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147 Cards in this Set

  • Front
  • Back
What does an UMN lesion indicate?
involvement of the corticospinal tract
What does a LMN lesion indicate?
involvement of motor neurons in brainstem or spinal cord
What does a Parkinsonism lesion indicate?
involvemnet of nigrostriatal tracts
What has Bradykinesis, rigidity, tremor at rest, postural inability?
What has right/left confusion, agraphia, acalculia, finger agnosia?
Gerstmann syndrome
What does a lesion of Gerstmann syndrome indicate?
involvement of left angular gyrus (dominant parietal lobe)
What has ataxia of gait, oculomotor abnormalities, and acute confusional state?
Wernicke’s encephalopathy (due to thiamine deficiency)
What has ataxia of gait, incontinence, dementia?
normal-pressure hydrocephalus
What has ataxia of gait, lightning-like pains, incontinence, loss of proprioception and vibration in legs, positive Romberg sign, areflexia, Charcot joints, Argyll Robertson pupils?
Tabes dorsalis
Muscle weakness can be the result of dysfunction of?
Upper motor neurons
Lower motor neurons
Peripheral nerves
Neuromuscular junctions
Basal ganglia dysfunction causes what?
Movement disorders, rigidity
Cerebellar dysfunction causes what?
incoordination, ataxia, dysmetria, dysdadochokineasia, hypotonia
What is most commonly involved if proximal weakness is greater than distal weakness?
muscle involvement
What do you have with proximal weakness being greater than distal weakness, normal tone, alittle bit of atrophy, no fasciculations, normal reflexes but decreased late, and no sensory loss?
muscle disorders
What do you have with fluctuating weakness, especially increasing weakness with exertion is most characteristic sign,
Proximal > distal, and have Ocular and Bulbar weakness,
normal tone and reflexes,
No atrophy or fasciculations,
No sensory loss?
neuromuscular junction disorder
Pain or sensory loss in dermatome root, normal to decreased tone,
Reflexes diminished to absent in involved areas,
Atrophy in denervated muscles,
Fasciculations not usually visible, but are seen on electromyography?
Peripheral neuropathy or ridiculopathy
Lesions in the posterior limb of the internal capsule cause?
weakness of the contralateral face, arm, and leg
Lesions in the somatosensory radiations or thalamus cause?
sensory loss in contralateral face, arm, and leg
What can a pituitary tumor cause?
Bitemporal hemianopia
What causes Cranial nerve dysfunction,
Crossed weakness--> ipsilateral cranial nerve palsy with contralateral hemiparesis,
Loss of pain and temperature on ipsilateral face and contralateral body (spinal tract of V + spinothalamic tract),
Loss of sensory modalities of one sensory pathway but not the other (can also occur in spinal cord), i.e., medial lemniscus and/or spinothalamic tract involvement,
Some gaze palsies?
Brainstem lesion
What caues miosis, ptosis, and anhydrosis?
Horner's Syndrome --> Can be seen if spinal cord lesion involves the intermediolateral column at T1-T2
What is a motor unit?
Lower motor neuron--> in anterior horn of spinal cord, OR in motor nucleus of brain stem
Axon of this neuron
All the muscle fibers innervated by that neuron
What type of muscle fiber does sustained force/weight bearing, is slow twitch, red, has many mitochondria, dark stain for NADH-TR, abundant lipids and scant glycogen?
Type 1
What muscle type does sudden movements, purposeful motion, is fast twitch, white, few mitochondria, light staining for NADH-TR, scant lipids but lots of glycogen?
Type 2
What is used to diagnose Vasculitis
Inflammatory neuropathies
Hypertrophic neuropathies?
Nerve biopsy
What develops slowly, is the most common cause of neuropathy, causes distal symmetric polyneuropathy, and most commonly diffuse distal symmetric polyneuropathy because of axonal degeneration?
Diabetic neuropathy
When are pyknotic nuclear clumps seen?
denervation atrophy
What is xlinked recessive on 21, causes calf pseudohypertrophy, increased serum CK, walking delayed, Gower's sign (weak pelvic girdle), respiratory difficulty ,and absence of dystrophin?
Duchenne muscular dystrophy
What is autosomal dominant, has CTG repeats, is a multisystem disease with myotonia, cadiomyopathy, ocular cataracts, endocrinopathy, ptosis, facial weakness, balding, and myotonia which is impaired relaxation and EMG wtih waxing and wanning high frequency discharge that continues after relaxation begins?
Myotonic muscular dystrophy
What is due to deficiencies in carnitine, acyl-CoA dehydrogenase, or carnitine palmitoyltransferase (CPT).
Lipid storage myopathy
What causes a subacute onset of weakness with proximal muscle weakness including neck, no rash, muscle bx important for diagnosis, responds to steroids and immunosuppressive therapy, and on pathology has a mononuclear cell infiltrate of CD8 positive cytotoxic T cells and macrophages?
What has a rash, more severe weakness, about 10 percent occuring after 40 are associated with cancer, skin changes include lilac heliotrope discoloration of eyelids, periorbital edema, and scaly red patches with perifascular atrophy and lymphocytes are b cells and cd4 positive t cells and capillaries are attacked by antibodies and complement with necrosis in the muscle?
What has no rash, does NOT respond to steriods, more likely to effect distal muscles, usually in men over 50, has rimmed vacuoles?
Inclusion body myositis
What is a type 2 fiber atrophy that shows clinical improvement with cholinergic drugs, ocular muscles usually involved, antibodies to acetylcholine receptors?
Myasthenia gravis
What is an infantile spinal muscular atrophy type 1 with defect on chromosome 5, autosomal recessive, and have muscule biopsy to confirm diagnosis and panfasicular atrophy?
Werdnig-Hoffmann disease
What is the most frequent acquired demyelinating neuropathy, with weakness that may be initally worse in proximal muscles, ascending paralysis, protein intially normal but rises, slowed nerve conduction velocity, damage to axons is characteristic histologically?
Guillian Barre syndrome or acute inflammatory demyelinating polyradiculoneuropathy
What is occluded if facial pulses are increased?
ICA before the ophthalmic artery branch point
What is supplied by the superior division of the MCA in the dominant hemisphere?
Brocca's area
What is supplied by the inferior division of the MCA in the dominant hemisphere?
Wernicke's area
What is occlusion of the inferior division of the MCA almost always due to?
What causes left hemiparesis, left homonymous hemianopia, right gaze preference, neglect, cortical sensory loss?
Lesion in the right frontoparietal lobe with Right MCA caused by thrombosis of the internal carotid artery due to atherosclerosis (because of history of amaurosis fugax)
What is due to retinal ischemia is often correlated with ipsilateral high grade carotid stenosis / thrombosis
Ophthalmic artery, first intracranial branch of the internal carotid artery
Transient Monocular Blindness or amaurosis fugax
What presents with Left hemiparesis: leg > arm > face
Left grasp reflex and motor impersistence
Left “alien hand syndrome”
Suggestion of cortical sensory loss
Clinical localization to the right primary motor cortex foot area supplemental motor area, and other adjacent regions of the right frontal or parietal lobes from an infarct of the right ACA
Alein hand syndrome is seen in what region of the brain?
supplementary motor area
What caueses Right hemiparesis (face & arm > leg)
Broca’s aphasia
Left gaze preference
Horner’s syndrome
L-MCA territory, superior division
How can a lesion in the L-MCA territory be related to Horner’s syndrome?
Dissection of the left internal carotid artery  thrombosis of the artery  Thromboembolism from L-ICA goes to L-MCA. The dissection also disrupts the sympathetic tract in the superior cervical ganglion -->Horner’s syndrome
What may result from an injury of the carotid in the neck?
Horner's syndrome
What causes Right hemiparesis involving face, arm, and leg equally
Mild dysarthria, but no aphasia
No somatosensory or visual field deficits
“Pure motor hemiparesis” syndrome
Lacunar infarcts in the posterior limb of the internal capsule causing a pure motor hemiparesis are usually due to occlusion of a lenticulostriate artery, or (less common) the anterior choroidal artery.
Location is commonly the posterior limb of the internal capsule

a lacunar infarction due to atherosclorsis caused this stroke
What supplies the basal ganglia?
anterior circulation
What can cause dizziness, diplopia, fuzzy vision, left hand cool and aching, loud bruit in left supraclavicular region, high pitched bruit at right carotid bifurcation?
vertebrobasilar ischemia
What is subclavian steal syndrome?
If there is an occlusion of subclavian artery proximal to origin of VA (at point “e” in the diagram), when the arm is exercised (thus increasing demand for blood flow to the arm), blood may be shunted away from the brain (along path indicated in diagram) resulting in vertebrobasilar symptoms
What causes Lethargy
Rightward gaze preference with inability to move eye past midline (i.e., can’t look left)
Right hemiparesis (UMN facial weakness, arm, and leg)?
infarct in the left pons involving the left corticospinal and corticobulbar fibers as well as the left abducens nucleus or PPRF
What is the Location of Lesions Causing Alerted Level of Alertness?
Upper brainstem reticular formation (purple area in figure)
Extensive bilateral involvement of cerebral cortex
Bilateral thalamus (medial and intralaminar nuclei)
Wrong way eyes can be caused by what three things?
1) ongoing seizure activity in one of hte cerebral hemispheres
2) lesions in the region of hte thalami which causes coma
3) lesions in the pons affecting the corticospinal tract (contralateral hemiparesis) and abducens nucleus (ipsilateral gaze preference)
What is subclavian steal syndrome?
If there is an occlusion of subclavian artery proximal to origin of VA (at point “e” in the diagram), when the arm is exercised (thus increasing demand for blood flow to the arm), blood may be shunted away from the brain (along path indicated in diagram) resulting in vertebrobasilar symptoms
What causes Lethargy
Rightward gaze preference with inability to move eye past midline (i.e., can’t look left)
Right hemiparesis (UMN facial weakness, arm, and leg)?
infarct in the left pons involving the left corticospinal and corticobulbar fibers as well as the left abducens nucleus or PPRF
What is the Location of Lesions Causing Alerted Level of Alertness?
Upper brainstem reticular formation (purple area in figure)
Extensive bilateral involvement of cerebral cortex
Bilateral thalamus (medial and intralaminar nuclei)
Wrong way eyes can be caused by what three things?
1) ongoing seizure activity in one of hte cerebral hemispheres
2) lesions in the region of hte thalami which causes coma
3) lesions in the pons affecting the corticospinal tract (contralateral hemiparesis) and abducens nucleus (ipsilateral gaze preference)
What disease of young adults has Multiple areas of demyelination
“Lesions separated in space and time”
Immune-mediated but reaction is modulated by:
Environmental factors
Genetic susceptibility, Multiple unlinked genes? Heat can exacerbate this disease
Multiple Sclerosis
What has CSF WBC is normal or slightly increased
2) Protein may be mildly elevated but is usually < 100 mg/dl.
3) Glucose is normal.
4) Oligoclonal bands represent IgGs synthesized intrathecally.
5) Myelin basic protein, a component of myelin, may be present
What has Episodes of exacerbations and remissions during which not
all symptoms resolve completely. The patient may be left with
permanent disability which may vary in severity. relapses are
often more severe than in the previous group. Relapses also
become more severe with time.
Relapsing remitting multiple sclerosis
What has an acute stage with
CD4+ TH1 that are activated by unknown antigenic stimulus
Immune response is polyclonal
These cell secrete toxic cytokines like TFN-γ
Lesions contain CD4+ > CD8+ cells, and macrophages
Antibody-secreting B-cells are also activated  “oligoclonal” response
What has Perivascular lymphocytes
Lymphocytes: helper-inducer T-cells
IL-2 receptors on many T-cells
Astrocytes express class II molecules (not seen in normal astrocytes)
May be involved in antigen presentation to T-cells
Tumor necrosis factor (TNF) toxic to oligodendroglia and myelin
Levels of TNF in CSF
What has well-demarcated WM lesions called “plaques”, often found in a periventricular location in the brain. Demyelination is accompanied by astrocytosis. Oligodendrocytes are decreased. Myelinated axons are seen as blue strands in normal area.
Chronic (relapsing remitting) MS
What acute disease exhibits more active inflammation that chronic disease. The lesions exhibit more readily visible contrast enhancement. Because of the inflammation and edema, these lesions can exert mass effect; this is sometimes called “tumefactive”.
Acute MS
When do you see Balo’s Concentric Sclerosis?
Acute MS
A variant of MS characterized by optic neuritis (usually bilateral) and myelitis, often occurring simultaneously or in close succession. Spinal cord lesion is often severe and necrotizing
Neuromyelitis optica (Devic’s syndrome)
Resembles acute MS

Monophasic illness

Rapidly progressive neuro sx develop 1-2 wks later: headache, lethargy, coma

Fatal up to 20% of cases
perivenous lymphocytes and macrophages in an area of acute demyelination.
ADEM Acute disseminated encephalomyelitis
Hyperacute form of ADEM

Usually follows respiratory
Infection, e.g., Mycoplasma

Usually fatal

Necrosis of small vessels and
hemorrhage into surrounding
Acute hemorrhagic leukoencephalitis
Name the infectious agents associated with
Progressive multifocal leukoencephalopathy (PML);
Subacute sclerosing panencephalitis (SSPE)?
PML--> Papova virus (JC virus)infects oligodendrocytes causing demyelination

SSPE--> rare progressive disease characterized by dementia, spasticity, and seizures related to measles at an early age; with measles vaccination this has largely disappeared
What has bizzare astrocytes?
What is the clinical state usually associated with central pontine myelinolysis (CPM).
Usually due to rapid correction of hyponatremia (causing extreme serum hyperosmolarity)
may develop locked in syndrome
Define “leukoaraiosis
Rarefaction of white matter as seen on imaging studies Correlated with aging and hypertension
What causes Ptosis and miosis on the left side;
2) Decreased pinprick and temperature sensation on the left side of the face;
3) On touching the left cornea, no blinking of either eye is seen;
Both eyes blink when the right cornea is touched;
4) Decreased palatal elevation on the left;
5) Decreased gag reflex;
6) No weakness of the face, arms, or legs; normal tone; normal reflexes;
7) Mild ataxia and dysmetria on finger-nose-finger testing and
dysrhythmic rapid alternating movements on the left;
unable to stand because of dizziness;
8) Decreased pain and temperature sensation in the right limbs and trunk below the neck;
9) Intact light touch, proprioception, and vibration.
Lateral medullary syndrome--> vertebral artery
What causes contralateral arm and leg weakness, contralateral decreased vibration and ipsilateral tongue problems?
Medial medullary syndrome--> paramedian branches of the vertebral artery
What caues right hemisensory loss (face, arm, and leg) to all primary modalities
Normal graphesthesia, stereognosis, motor function, visual fields, and language; no extinction on DSS.
“Pure sensory stroke” syndrome
Lacunar infarcts in the thalamus causing a pure sensory stroke are due to occlusions of thalamoperforators.
The thalamus is supplied by?
The posterior circulation
What can cause one sided primary sensory loss with no other findings?
Lesions of the contralateral ventral posterior thalamus
What does the cortical branchs of the PCA supply?
Inferomedial temporal lobe
Involvement of this area may be associated with a memory defect if the dominant hemisphere is affected
Medial occipital lobe (including the visual cortex
What can cause Alexia without agraphia
Color anomia
Right homonymous hemianopia
PCA blockage or Lesion involves the left visual cortex and extends into the splenium of the corpus callosum
Visual information from both hemispheres is disconnected from the language centers
What can cause a loss of OKN when the moving stimulus is moving toward the side of the lesion?
Lesions of the parietal lobe
What causes a tonic deviation in the direction of the target occurs, but little or no fast phase correction.
Lesions of the frontal lobe
What caues Right carotid bruit (turbulent flow in right carotid consistent with stenosis),
UMN weakness of proximal left arm and proximal left leg
Decreased OKN (decreased left-beating fast phase when OKN strip is moved to right)
Intact sensation (primary and cortical)
Right frontal lobe junction of ACA and MCA cause cause the left arm and leg proximal weakness, frontal lobe cause cause decresed OKN
Systemic hypotension
bilateral watershed territory infarcts
ACA-MCA territory supplies?
proximal arm and leg weakness (man in a barrel)
MCA-PCA territory affects?
disturbances of higher order visual processing
Watershed infarcts in the dominant hemisphere are associated with?
Transcortical aphasias
What causes Decreased level of alertness
UMN signs in left leg
Right homonymous hemianopia
Right arm dysmetria
Trick question…There is no way all of these signs can be due to one lesion.
Decreased level of alertness --> both hemispheres or brainstem
UMN signs in left leg--> right ACA
Dysnomia --> left hemisphere
Right homonymous hemianopia --> left PCA
Right arm dysmetria --> right cerebellum
Bilateral lesions in multiple vascular territories, including both anterior and posterior circulation --> cardiogenic embolism
What casuses right hemiparesis, Multifocal seizures
Bilateral Babinski signs, following delivery?
Bilateral hemorrhagic infarcts
Superior sagittal sinus thrombosis
What would occlusion of the left ICA affect?
entire left hemisphere and the ACA territory on the right
What happens if the more proximal ICA is occluded by thrombosis? (ipsilateral carotid stenosis)
transient monocular blindness aka amaurosis fugax
What are the clinical features and possible locations for infarct and possible vessels invovled in pure motor hemiparesis?
1) unilateral face, arm, and leg umn type weakness with dysarthria
2) posterior limb of internal capsule
3) lenticulostriate arteries
A common site to see small, asymptomatic infarcts is at the?
In the cerebellar watershed territory that is the lateral surface of the cerebellum
What can cause alexia withouth agraphia?
A larger lesion in the left PCA territory extending into the splenium of the corpus callosum
Which artery arises on the left side anywhere from T5-L3 (usually between T9-T12) and it major blood supply to lumbar and sacral cord?
The artery of Adamkiewicz
What is supplying the T4-T8 cord?
watershed area
Where does Most of blood from the superior sagittal sinus drains into and where does most of blood from straight sinus drains into
1) right transverse sinus
2)left transverse sinus
What is an empty detal sign?
when the posterior aspect of the superior sagittal sinus (triangular shape) is filled with thrombus
What type of rigidity do you get with UMN?
clasp knife
What type of rigidity do you get with parkinsons?
What type of rigidity do you get with basal ganglion?
lead pipr or plastic
Which disease has a resting tremor sometimes called a pill rolling tremor?
What is unilateral dyskinesia or unilateral basal ganglia movement disorder in relation to the basal ganglion lesion?
Too much output from the GPi and SNr will tend to do what and cause what?
1) result in bradykinesia or hypokinesia and rigidity
2) parkinsons
Too little output from the GPi and SNr will tend to do what and cause what?
1) result in hyperkinesia
2) huntingtons
What selectively dies in parkinsons and provides the neurotransmitter dopamine to striatal neurons?
What are the four channels of the basal ganglion?
1) motor functions
2) occulomotor
3) cortical
4) emotional
What are the motor parts of the thalamus and which ones do the BG and cerebellum project to?
VA and VL
1) basal ganglion projects to both
2) cerebellum projects to VL
In the direct pathway, how does it begin?
spiny neurons in the putamen express substance P and GABA that project directly onto the output nuclei
What do dopamine and anticholinergic drugs do for hte indirect pathway?
inhibits it so you get more movement
What are the two thalamic fasciculus and how do they travel?
1) lenticular fasciculus travels from GPi above the STN
2) Ansa lenticularis traves from GPi below the STN
What is Autosomal dominant neurodegenerative disease
mutation associated with trinucleotide repeat in the gene (HD) on chromosome 4p.
Cell Death in Striatum (severe Caudate degeneration)
Decreased activity and death of Enk-containing neurons (loss of indirect pathway)
Progressive loss of all BG and Forebrain Functions:
Chorea, dementia, psychiatric disorders, death
Huntingtons disease
What is Unilateral Subthalamic Nucleus Lesion
Loss of BG output (GPi) stimulation
Decrease in thalamic inhibition
What causes release of excitatory amino acid neurotransmitters (e.g., glutamate)  persistent opening of membrane channels (e.g., N-methyl-D-aspartate and kainate receptor stimulation)  influx of calcium and nitric oxide
What effects CA1 region of hippocampus (Sommer’s sector), see red arrow
Purkinje cells of cerebellum
Large cortical neurons in layers 3 and 5
Global cerebral ischemia
What is due to systemic hypotension?
watershed infarction
What is more sensitive to ischemia?
Deeper cortical layers
If someone survives severe systemic hypotension what may be seen at autopsy?
laminar necrosis
What is the breaking down of a definite cell layer in the cerebral cortex, encountered typically after temporary cardiac arrest or perinatal hypoxia.
laminar necrosis
What are susceptible sites for atherosclorsis?
branch points of large vessels
What are sources of emboli to the brain?
cardiac mural thrombus
b = vegetations on valves
c = emboli from carotid plaque
d = infarct distal to embolus
What type of infarct is often embolitic?
What is an infarct measuring < 1.5 cm which results from lipohyalinosis of small penetrating end arteries. This type of vascular disease is usually associated with hypertension, diabetes, and aging.
A lacune--> usually found in the basal ganglia
Hypertension is the number one cause of what?
intercerebral hemorrhage
Which areas of hte brain are hypertensive intracerebral hemorrhages usually found?
mainly putamen, but also caudate, thalamus, pontine, cerebellar, subcortical
What do these cause?
Hypertension (>50% of cases)
Coagulopathy (disease vs iatrogenic)
Vascular malformations and aneurysms
Amyloid angiopathy
Hemorrhage into neoplasm
Drugs (cocaine, amphetamines
intracerebral hemorrhages
PComm aneurysm can compress oculomotor nerve--> what type of aneurysm is this?
saccular aneurysm
The most common cause of spontaneous subarachnoid hemorrhage (SAH)?
ruptured saccular aneurysm
80-90% of aneurysms are in the anterior circulation; the most common site is the ?
anterior communicating artery
What causes worst headache of life? orgasm death?
ruptured saccular aneurysm
What are these causes of?
Pregnancy, post-partum, BC pills
Tumors: meningioma, metastases
Malnutrition and dehydration
Infection (thrombophlebitis)
Hematologic diseases
Paraneoplastic syndrome
Venous sinus thrombosis
What is the anterior portion of the pituitary formed from? What is the posterior and infundibulum formed from?
1) Rathke's pouch
2) diencephalon and invagination of the 3rd ventricle so called neurohyposis
Which regions of the hypothalamus contain warm sensitive neurons? which contain cold sensitive neurons?
1) preoptic and anterior
2) posterior
Lesions of what part of the hypothalamus cause chronic hyperthermia?
anterior and preoptic
Lesions of what part of the hypothalamus cause hypothermia?
Which areas of the hypothalamus are for male sexual behavior and which are for female sexual behavior?
1) preoptic
2) ventromedial
What disrupts the sleep wake cycle?
Lesion of the Suprachiasmatic Nucleus
What does the magnocellular neurons from the paraventricular and supraoptic nuclei produce?
ADH and oxytocin
A defect in vasopressin (ADH) synthesis or ADH release results in what?
Neurogenic diabetes insipidus
What is is often a result of hypothalamic damage to the magnocellular neurons.
It results in polyuria (high volume production of clear dilute urine) and polydipsia (unquenchable thirst) to restore volume.
Diabetes insipidus
What are the six hormones released by the anterior pitutary gland? indirect control
GH – growth hormone
TSH – thyroid stimulating hormone
ACTH – adrenocorticotropic hormone
FSH – follicle stimulating hormone
LH – luteinizing hormone
What is caused by an anterior pituitary tumor that over-secrets ACTH?
Cushings disease
What is the major source of descending output from the hypothalamus regulating the ANS?
Paraventricular nucleus
WHat makes up the grand limbic lobe of broca?
cingulate gyrus, parahippocampal gyrus, orbital frontal gyrus
What is used to store memory?
Loop of Papez
What is the major connection bridge between the amygdala and the hypothalamus and septal nucleus?
stria terminalis
The major connections of the amygdala with the hypothalmus are what?
Stria terminalis and ventral amydalofugal pathway