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Internal Med EOR > Neuro 2 > Flashcards

Neuro 2 Flashcards

1
Q

what is Huntington’s disease

A

autosomal dominant neurodegenerative disorder characterized by involuntary choreatic movements with cognitive and behavioral disturbances

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2
Q

pathophys of Huntington disease

A

CAG repeats on chromosome 4

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3
Q

when do sx usually begin for huntington

A

30-50

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4
Q

sx huntington

A

3 hallmarks - mood, movement, memory - behavioral and mood changes, chorea (rapid involuntary movements), dementia

chorea - face, neck, trunk, limbs - may disappear during sleep and worse with stress and voluntary movements

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5
Q

PE huntington

A

restlessness, fragility
quick, involuntary hand movements
brisk DTR

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6
Q

dx huntington

A

clinical sx + FHx + genetic confirmation

CT or MRI - cerebral and striata (caudate nucleus and putamen) atrophy with subsequent widening in the frontal horns of the lateral ventricle (boxcar ventricle sign)

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7
Q

tx huntington

A

no cure - usually fatal within 15-20 years after presentation

tetrabenazine for dyskinesia or chorea

antidopaminergics - typical and atypical antipsychotics

benzos intermittently may help w chorea and sleep, esp during stressful situations

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8
Q

what is essential tremor

A

autosomal dominant disorder
incidence increases w age

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9
Q

sx essential tremor

A

intuitional tremor - postural b/l action tremor most commonly affecting the upper extremities and head (hands, forearms, head, neck, voice)

tremor worsened with action and intentional movement, postural - holding affected body part against gravity, adrenergic activity - stress, anxiety

tremor improved with alcohol ingestion, when body part is supported, at rest

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10
Q

PE essential tremor

A

more pronounced with arms suspended against gravity in a fixed posture

during goal-directed activity (finger to nose testing) the tremor increases at the end of approaching the target or holding a position against gravity

may see cogwheel phenomenon

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11
Q

dx essential tremor

A

dx of exclusion based on history, FHx, physical exam

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12
Q

tx essential tremor

A

no needed if mild

first line - propranolol - first line - or primidone (barb) if no relief w propranolol

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13
Q

parkinson disease

A

neurodegenerative movement disorder due to decreased dopamine resulting from idiopathic loss of dopaminergic neurons in the striatum and substantia nigra and the presence of Lewy bodies

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14
Q

pathophys Parkinson

A

decreased dopamine –> imbalance of dopamine and acetylcholine –> improper movement due to failure of acetylcholine inhibition in basal ganglia

eosinophilic cytoplasmic inclusions (Lewy bodies) and loss of pigment cells in substantia nigra

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15
Q

sx parkinson

A

motor triad - -resting tremor, bradykinesia, muscle rigidity

resting tremor - pill-rolling; worse at rest, emotional stress, excitement, walking; better with voluntary activity, intentional movement, sleep

cogwheel rigidity

postural instability - stooped posture and loss of postural reflexes

loss of smell (anosmia), mood disorders (depression), constipation, excess salivation or drooling (sialorrhea), sleep dysfunction

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16
Q

PE parkinson

A

normal DTRs
no weakness
face involvement - fixed facial expression
myerson’s sign - tapping bridge of nose causes a sustained blink
postural instability - pull test - standing behind patient and pulling the shoulders causes the patient to fall or take steps backwards

17
Q

tx parkinson

A

levodopa-carbidopa - most effective

dopamine agonists - bromocriptine, pramipexole, ropinirole); can be used to delay use of levodopa in younger patients

18
Q

Guillain barre

A

group of conditions due to acquired autoimmune-mediated demyelinating polyradiculopathy of the peripheral NS characterized by rapidly evolving muscular weakness

19
Q

most common cause of Guillain barre

A

campylobacter jejune MC
other antecedent GI or respiratory infections - influenza A and B

20
Q

pathophys Guillain barre

A

molecular mimicry –> autoantibodies attack schwwann cells

21
Q

sx Guillain barre

A

symmetric, ascending, progressive, flaccid muscle weakness and sensory changes (paresthesias, pain)

cranial nerve and bulbar sx - cranial nerves 3, 4, 6

weakness of respiratory muscles

sensory involvement - paresthesias, pain, cramps

22
Q

PE Guillain barre

A

LMN signs - decreased DTR

sensory deficits and cranial nerve palsies (3, 4, 6)

autonomic dysfunction - tachycardia, arrhythmias, hypotension, HTN, breathing difficulties

23
Q

dx Guillain barre

A

electrophysiologic studies - decreased motor nerve conduction velocities and amplitude

CSF analysis - high protein with normal WBC

autoantibodies - anti-GM1, anti-GD1A, anti-GT1A, anti-GQ1B

PFTs - decreased peak inspiratory pressure and forced vital capacity

24
Q

tx Guillain barre

A

plasmphaeresis or IVIG first line for antibody removal

prednisone not indicated

25
Q

what is myasthenia gravis

A

autoimmune peripheral nerve disorder of the neuromuscular junction due to autoantibodies against the acetylcholine receptor on the muscles –> weakness

26
Q

epidemiology myasthenia gravis

what other abnormality is strongly associated with myasthenia gravis

A

bimodal distribution - most common in young women < 40 and men > 50

strong associated w an abnormal thymus gland (hyperplasia or thymoma)

27
Q

what type of hypersensitivity rxn is myasthenia gravis

A

type 2 - autoantibodies against acetylcholine (nicotinic) postsynaptic receptor at the neuromuscular junction

28
Q

sx myasthenia gravis

sensation and DTR?

A

ocular weakness and generalized weakness worsened with repeated use

diploma and ptosis; pupils are spared

fluctuating skeletal muscle weakness worsened with repeated muscle use throughout the day, often with true muscle fatigue

bulbar weakness - weakness w prolonged chewing, dysphagia, dysphonia, dysarthria

respiratory muscle weakness may lead to respiratory failure - myasthenic crisis

sensation and DTR preserved

bedside ice pack test - can be used if ptosis, but not helpful w extra ocular muscle weakness; application of ice for 10 minutes improves ocular sx of MG

29
Q

dx myasthenia gravis

A

serologic antibody testing - acetylcholine receptor antibodies - initial test of choice - AChR-Ab positive

MuSK antibodies obtained if ACR antibodies negative

anti-striated muscle antibodies - usual marker for thymoma in early onset MG

electrophysiology testing - repetitive nerve stimulation and single finger electromyography are most accurate tests - repetitive nerve stimulation - decrement is > 10%

chest imaging - in all patients w MG to detect thymus gland abnormalities

30
Q

tx myasthenia gravis

A

myasthenic crisis/severe - plasmapheresis or IV immunoglobulin

long term - acetylcholinesterase inhibitors (pyridostigmine or neostigmine); glucocorticoid as alternative

thymectomy - even if thymus gland is normal can improve sx and removes source of antibodies; useful if no improvement with medical management

31
Q

what meds should ppl w myasthenia gravis avoid

A

BB
aminoglycosides
fluoroquinolones

32
Q

what is multiple sclerosis

A

autoimmune, inflammatory demyelinating disease of the CNS with axon degeneration of white matter (brain and spinal cord)

33
Q

who does MS most commonly affect

A

women and young adults 20-40

34
Q

3 main types of MS

A

relapsing-remitting MC - episodic exacerbations

progressive disease - progressive decline without acute exacerbations

secondary progressive - relapsing-remitting pattern that becomes progressive

35
Q

sx MS

A

sensory disturbances followed by weakness and visual disturbances (diplopia, optic neuritis)

trigeminal neuralgia

uhthoff’s phenomenon - worsening sx w heat

may have bowel or bladder dysfunction

36
Q

PE MS

A

UMN signs - spasticity, upward babinksi, hyperreflexia

lhermittes sign - neck flexion causes lightening shock type pain radiating from spine down the leg

Marcus-gunn pupil - during swinging-flashlight test from the unaffected eye into the affected eye, the pupils appear to dilate

internuclear ophthalmoplegia - inability to adduct the eye not he side of the lesion w nystagmus on the other

cerebellar - Charcot’s neurologic triad - nystagmus, staccato speech, intentional tremor

bladder, bowel, or sexual dysfunction

37
Q

dx multiple sclerosis

A

at least 2 distinct episodes of CNS deficits

MRI with gadolinium - best initial and most accurate - hyper intense white matter plaques; at least 2 ares of white matter involvement

LP if MRI negative - increased IgG and oligoclonal bands

38
Q

tx multiple sclerosis

A

IV high dose glucocorticoids for acute exacerbation

prevention of relapse and progression - beta interferon or glatiramer

39
Q
A

Internal Med EOR (17 decks)

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