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285 Cards in this Set

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  • Back
Are brainstem reflexes intact during sleep?
Yes
Are brainstem reflexes intact during coma?
No
What is the function system that mediates wakefulness/arousal?
The reticular formation
Describe the pathways from the reticular formation that regulate wakefulness and arousal:
(from 3 place to 3 places)
Axons from:
1. Pontine cholinerigic nuclei
2. Raphe nucleus (seratonerigic)
3. Locus coeruleus (noradrenergic)

Project to:
1. Reticular nucleus of the thalamus
2. Hypothalamus
3. Basal forebrain (ACh)
Are the sleep-wake cycle and the autonomic functions intact in a vegetative state?
Relatively so, yes
What time periods define a persistent vs. permanent vegetative state?
Persistent = at least 1 mos
Permanent = > 3 mos (non-trauma) or > 1 yr (trauma)
In what state is a pt that has inconsistent and limited ability to follow simple commands, respond yes/no verbally or non-verbally, produce intelligible speech, and display purposeful behavior?
Minimally conscious-state
Where is the lesion typically located in locked-in syndrome?
The ventral pons
What are the physiological components of awareness?
Arousal
Sensory processing
Selective attention
Working memory
What are the two general mechanisms of coma?
1. Diffuse processes affecting both cerebral hemispheres (usually metabolic or toxic)
2. Focal lesions in the brainstem (trauma, vascular, or hypoxic-ischemic etiology)
What parts of the brain are most affected by damage due to ischemia?
Border-zone areas between vascular territories
What parts of the brain are most vulnerable to damage due to hypoxia?
Hippocampus
Purkinje cells of the cerebellum
What provides the location of a lesion in a coma due to a focal abnormality?
Abnormal pupillary responses
Ocular motility disorders
Where is the lesion in a pt in a coma that has fixed, large dilated pupils and what is the most common cause of this condition?
Along CNIII, anywhere from midbrain to the orbit
Most common cause is transtentorial herniation of the medial temporal lobe from a supratentorial mass (uncal herniation)
Where is the lesion in a comatose pt with pinpoint pupils?
Pons
Where is the lesion in a comatose pt with fixed, midsized pupils?
Midbrain
What pharmacological agent can cause pinpoint pupils?
Opiates
What is the location of the lesion in a comatose pt that whose fixed gaze and hemiparesis are on contralateral sides?
The cerebrum (Frontal Gaze Center)
What is the location of the lesion in a comatose pt whose fixed gaze and hemiparesis are on ipsilateral sides?
The pons (pontine gaze center)
Which examinations test the oculovestibular and the oculocephalic reflexes?
Oculovestibular = caloric testing
Oculocephalic = doll's eyes
How is caloric testing performed? What is a normal response and what does it indicate?
Test = cold water in ears

Normal = tonic deviation towards irrigated side

Indication = integrity of pathways from midbrain to lower pons
(Oculovestibular reflex)
How is doll's eye test performed? What is a normal response and what does it indicate?
Test = brisk movement of head

Normal = Tonic conjugate eye movements toward contralateral side

Indication = intact reflex pathways from midbrain to cervico-medullary junction
What is decorticate posturing and what is the location of a lesion associated with it?
Posture = flexed UE and extended LE

Lesion = upper midbrain (thalamus)
What is decerebrate posturing and what is the location of a lesion associated with it?
Posture = extended UE and LE

Lesion = lower midbrain
Where is the lesion in a comatose patient with flacid posture?
Lower pons (actual spinal cord injury)
What motor signs would be expected in a comatose patient with a frontal lobe lesion?
Primitive reflexes (grasp, Babinski)
What are the early and late signs of central herniation syndrome?
Early = Small, poorly reactive pupils; decorticate posturing

Late = Mid-position, fixed pupils; (-) Doll’s eyes, decerebrate posturing
What are the early and late signs of uncal herniation syndrome?
Early = Unilateral dilated pupil (parasymp. fibers III nerve); brainstem reflexes intact

Late: (-) Doll’s eyes, dysconjugate gaze; decerebrate posturing
What are the components of the Glasow Coma Scale (3) and the FOUR Score (4)?
Glasow:
Eye response
Motor response
Verbal response

FOUR:
Eye response
Motor response
Brainstem reflexes
Respiration
What is the hallmark of metabolic encephalopathy?
Reactive pupils in the presence of otherwise impaired brainstem function
What are the three clinical syndromes associated with frontotemporal dementia?
FTD - prominent behavior and personality changes (R>L, frontal and temporal)
Primary Progressive Aphasia (PPA) - early speech and language dysfunction (L frontal and temporal)
Semantic Dementia (SD) - loss of word and object meaning
What portion of patients with frontotemporal dementia also have parkinsonism or motor neuron disease?
1/3
What is the clinical triad of normal-pressure hydrocephalus?
Gait disturbance
Dementia
Urinary incontinence
What are the diagnostic MRI findings for normal-pressure hydrocephalus?
None - MRI is not diagnostic
What are the three non-motor fronto-subcortical circuits?
Inhibition - orbitofrontal circuit
Executive - dorsolateral prefrontal
Motivation - ant. cingulate/med. frontal
What are the core features (3) and the suggestive features (2) of dementia with lewey-bodies?
Core Features:
1. Fluctuating cognition
2. Recurrent visual hallucinations
3. Spontaneous parkinsonian motor signs

Suggestive Features:
1. Neuroleptic sensitivity
2. REM sleep behavior disorder
What is the time course for delirium?
Develops over short period (hours to days) and usually resolves in 10-12 days (may last up to 2 months)
What are the clinical features of delirium? (8)
1. Prodrome
2. Fluctuating course
3. Attentional deficits
4. Arousal/psychomotor disturbance
5. Impaired cognition
6. Sleep-wake disturbance
7. Altered perceptions
8. Affective disturbances
What are the 11 specific causes of delirium?
"I WATCH DEATH"
1. Infections (encephalitis, meningitis, sepsis)

2. Withdrawal (ETOH, sedative-hypnotics, barbituates)

3. Acute metabolic (acid-base, electrolytes, liver or renal failure)

4. Trauma (brain injury, burns)

5. CNS Pathology (hemorrhage, seizures, stroke, tumor (mets))

6. Hypoxia (CO poisoning, hypoxia, pulmonary or cardiac failure, anemia)

7. Deficiencies (thiamine, niacin, B12)

8. Endocrinopathies (hyper- or hypo-adrenocortisolism, hyper- or hypoglycemia)

9. Acute vascular (hypertensive encephalopathy and shock)

10. Toxins or drugs (pesticides, solvents, meds, drugs of abuse)

11. Heavy metals (lead, manganese, mercury)
Description of an abnormal movement should include what four characteristics?
1. Distribution
2. Relation to posture, activity, sleep
3. Precipitating and relieving factors
4. Suppression
What are the three diagnoses under the category of akinetic-rigidity syndrome?
1. Drug-induced parkinsonism
2. Parkinson's disease
3. Parkinson's-plus syndrome
Tremors are dyskinesias with what pattern?
Rhythmic, sinusoidal movement
Dystonia is a dyskinesia with what pattern?
Muscle spasm, twisting, sometimes repetitive
Dyskinesias that can't be classified as tremors or dystonia are what?
Jerks
Parkinson's tremors occur at rest, with action, or both?
At rest
Essential tremors and cerebellar tremors occur at rest, with action, or both?
With action
Holm's (Rubral) tremor occurs at rest, with action, or both?
Both
What are the three types of jerks and what are their distinguishing features?
1. Tics - can be voluntarily suppressed
2. Chorea - "dance like," unpredictable
3. Myoclonus - fast, "shock like"
Movement control signals from the cerebral cortex to the spinal cord are modified by what two areas of the brain?
Basal ganglia
Cerebellum
What is the most common etiology of Parkinson's disease?
Idiopathic
What are the four primary clinical findings in Parkinson's Disease?
1. Tremor
2. Rigidity
3. Hypokinesia
4. Abnormal gait and posture
What areas are most often affected by essential tremor?
One or both hands
Head and voice
(Legs typically spared)
What is the typical reflex response in Parkinson's disease?
Normal
Other than family history, what feature often differentiates Huntington disease from Parkinson's disease?
Dementia at the time of initial presentation
What commonly effective treatment for essential tremor can help to distinguish it from Parkinson's disease?
Alcohol
What physical finding can help to distinguish Progressive Supranuclear Palsy from Parkinson's disease?
Voluntary downward and horizontal gazes are typically lost in progressive supranuclear palsy, but not Parkinson's
(tremor is also rare in PSP)
Progressive supranuclear palsy is an idiopathic degeneration of what area of the brain?
Subcortical gray matter
(midbrain, pons, basal ganglia, and dentate nuclei of cerebellum)
What are the two primary symptoms of Huntington disease?
Dementia
Chorea
Does healing equal the elimination of symptoms?
No
Is stress involved in the pathogenesis and/or maintenance of illness in a majority of cases?
Yes
Cerebral ischemia is possible when the cerebral perfusion pressure drops below what?
800 mm H2O
What is the normal adult intracranial pressure?
70-180 mm H2O
Name and describe the three types of brain edema.
1. Interstitial - blocked CSF pathways

2. Vasogenic - breakdown of the blood-brain barrier

3. Cytotoxic - cellular swelling
List three conditions that result from interstitial edema (block in CSF pathway).
1. Hydrocephalus
2. Normal Pressure Hydrocephalus
3. Pseudotumor Cerebri
What are six signs and symptoms of hydrocephalus?
1. Headache
2. Nausea and vomiting (projectile)
3. Double vision
4. Blurred vision
5. Increased reflexes, extensor plantar responses, weakness (all upper motor neuron findings)
6. Increasing head size in young children
What features of normal pressure hydrocephalus predict a good outcome with shunting? (6)
1. Recent onset of symptoms
2. Known prior cause
3. Gait disturbance most prominent
4. No co-existing CNS disease
5. Younger age at onset
6. Limited brain atrophy or infarcts
What physical findings are expected in a patient with pseudotumor cerebri?
Normal exam except for papilledema
What CT and MRI findings are expected in a patient with pseudotumor cerebri?
Normal images
What are five treatments that can be used for vasogenic edema?
1. Osmotic agents (mannitol)
2. Acetazolamide or furosemide to reduce CSF formation
3. Hyperventilation
4. Upright head position for venous drainage
5. Steroids
Which treatment option for vasogenic edema has a very poor response in the treatment of cytotoxic edema?
Steroids
What is the characteristic of the headache associated with low CSF pressure syndrome?
The headache occurs on sitting or standing and is improved with lying down
What is the function of each of the following portions of the spinal cord?
1. Posterior columns
2. Spinothalamic tracts
3. Corticospinal tracts
4. Spinocerebellar tracts
5. Anterior columns
6. Commissural fibers
7. Grey matter
1. Posterior columns - position and vibration
2. Spinothalamic tracts - pain and temperature
3. Corticospinal tracts - motor
4. Spinocerebellar tracts - coordination
5. Anterior columns - touch
6. Commissural fibers - pain and temperature
7. Grey matter - anterior horn cells, sympathetic nerves, sensory relay neurons
Urinary retention without pain is indicative of a spinal lesion at what level?
Above T6
(below T6 = retention with pain)
Bowel incontinence is only common in what type of spinal cord lesion?
Conus lesions
What types (2) of spinal cord tumors have an intradural, extramedullary location?
Meningioma
Neurofibroma
What types (2) of spinal cord tumors have an intradural, intramedullary location?
Ependymoma
Glioma
A spinal cord tumor with an extradural lesion is of what type?
Metestatic
What is the neurological pathology of B12 deficiency and at what level does it occur?
Posterior column demyelination
Cortical spinal tracts ~T8 and below
What are the neurological signs and symptoms of B12 deficiency?
1. Absent deep tendon reflexes
2. Extensor plantar responses
3. Weakness, paresthesia or burning pain
4. Romberg positive
What are the clinical findings of Brown-Sequard syndrome?
Ipsilateral loss of position and vibration sensation
Contralateral loss of pain and temperature sensation
What are the clinical findings of a spinal lesion that results from loss of blood supply to the spinal cord from the anterior spinal artery?
Sensory loss will depend on size of lesion, but sense of position and vibration most often is maintained because of its posterior location in the spinal cord
What are the clinical findings that result from a posterior cord lesion?
Primarily loss of position and vibration sensation below and on the side of the lesion
What are the clinical findings that result from a central cord lesion?
Primarily bilateral loss of pain and temperature sensation
What are the clinical findings that result from a lateral cord lesion?
Loss of motor function in one extremity, usually lower
Loss of contralateral pain and temperature sensation below the level of the lesion
What type of tumor typically occurs in the cervical and lumbar outflow tracts?
Glioma
What type of tumor typically occurs in the thoracic cord?
Meningioma
What type of tumor typically occurs in the conus medularis?
Ependymoma
A syrinx occurs in what part of the spinal cord?
The central
Syrinx results from what congenital malformation?
Arnold-Chiari malformation
A syrinx will affect motor function in what portion of the body?
Upper (arms, shoulders, hands)
Epidural abscesses typically occur where on the spinal cord (anterior, posterior, central, etc)?
Posterior
What diagnosis should be considered in a patient with post-operative leg weakness?
Anterior spinal artery syndrome
What % of our muscle fibers needs to be active before weakness sets in?
50%
What stimulates the release of ACh in the generation of a MEPP?
Nothing - it is spontaneous
Weakness that varies markedly over time is typical of what NMJ disorder?
Myasthenia gravis
Transient improvement of symptoms with administration of what drug is indicative of the diagnosis of myasthenia gravis?
Edrophonium (Tensilon)
The safety factor is reduced in what type of NMJ disorders?
Post-synaptic
(no abnormality in the release of ACh, but in the response to its release)
Repetitive stimulation testing in the diagnosis of myasthenia gravis is lacking in which, sensitivity or specificity?
Sensitivity
What is the gold standard diagnostic test in the diagnosis of myasthenia gravis?
Single Fiber EMG
The thymus gland plays a critical role in the development of what neuromuscular disorder?
Myasthenia gravis
What findings on repetitive nerve stimulation are indicative of pre-synaptic NMJ disorders (Lambert-Eaton syndrome)?
Reduced initial CAMP amplitude
Progressive decrement at slow rates of stimulation
Marked facilitation with fast rates of stimulation (>100%)
Which type of botulism produces a more severe and longer lasting form of illness?
Type A
Rapid nerve stimulation has what findings in a pt with botulism toxin?
Reduced CAMP amplitudes
Decrement to slow rates of stimulation
Post activation facilitation
What is the pattern of weakness in the clinical presentation of classic botulism?
Descending
What are three findings suggestive of botulism as the cause of a NMJ disorder?
1. Rapidly progressive weakness that occurs in a descending fashion
2. Associated opthalmoparesis
3. Sluggish pupils
Ocular muscle and eyelid involvement is typical of which NMJ disorder?
Myasthenia gravis
Why are the number of quanta released less than normal in a patient with a pre-synaptic NMJ disorder?
Decreased calcium in the pre-synaptic terminal
Voltage-Gated Calcium Channel Antibodies are found in 50-80% of patients with what condition?
Lambert-Eaton Syndrome
Describe the muscle stretch reflexes in a patient with Lambert-Eaton syndrome?
Diminshed
Progressive weakness in the presence of autonomic symptoms is suggestive of what NMJ disorder?
Pre-synamptic (LES)
What is the most common dystrophy in adults?
Myotonic Dystrophy
What is the inheritance pattern of myotonic dystrophy?
Autosomal dominant
Myotonic dystrophy is a syndrome of weakness of what muscles?
Facial, neck and distal limb
Kearnes-Sayre syndrome is characterized by what four things?
1. Progressive external opthalmoplegia
2. Proximal weakness
3. Retinitis pigmentosa
4. Hearing loss
Which myopathy is closely associated with systemic neoplasm?
Dermatomyositis
What is the most common inflammatory myopathy in adults?
Inclusion body myositis
What is the laboratory finding diagnostic of inclusion body myositis?
Inclusion bodies on EM
Which part of the nervous system is affected by vasculits (central vs peripheral)?
Peripheral (typically with mononeuropathy multiplex)
What is the most common category of immune mediated vasculitis processes?
Circulating immune complexes
What type of antibiotics should not be used in the tx of meningitis?
Those that lyse bacteria
In addition to antibiotics, what other medication should be administered to patients with bacterial meningitis?
Steroids
What is the classical triad of brain abscess presentation?
Fever, headache, and focal neurological signs (only present in 50% of pts)
Which of the dystrophinopathies is marked by mental retardation?
Duchenne
Which of the dystrophinopathies is generally more severe?
Duchenne
What are four conditions/traits associated with myotonic dystrophy?
1. Cataracts
2. Male pattern baldness
3. Cardiac conduction blocks
4. Endocrine abnormalaties
What is the histological finding that is diagnostic of mitochondrial myopathies?
"Ragged Red Fibers" on Gomori trichome stain
What are the typical features of MELAS?
Mitochondiral myopathy
Encephalopathy
Lactic Acidosis
Strokelike Episodes
What is the most common cause of acquired muscle weakness?
Inflammatory Myopathies
What are the three inflammatory myopathies we need to know?
Polymyositis
Dermatomyositis
Inclusion body myositis
Describe the pattern of muscle weakness seen in polymyositis and dermatomyositis
Subacute onset of symmetrical, proximal weakness
What are the CK levels typically found in polymyositis and dermatomyositis?
Elevated (up to 50x normal)
Which of the inflammatory myopathies has an associated rash?
Dermatomyositis
Which of the inflammatory myopathies often overlaps with mixed connective tissue/autoimmune diseases?
Polymyositis
Males are affected with which myopathy three times more often than females?
Inclusion body myositis
Metabolic myopathies are characterized by weakness or paralysis due to abnormalaties in what serum electrolyte?
Potassium
A painless myopathy with slow onset of proximal muscle weakness of the lower extremities and seldom of the upper extremities is characteristicly caused by what substance?
Corticosteroids
Steroid myopathy is worse in patients also receiving what drug?
Neuromuscular blocking drugs
Malignant hyperthermia is caused by a defect in what receptor?
Ryanodine (Ca channel on SR)
Malignant hyperthermia is precipitated by what drug?
Neuromuscular blocking agents (succinylcholine)
Hemorrhage, anorexia, emotion and pain, mictruition, carotid sinus syndrome, and glossopharyngeal neuralgia are all precipitating factors of what kind of syncope?
Vasovagal
Vasovagal syncope typically occurs when patients are in what position(s)?
Standing or sitting
Respiratory syncope can be caused by what activities? (3)
Coughing
Weight lifting
Straining against a closed airway
True of False: The brain can learn to have seizures.
True
Will an altered balance of excitation/inhibition produce seizures even in the normal brain?
Yes
What are two receptor or neurotransmitter changes that can be the mechanisms involved in chronic partial epileptogenesis?
Enhanced glutamate-mediated neruotransmission
Reduced GABA-mediated inhibition
What are two nonsynaptic changes that can be the mechanisms involved in chronic partial epileptogenesis?
Alterations in glial buffering
Increased electronic coupling
What are three alterations in neuronal networks that can be the mechanisms involved in chronic partial epileptogenesis?
Loss of inhibitory neurons
Aberrant synaptogenesis such as the formation of excitatory recurrent collaterals
Alterations of dendrites, axons, and synaptic terminals
Which type of epilepsy (partial or generalized) is more commonly due to genetic causes?
Generalized
What factors increase one's risk of developing a genetic seizure disorder?
Mother is the parent with epilepsy
Early age at onset of seizure in a parent with epilepsy
Number of affected relatives
Generalized spike and wave EEG
Type of seizure, especially absence seizures
How many in 10 individuals will have a seizure at some point in their life?
1
Recruitment of neurons is key to the development of what class of seizures?
Partial/focal
What distinguishes a simple from a complex partial seizure?
Complex has associated impairment of consciousness and/or memory loss
Simple has neither
What are the four types of events that can occur with simple partial seizures?
1. Sensory (olfactory, visual, somatosensory)
2. Motor
3. Psychic
4. Autonomic
What are five hormones involved in the stress response?
CRF
Dopamine
ACTH
Cortisol
Epinephrine
What is the main behavioral feature of seizures?
Stereotypia
Turning, bicycling, posturing, vocalization, speech arrest, head turning, eye deviation, tonic and clonic activity are all typical of partial seizures located in what portion of the brain?
Frontal lobe
Somatosensory illusions, metamorphopsia, and rotatory sensations are typical of partial seizures located in what portion of the brain?
Parietal lobe
Autonomic, psychic or olfactory hallucinations, epigastric rising sensation, and oralimentary automatisms are all typical of partial seizures located in what portion of the brain?
Temporal lobe
What are the six types of primary generalized seizures?
1. Absence
2. Atonic
3. Tonic
4. Clonic
5. Tonic-Clonic
6. Myoclonic
What type of seizure is associated with a 3-per-second Spike-Wave discharge?
Absence
Is postictal confusion a feature of absence seizures?
No
What age group are atonic seizures more likely in?
Children
What is the associated EEG pattern of primary generalized tonic seizures?
Low amplitude fast EEG pattern
How long do tonic-clonic seizures usually last?
Less than 5 minutes
Is postictal confusion a feature of myoclonic seizures?
No
Are idiopathic epilepsies usually genetic?
Yes
Does the majority of adults have a clear identifiable cause for seizures?
No
A seizure can be caused by anything that causes disfunction of which, white or grey matter?
Grey
Epilepsies with an idiopathic/genetic syndrome as the etiology are most likely to manifest themselves at what age?
Childhood and adolescence
Epilepsy in an infant has what three likely etiologies?
1. Birth injury
2. Inborn error of metabolism
3. Congenital malformation
What is the required laboratory data in the workup of a patient with epilepsy?
Head Imaging
EEG
Is lumbar puncture a routine part of the workup of a patient with new onset seizure/epilepsy?
No
Used only if meningitis or encephalitis is suspected and potential for brain herniation is ruled out
Which patients with seizures should be treated?
1. Those at risk for another seizure
2. Those at risk from major morbidity from a seizure
True or false: a patient may have more than one seizure type in only one type of epilepsy?
True
What are the six features of an upper motor neuron lesion?
1. Increased muscle tone or spasticity
2. Increased or brisk reflexes
3. Extensor plantar responses (Babinski)
4. Minimal muscle wasting, usually due to disuse
5. Mono-, hemi-, para-paresis or plegia
6. Clonus or other pathological reflexes
What are the five features of a lower motor neuron lesion?
1. Muscle atrophy/wasting
2. Flaccid weakness
3. Diminished deep tendon reflexes
4. Muscle fasciculations
5. Absent or flexor plantar responses
Could a chest x-ray be indicated in the workup of a patient with peripheral neuropathy?
Yes
(along with paraneoplastic antibodies, if indicated)
What is the responsible gene that is involved in spinal muscular atrophy and where is it located?
Survival motor neuron gene
Chromosome 5
Which is the most severe of the spinal muscular atrophy types?
Type 1 - Werdig-Hoffmann disease
Is the muscle weakness associated with poliomyelitis symmetric or asymmetric?
Asymmetric
ALS is a degenerative disease of what portion of the nervous system?
Anterior horn cells and corticospinal tract
(combined upper and lower motor neuron disease)
What nerve roots (by spinal level) are most commonly affected by disc herniation or degenerative diseases of the spine?
C6/7 and L4-S1
What are the four pathological processes affecting the peripheral nerves?
1. Wallerian degeneration
2. Axonal degeneration
3. Primary neuronal degeneration (ant. horn cell/lower motor neuron)
4. Segmental demyelination
What is the most common entrapment neuropathy?
Carpal tunnel syndrome
What are the symptoms of radial nerve palsy?
Wrist drop and inability to extend hand at wrist
What are three things that can cause peroneal nerve palsy?
Weight loss
Excessive leg crossing
Brace
What are the symptoms of peroneal nerve palsy?
Foot drop and preserved ankle jerk
Multifocal motor neuropathy is associated with serum antibodies to what?
Myelin-associated proteins
What is the treatment for multifocal motor neuropathy?
Immunosuppressive therapies
What is the genetic defect involving chromosome 17 that is associated with HMSN type 1A?
Gene duplication
What are five clinical features of HMSN 1?
1. Rarely presents with sensory symptoms
2. Pes cavus and hammertoes
3. Abnormal gait, clumsiness in running
4. Loss of deep tendon reflexes
5. Enlarged palpably firm nerves
What happens to the peripheral nerves in HMSN 1?
Demyelination
What is the genetic alteration that occurs to the gene associated with hereditary neuropathy with liability to pressure palsy?
Deletion
Increased susceptibility to nerve injury from mechanical compression or traction is a feature of what lower motor neuron disease?
Hereditary Neuropathy with Liability to Pressure Palsoy(HNPP)
Is pain associated with the entrapments that occur in HNPP?
No
What is the pattern of paralysis seen in Guillain-Barre syndrome?
Progressive ascending
What is the most common acute paralytic disease in Western countries?
Guillain-Barre syndrome
What is the typical course of Guillain-Barre syndrome?
Majority recover within one year
What is the acute treatment for Guillain-Barre syndrome?
Plasmapheresis or IVIG
What lower motor neuron disease is characterized by predominantly motor symptoms of proximal and distal, symmetric muscle weakness of upper and lower extremities?
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)
What will be revealed with reflex testing in a patient with CIDP?
Loss of deep tendon reflexes
Excess supplementation with which vitamin can result in toxic neuropathy?
B6
Deficiency of what vitamins can result in toxic neuropathy?
B1
B6
B12
E
How many and what types of cells are found in septic CSF?
400-40,000 polynuclear cells
How many and what types of cells are found in aseptic CSF?
5-2000 mononuclear cells
What are the four criteria for restless legs syndrome?
1. Discomfort of the legs
2. Symptoms worse at rest
3. Relieved by activity
4. Symptoms worse in the evening or night
Why are the symptoms of restless legs syndrome worse at night?
Dopamine has a circadian rhythm
What are the expected EMG findings in myofascial pain?
Normal
What is the most common neurological cause for admission to hospital?
Stroke
What is the time duration of most TIAs?
<20 minutes
What are six things that can mimic stroke/TIA?
1. Migraine (w/ or w/o headache)
2. Focal seizure
3. Intracranial mass
4. Subdural hematoma
5. Hypoglycemia
6. Primary ear or eye disease
How do the visual symptoms of a TIA in the carotid circulation differ from one in the vertebrobasilar circulation?
All the visual symptoms for the carotid circulation are ipsilateral monocular, whereas those for the vertebrobasilar circulation are binocular
Vertigo is a symptom of a TIA involving which circulation?
Vertebrobasilar
On which side does weakness, clumsiness, and numbness of the hand, arm and face occur in a TIA involving the carotid circulation?
Contralateral
How soon after a stroke can it be detected on MRI using DWI or PWI?
1 hour
In the absence of medical emergency ICH or thrombolysis consideration, how high should a stroke patient's blood pressure get before treatment is initiated?
Systolic >200-220 mm Hg
What drug is NOT recommended in the medical treatment of brain edema following a stroke?
Corticosteroids
For what period of time following a stroke is anticoagulation typically prescribed?
Many years
What are the three stroke situations in which heparin may play a role?
1. Cardiac embolism
2. Large cerebral vessel stenosis
3. Stroke-in-evolution or worsening
What is the only FDA approved treatment of acute ischemic stroke?
rTPA
How soon following onset of stroke symptoms should rTPA be initiated?
3 hours
What are four nonmodifiable risk factors for stroke or TIA?
1. Age
2. Gender
3. Race/ethnicity
4. Hereditary
Do studies indicate significant benefit of using warfarin over aspirin in preventing recurrent ischemic strokes?
No
What are the four factors considered in TIA risk stratification?
Age (≥60 yrs)
Blood Pressure (>140 and/or ≥90)
Clinical Features (weakness = 2, slurred speech = 1)
Duration (≥60 min = 2; 10-59 min = 1)
Kernig's and Brudzinski's signs are inidicitive of what?
Meningeal irritation
What are the three mechanisms of vasculitis that affect the CNS?
1. Secondary to infection
2. Immune mediated
3. Tumor invasion of vessel wall
Over what regions of the brain do subdural fluid collections more commonly occur?
Cerebral hemispheres
What is the most common pathogen associated with meningitis before 3 mos of age?
Group B strep
(also E. coli and L monocytogenes)
What are the most common pathogens associated with meningitis in ages 3-8? (3)
S. Pneumonia
N. Meningitides
H. influenza
What are the two most common pathogens associated with meningitis in ages 18-50?
S. Pneumonia
N. Meningitides
What are the most common pathogens associated with meningitis in individuals over age 50? (3)
Gram negative rods
S. Pneumonia
L monocytogenes
What treatment for meningitis has been shown to decrease the incidence of hearing loss in children?
Steroids
What treatment for meningitis has been shown to decrease morbidity and mortality in adults?
Steroids
Where do brain abscesses start?
Cortical-medullary junction
To which type of cerebral matter do abscess have a predilection to spread and why?
White matter
It is better vascularized and thus the abscess can form a better capsule
Is rupture of brain abscesses into the subarachnoid space common or rare?
Rare
What lobes are affected by abscess?
Frontal
Parietal
Occipital
(Temporal and cerebellum spared)
In what percentage of HIV patients are neuro symptoms the presenting feature?
10%
What percentage of HIV patients have a mild cognitive motor disorder?
20%
AIDS Dementia Complex is rare if the CD4 count never drops below what level?
200
What are the early problems of AIDS Dementia Complex?
Concentration and memory
What are the pulse and blood pressure findings found in Cushing's reflex?
Pulse = low
BP = high
What are three major consequences of sleep deprivation?
1. Changes in mood
2. Decrease of consistent performance
3. Promotes weight gain
What are five physical features of sleep apnea?
1. Hight blood pressure
2. Tachycardia
3. Narrow airway
4. Large neck
5. Edema
Periodic limb movements of sleep are more common in patients with what?
Spinal cord processes
What are the six etiologies of PLMS/RLS?
1. Idiopathic
2. Iron deficiency
3. Uremia
4. PVD
5. Neuropathy
6. Arthritis
Why are patients with RLS due to iron deficiency not anemic?
Because the defect is in transport of iron across the BBB
Reduction in what type of neurons is found in patients with narcolepsy with cataplexy?
Orexin
What are the three factors that determine sleep onset?
1. Environment
2. Psychological state
3. Neurophysiological state
What is the most common cause of chronic insomnia?
Psychiatric disorders
(then psychological issues)
What should the history in a patient with insomnia focus on?
Daytime activities
What is the best long term therapy for chronic insomnia?
Cognitive behavioral therapy
What part of the brain controls the daily rhythm?
The suprachiasmatic nucleus
Migraine is associated with a fundamental change in function of what three things?
1. Cortex
2. Brainstem
3. Trigeminovascular system
What are the four characteristics of migraine?
1. Unilateral
2. Throbbing
3. Moderate-severe intensity
4. Pain worsened by exertion
Half of all migraine onsets occur before what age?
20
Does the incidence of migraines peak earlier in boys or girls?
Boys (12 vs 15)
Is the prevalence of migraines higher in males or females?
Females (3:1)
What is the age range in which migraines peaks?
25-55
What percentage of migraine patients experience the classical symptoms of migraine with aura?
Only 15%
What is the second most common type of migraine associated aura?
Sensory
Trigeminovascular activation promotes release of what?
Neuropeptides
(CGRP and substance P)
In the treatment of migraines, pain-free efficacy is more easily achieved if pain is treated before what occurs?
Central sensitization
Migraine is present in roughly what % of the population?
12%
What type of fibers trasmit sharp immediate pain?
A-delta
What type of fibers mediate prolonged, unpleasant burning pain?
C fibers
What is the cause of redicular pain?
Compression or infiltration of nerve root
What type of pain has a sharp, shooting, burning, prickling or aching quality?
Neuritic/Neuropathic
What is the cause of neuritic/neuropathic pain?
Deterioration or compression of peripheral nerves
What two things can mimic radicular and/or neuropathic pain?
Facet Joint Arthropathy
Myelopathy (spinal cord injury)
What is the female to male prevalence of myofascial pain?
3:1
What four agents can be used in the acute pharmacologic treatment of myofascial pain?
1. NSAIDs
2. Corticosteroids
3. Muscle relaxers
4. Opiates
What three symptoms must be present, and for what duration, to meet the criteria for widespread pain in fibromyalgia syndrome?
1. Pain bilaterally
2. Pain above and below the waist
3. Axial spine pain

All for ≥ 3 months
A detailed neurological evaluation of patients with fibromyalgia should be done to exclude what? Why?
Cervical myelopathy
It is potentially treatable
Fibromyalgia has been associated with a loss of what type of sleep?
Delta wave
What non-pharmacological modalities have been proven to treat and/or prevent pain in fibromyalgia? (3)
1. Cardiovascular fitness training
2. Biofeedback training
3. Cognitive/behavioral therapy
What are the two key features of myofascial pain? Is it focal or diffuse?
Taut Band
Trigger Point

Focal
Is the presence of taut bands necessarily characteristic of fibromyalgia?
No
What five pharmacologic agents can be used in the preventive treatment of myofascial pain?
1. Tricyclic anti-depressents
2. Scheduled muscle relaxers
3. NSAIDs
4. Sleep aids
5. Long-acting opiates
In addition to the drugs used to prevent myofascial pain, what two drugs can be used to prevent pain in fibromyalgia?
Dopamine agonists
Anticonvulsants
What is the most common cause for peripheral neuropathy worldwide?
Leprosy
What is the most common acute paralytic disease in Western countries?
Guillain-Barre Syndrome
What is the abnormal protein associated with Parkinson's disease and dementia with Lewy Bodies?
Alphasynuclein
What are the typical signs of CJD? (3)
Dementia
Myoclonus
Ataxia
Why would an autoimmune thyroid panel be included in the workup of CJD?
To rule out Hashimoto's encephalopathy