Neurology 1
Terms
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- 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 -
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)