Neuro Dz 1 Flashcards

1
Q

most freq. cause of acute flaccid paralysis

A

GBS

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2
Q

GBS

A

immune mediated disorder directed against myelin or Schwann cells

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3
Q

variants of GBS

A

AIDP
MFS
AMAN
AMSAN

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4
Q

common antecedent illnesses of GBS

A

campylobacter infection

preceded by URI or GI infection

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5
Q

other potential causes of GBS

A

HIV, Zika, influenza, EBV, CMV etc

vaccinations

PCN, OCs, TNFs

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6
Q

epitropic mimicry

A

found in GBS

following illness, immune system goes into overdrive and begins attacking the myelin sheath and/or Schwann cells on peripheral n.

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7
Q

Pathophys of GBS

A

following epitropic mimicry,

demyelination of the nerve and axon degeneration

unable to pass on nerve impulses

all myelinated nerves are affected + protein movement to CSF = edema

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8
Q

GBS epidemiology

A

Bimodal presentation (50-79 y/o, 15-35 y/o)

Men > Women (pregnant women have increased incidence)

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9
Q

GBS presentation

A

symmetric paresthesias
early loss of DTRs
affecting lower extremities first

ascending weakness (can get to muscles of respiration)

severe pain

sensory changes

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10
Q

what is a significant source of mortality in GBS pts

A

autonomic changes + decreased Lung expansion/respiratory failure

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11
Q

autonomic changes in GBS

A

tachycardia –> bradycardia

paroxysmal HTN/HoTN

Urinary retention, ileum, loss sweating, flushing

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12
Q

cranial nerves affected by GBS

A

facial weakness (VII)

impaired EOMs, dysphagia and dysarthria (VI, III, XII, V, IX, X)

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13
Q

natural progression of GBS

A

rapidly progressive weakness and others peak 14-30 days

plateau phase 2-4 weeks (persistent, non progressive)

recovery time is 7 months

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14
Q

steps in diagnosis of GBS

A
  1. CBC/Chem panel
  2. Imaging
  3. LP
  4. Conduction studies
  5. NIP/FEV (staging)
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15
Q

LP of GBS

A

albuminocytologic findings

high protein levels, normal WBCs

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16
Q

tx of GBS

A

hospitalized +/- intubation, supportive care

plasmapheresis OR IVIG (no steroids)

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17
Q

plasmapheresis

A

HD catheter to filter out and remove Ags attacking the myelin

replace plasma with donor plasma so they don’t attack myelin

6-10 tx

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18
Q

IVIG

A

gives pts donor IVIG to decrease body’s production of bad IG cells

“tames immune system”

caution for renal failure, thrombosis

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19
Q

which tx for unstable GBS pt?

A

`IVIG

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20
Q

CIDP

A

chronic (over 8weeks) demyelination and demyelination of nerves + axon degeneration

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21
Q

CIDP s/s

A

fairly symmetric motor and sensory loss

motor > sensory

large and small muscles affected

diminished DTRs globally

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22
Q

CIDP work up

A

Lab work up
LP
imaging
EMG

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23
Q

CIDP tx

A

glucocorticoids
plasmapheresis
IVIG

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24
Q

distinguishing features of CIPD

A
Chronic onset 
motor > sensory 
old men 
not as commonly cranial nerves 
Tx with steroids
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25
Q

distinguishing features of GBS

A
acute onset 
all nerves equally affected 
bimodal presentation 
EOM issues
steroids don't work
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26
Q

myasthenia Gravis

A

autoimmune destruction of nicotinic acetylcholine post synaptic receptors of skeletal muscle

abnormality of T cells and thymic function

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27
Q

pathophys of MG

A
  1. autoAbs block and destroy Ach Receptors
  2. deep folds of motor endplate are flattened = widened gap

THEREFORE: normal Ach release but insufficient number of receptors on muscle is triggered so contraction is weak or non existent

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28
Q

muscles affected by MG

A

skeletal muscles ONLY

no smooth/cardiac muscles

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29
Q

symptoms of MG

A

most commonly affected areas of small skeletal muscle that are used repeatedly

muscle fatigue with REPEATED use due to less contractile force

30
Q

symptoms and staging of MG

A

at beginning - symptoms occur intermittently

eventually = fluctuate in severity, persistent

31
Q

cardinal symptoms of MG

A
  1. ocular
  2. bulbar
  3. facial weakness (snear/snarl)
  4. head drooping
  5. limb weakness
32
Q

ocular MG manifestations

A

ptosis/diplopia are first manifestation

pupils are always spared

33
Q

what factors worsen MG symptoms?

A

end of the day feel more tired

sunlight, surgery, immunizations, emotional stress, heat, menstruation, illness

34
Q

alleviating MG symptoms?

A

rest and relaxation

good self care

35
Q

medications that exacerbate MG

A
  1. Abx
  2. BB
  3. Mg
  4. Prednisone
  5. Anti-cholinergics
  6. Verapamil
  7. lithium
36
Q

PE findings of MG

A

bulbar weakness
weakness of facial muscle
weakness of limbs

37
Q

which part of exam are unaffected by MG?

A

DTRs
Pupillary reflexes
Intact sensation

38
Q

diagnostic tests for MG

A
  1. curtain sign
  2. ice pack test
  3. tensilon test
  4. AcH Ab lab testing
  5. MuskAb testing
  6. repetitive nerve stimulation/fiber MG
39
Q

disease associated with MG

A

thymic hyperplasia/thymoma

must rule out via contrast CT scan of chest or MRI –> locally invasive and removed surgically

associated with other autoimmune dz

40
Q

tensilon test

A

edrophonium (short acting actylcholinestersase inhibitor) given so that they can move the eye and open eye fully

diagnose MG

41
Q

tx of MG

A
  1. Cholinesterase inhibitor
  2. Immunosuppressants
  3. Surgical
42
Q

cholinesterase inhibitors we use MG

A

pyridostigmine (mestinon)

slow down degradation of ACh

43
Q

mestinon

MOA, brand name, dosing

A

Pyridostigmine

individualized dosing

blocks acetocholinesterase, so that it allows Ach to remain in the NMJ for longer

44
Q

mestinon

ADR

A

bradycardia, respiratory suppression, diarrhea, diaphoresis, abdominal cramps/pain

45
Q

immunosuppressants used in MG

A

high dose prednisone
mychophenolate mofetil
azitharoprine

46
Q

high dose prednisone MG

A

begun in hospital bc can exacerbate dz

80% of people are helped while they are on the steroids

slowly taper up

47
Q

Mycophenolate mofetil (Myfortic)

A

alternative to pred, long term EBM data lacking

48
Q

Azathiprine (Imuran)

A

non glucocorticoid drug of choice

interferes with T and B cell production and sustained improvement

slowly working, blood dyscrasia, BBW for malignancy risk

49
Q

surgical thymectomy

A

standard of care 10-55 y/o

thymoma removed (transiently post op period)

may induce remission in some patients

50
Q

general MG care

A

avoid drugs that worsen

considered immunocompromised

pregnancy can worsen (can cross fetus - neonatal)

51
Q

myasthenia crisis

A

severe exacerbation of MG

causes acute respiratory insufficiency due to weakened diaphragmatic and intercostal muscles

should be suspected with a cold, recent medication

admit to ICU

52
Q

tx of myasthenic crisis

A
  1. plasmapheresis

2. IV IG

53
Q

essential tremor

A

MC involuntary movement dz

familial tremor that peaks at age 70

autosomal dominant

54
Q

describe the tremor of essential tremor

A

high frequency, bilateral, symmetric

upper extremities and head

postural and kinetic (pronator drift)

55
Q

tremor will improve with ___ worsen with ___-

A

improve with Alcohol

worsen with stress, strong emotion, fatigue, hunger, temperature extremes

56
Q

treatment of ET

A

most cases pharm doesn’t work

primidone (mysoline)
Propanol (inderal)

57
Q

primidone

A

mysoline
metabolized to phenobarbital but more active

ADRs common on initiation

58
Q

propanolol

A

inderal

younger pts, MOA for tremor unknown,

59
Q

Pseudotumor Cerebri

A

aka idiopathic intracranial hypertension

NOT benign, intractable, disabling, HA, risk of severe, permanent vision loss

60
Q

S/s of Pseudotumor Cerebri

A

Elevated ICP (HA, papilledema, seizures, AMS)

worsened with bearing down or bend over

61
Q

elevated ICP without (pseudo tumor cerebra)

A
  1. space occupying lesions on imaging

2. abnormalities of cerebrospinal fluid composition

62
Q

etiology of Pseudotumor Cerebri

A

obese young women

often idiopathic, but may be vascular, otitis media/chronic mastoiditis, endocrine disturbances, medications)

63
Q

symptoms of Pseudotumor Cerebri

A

HA, visual disturbances, intracranial noise, papilledema, vision loss, sixth cranial nerve palsy

64
Q

work up of Pseudotumor Cerebri

A

CT of head, LP opening pressure

try to eliminate hydrocephalus, mass lesion

65
Q

tx of Pseudotumor Cerebri

A

dc of associated meds, weight loss for pts, evaluation for other dz, pharm tx, serial LP, surgery

66
Q

pharm tx of Pseudotumor Cerebri

A

Acetazolamide (Diamox)
Furosemide
Prednisone

67
Q

surgery options for Pseudotumor Cerebri

A

VP shunt

optic nerve sheath fenestration (vision only, no HA relief)

68
Q

Huntington’s chorea

A

autosomal dominant dz

affects lg motor movements

onset between 30-50, live 20 yrs

abnormal movements, intellectual changes, subtle but progress to severe

69
Q

tx of Huntington’s chorea

A

purely symptomatic

progression can’t be halted

typically Haldol and reserpine to control dyskinesia and behavior

70
Q

Huntington’s chorea workup

A

genetic formation of inclusion bodies in neuron

MRI atrophy of caudate nuclei and putamen