD
|
HTN
Seizures Agitation/coma Acidosis Rhabdomylitis |
|
|
Cocaine withdrawal
|
Craving, fatigue, restlessness
Depression Craving lasts forever |
|
|
Cocaine medical complications
|
Chest pain, coronary vasospasm, MI, cardiomyopathy
Systemic vasculopathy Rhabodomyelitis, interstitial nephritis, hepatotoxicity Infections Destruction of nasal septum, palate Trauma Cerebral infarction, hemorrhage Cerebral atrophy |
|
|
Cocaine and obstetrics
|
Increases risk of most prenatal complications
Generally poor prenatal care Increased risk of most birth defect Neonates are irritable, tremor, seizures |
|
|
THC mechanism
|
Binds to CB1 and CB2 in hippocampus, basal ganglia, brainstem
G protein/cAMP mediated Decreased neuronal firing by altered K and Ca curretns |
|
|
Cannabis effects
|
Thirst, decreased salivation, increasd appetite, tachycardia, hypertension, conjunctival injection
Initial anxiety --> dreamy euphoria Symptoms of psychosis -- illusion of profundity of normal objects Cognitive slowing |
|
|
THC effects over time
|
Hallucination, depersonalization, altered perception of time
Stupor, hypotension Sensitization to high? Withdrawal with yawning, tremor, diaphoresis |
|
|
Pathologic intoxication with alcohol
|
Rage, anger, destructive behavior
Followed by sleep/amnesia Not necessarily dose related |
|
|
Delirium tremens
|
Peaks about 96 hours into withdrawal
Confusion, disorientation, agitation, hallucination, restlesssness Autonomic activation -mydriasis, tachycardia, diaphoresis --->death Treated in ICU w/ benzos 2% death rate |
|
|
Alcohol tobacco ambylopia
|
Subacute onset of optic neuropathy
Blurred vision Central scotmata Treated with vitamins |
|
|
Alcohol brain damage
|
Cerebellar atrophy and ataxia
incomplete response to abstinence Alcoholic dementia? Neuronal toxicity? -J shaped effect on dementia FAS |
|
|
Hepatic encephalopathy
|
With liver failure, increased ammonium because not converting to urea
Ammonium is toxic Ammonium is converted to glutamine Glutamine toxicity GABA increased as well Drowsiness--delirium -- coma |
|
|
Hallucinogen mechanisms
|
Indoles -- serotonin like
Alkyl amine -- amphetamine like All activated 5-HT2 Some also have dopaminergic effect |
|
|
Effects of hallucinogens
|
Eurphoria, anxiety, hallucinations
Mystical insight perceived Intense depression, paranoia, panic Flashbacks |
|
|
Hallucinogen treatment
|
SSRIs, benzos
Not TCAs, neuroleptics |
|
|
PCP intoxication
|
Wide range
Euphoria, agitation, psychosis, pain-less self injury, violence with superhuman strength Hyperthermia, stroke, cerebral hemorrhage |
|
|
PCP chronic use
|
Associated with schizophrenia that can be treated with neuroleptics
|
|
|
Inhaled organic solvents
|
Similar effects to alcohol
|
|
|
Heteroplasmy
|
Different types of mtDNA found in a person
Can even be different types in a mitochondria |
|
|
Mitochondrial nature of genetic disease affects them how?
|
Random segregation of heteroplasmic mitochondria into new cells
Different distributions of mutant proteins Threshold effect reached for pathology Different for different tissues based on energy needs |
|
|
Disease mitochondria produce what symptoms
|
Muscular weakness, fatigue
Seizures, stroke, dementia Cardiac conduction problems Hearing loss Retinopathy Pancytopenia Episodic N/V Fatigue ....lots |
|
|
Mitochondrial encephalomyopathies
|
Electron transport chain disorders
Can arise in either mtDNA (25%) or nuclear DNA (75%) Brain, skeletal muscle +/- other organs |
|
|
MELAS
|
Mitochondrial Encephalomyopathy Lactic Acidosis Stroke-like episodes
Movement disorder with stroke like episodes and memory loss point mutation in leu tRNA gene of mitochondrial DNA |
|
|
MERRF
|
Myclonic epilepsy with ragged red fibers
point mutation in mtDNA of lys tRNA |
|
|
NARP
|
Neuropathy
Ataxia Retinitis pigmentosa Materally inherited Leigh syndrome Proximal muscle weakness, ataxia, developmental delay, seizures, dementia, retinitis pigmentosa Pt mutation in mtDNA ATPase 6 |
|
|
Treatment of mitochondrial disorders
|
Palliate symptoms
Administer vitamins, cofactors, oxygen radical scavengers Experimental including gene therapy, Cu supplements for cytochrome c oxidase deficiency |
|
|
Genetic counseling in diseases with mitochondrial inheritance
|
Predictive testing/prenatal testing unreliable
Test symptomatic family members |
|
|
Monitoring in people at risk for a disease of the mitochondria
|
Monitor cardiac health
Extensive neuro exams Kidney, liver fnc Screen for diabetes, hypothyroidism |
|
|
Kearns-Sayre syndrome
|
Mitochondrial inheritance
<20 onset of Opthalmoplegia, atypical retinitis pigmentosum, cardiac conduction defect, cerebellar syndrome, CSF protein elevation >100 |
|
|
Pearson syndrome
|
Mitochondrially inherited
Severe, transfusion dependent macrocytic anemia with some neutropenia, thrombocytopenia |
|
|
mtDNA and mutations
|
10x more than nuclear DNA
No histones, repair mechanisms Exposed to free radicals made during oxidative phosphorylation All introns -- all mutations expressed |
|
|
Frequent lab findings with mitochondrial dysfunction
|
Lactic acidosis
Carnitine increased |
|
|
Types of infarction in terms of etiology
|
Thrombotic
Embolic Hypotensive/hyopvolemia Anoxia Venous |
|
|
Causes of CNS infarction
|
Atherosclerosis
Embolism Vasculitis Swelling/edema (leading to hyperperfusion) |
|
|
Where to atheromatous plaques develop that end up causing strokes
|
Aortic arches
ICAs Basilar's Circle of willis |
|
|
Modifiable risk factors for cerebral infarction
|
Afib
HTN Isolated systolic HTN MI/heart disease Diabetes TIAs Smoking |
|
|
Mechanisms of atherosclerotic plaques causing cerebral infarcts
|
Diminish flow below threshold
Thrombus over plaque Hemorrhage into plaque Atheroembolic |
|
|
Evolution of gross brain pathology with stroke
|
Massive edema from 3-7 days
7 days - beginning of liquifactive necrosis By 3-4 weeks extensive brain loss One month -- gliotic scar Years -- cystic cavity |
|
|
Granular atrophy of the cortex
|
Consquence of microemboli
|
|
|
Lacunar infarcts
|
HTN related often
|
|
|
Which CNS areas are sensitive to ischemia?
Resistant |
Hippocampus, cerebellar Purkinje cells, some layers of cortex
Brain stem motor nuceli, pontine neurons pretty resistant |
|
|
Which CNS cells are sensitive to ischemia
|
Neurons
Oligos Astrocytes Endothelial Microglia |
|
|
What develops histologically after ischemia to brain?
|
6-8 hours later you see red neurons
|
|
|
Evolution of cerebral infarct histologically
|
0-6 hours -nothing
6-12 hours -red neurons 12-24 - endothelial rx, some edema, first PMNs 24-36 - PMNs abundant around border, vessels 36-72 - macros replaces PMNs 72-months -- macros eat brain 7-9 days - astrocytes make gliotic scar |
|
|
Embolic strokes tend to be .....
Why? |
Hemorrhagic
Thrombus breaks down eventually returning the flow of blood into dead tissue -- hemorrhage |
|
|
What kind of damage to brain can be seen after resuscitated cardiac arrest?
|
Laminar necrosis of cortex
Can lead to vegetative state |
|
|
Boundary zone or watershed necrosis
|
With drop in perfusion pressure areas at edges of distribution become anoxic
Often has an iatrogenic cause |
|
|
Venous infarctions of CNS
|
Rare
Bad Blood comes in at arterial pressure but cannot get out = bleeding into brain and infarction |
|
|
Most likely place to get a cerebral hemorrhage with HTN?
|
Putamen
also other basal ganglia, cerebrellar white matter, basal pons |
|
|
What happens with large basal pons hemorrhage
|
fall over dead
|
|
|
Changes to cerebral vessels from HTN?
|
Charcot-Bouchard aneurysms
In small vessels coming off big vessesl at right angles |
|
|
Germinal matrix hemorrhage
|
Typically seen in premature infants
Germinal matrix is source of neurblasts Lies over basal ganglia in lateral ventricle |
|
|
Berry aneurysms
|
Congenital aneurysms
Commonly seen in Circle of Willis at areas of fusion (these have no media) --Walls are just fibroblasts and endothelium Predispose to subarachnoid hemorrhage Intervention needed when >5mm |
|
|
Vascular malformations of brain
|
Telangectasias -- small dilated vessels, clinically not important
Cavernous hemangiomas AVMS |
|
|
Cavernous hemangiomas of brain
|
Large closely packed channels of blood separated by collagen within one endothelium
Associated with recurrent bleeds and seziures |
|
|
AVMs of brain
|
Typically in leptomeninges
Arteries and arterialized veins with large vessels leading to malformation Can cause ischemia Can cause fatal hemorrhage |
|
|
Amyloidosis of cerebral blood vessels
|
Beta amyloid deposited in walls of small cerebral blood vessels
Hemorrhage in cortex, superficial white matter Associated with age, AD |
|
|
Classification of spinal muscular atrophy
|
Type 1 - werdnig-hoffman
infantile weakness, death by2 Type 2 sit but do not walk Type 3 (kugelberg-welander) walk but don't run Severity is about how much compensation you can get from SMN2 |
|
|
Charcot-Marie-Tooth
|
Inherited neuropathy
Deficiency is in myelin packing protein -- body breaks down myelin, remyelination Progressive distal weakness with foot drop |
|
|
Babies of mom's with MG
|
Transient mystenia problems
Need support of airway for a couple of weeks IgGs have crossed the placenta |
|
|
Congenital MG
|
Not autoimmune
Some structural problem with NMJ Sometimes do respond to anticholinesteraces |
|
|
Dystrophic muscle?
|
Fibrosis
Inflammation Atrophic fibers Rounding of fibers |
|
|
Congenital muscular dystrophy
|
Problem is with lamnin attenae sticking out of dystrophin complex
Weakness, feeding problems, failure to thrive, sit at 3, never walk Significant brain changes -leukodystrophy, cortical abnormalities/epilepsy |
|
|
Infantile spasms
|
Epilepsy seen in 4 mos - 18 mo
(mostly <1) Generalized -- flexion, extension, head drop West- + chaotic EEG, developmental dely High associated with MR |
|
|
Infantile spasms underlying etiologies
|
Increased CRH receptor expression?
PKU, tuberous sclerosis, down, pyridoxine dependency, aicari |
|
|
Treatment of infantile spasms?
|
ACTH/prednisone
|
|
|
Febrile seizures
|
Children, peak is 16-18 months
Not considered epilepsy as they are provoked If complicated (last longer, generalized) more likely to predict an upcoming epilepsy |
|
|
Common presentation of absence seizures
|
School failure and ?ADHD
|
|
|
Benign Rolandic epilepsy
|
AD, incomplete penetrance
Resolve by 16 Typically simple, brought on by sleep Noted increase in learning disabilities |
|
|
Focal cortical dysplasia
|
Neuro/glial migration in development not perfect. get weird differentiation
Creates seizure focus or connections to it create a seizure focus |
|
|
Neurofibromatosis I
|
Peripheral type
Mutation in tumor suppressor neurofibromas, peripheral nerve tomorrows, optic gliomas Associated with leukemia, pheo, wilms, neuroblastoma |
|
|
How are most diagnoses of NFI made
|
6 or more cafe au lait spots and a first degree relative
|
|
|
Plexiform neuromas
|
Larger tumors seen in NF1
|
|
|
Appearance of pt with NF1
|
Short
Macrocephaly Scoliosis Can have dural ectasia 40% of LD, a few MR |
|
|
Risks in NF1
|
Tumors
Seizures Stroke |
|
|
NF2
|
AD, less common, central
Bilateral acoustic neuromas Loss of contact mediated growth inhibitor Associated with gliomas, meningiomas, other CNS tumors |
|
|
Tuberous sclerosis
|
AR
Mental retardation, seizures, ash leaf spots, orange peel skin, brain tumors Tubers - hartomatous lesions of gliotic tissue, astrocytes, cortex, calcification |
|
|
Kids with rhabdomyoma of heart?
|
50% chance of having tuberous sclerosis
|
|
|
Sturge Weber
|
Venous angioma of pia and portwine stain of V1
Seizures, hemispheric brain atrophy Progressive hemiplegia Hemianopsia |
|
|
Kids with tuberous sclerosis and typical intelligence
|
1/3
|
|
|
Sturge Weber is an indication for..
|
Hemispherectomy because of catastrophic infantile seizures
|
