Trigger - PN 2 Flashcards

1
Q

diminished/absent DTRs with proximal muscle weakness and paresthesias moving proximally

A

GBS

also presents with:
pain w minimal movement
ANS dysfunction (tacycardia)
impaired proprioception

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2
Q

Albuminocytologic dissociation in LP

A

GBS
also see:

elevated CSF protein with normal WBC count.
commonly + even just 1 week of onset

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3
Q

CMV and campylobacter jejuni

A

MC viruses in GBS

camp is MC

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4
Q

Thickening and enhancement of intrathecal spine roots and cranial nerve roots

A

GBS MRI brain/spine findings

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5
Q

what is the immunotherapy treatement for GBS

A

plasmapheresis or IVIG

ONLY IN NON AMBULATORY PTS OR IF NOT RECOVERIG IN 4 WEEKS

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6
Q

Antibodies destroy ACh receptors and postsynaptic folds.

A

myasthenia gravis

leads to over release of Ach to compensate. resulting in reduced strength and increased rest time

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7
Q

anti-AChR antibodies (MC)

A

myesthenia gravis

can also see anti-musk antibodies

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8
Q

MC seen with thymic disease

A

myasthenia gravis

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9
Q

What drugs are known to induce or exacerbate MG?

A

ABX (aminoglycosides, polymyxins, cipro, erythro, and ampicillin)
BBs
Verapamil
Mg
Procainamide
Quinidine
Chloroquine
Penicillamine
Statins
Prednisone
Timolol (topical)
Anticholinergics
Lithium
Neuromuscular blocking agents

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10
Q

MC presenting symtpom is ptosis, diplopia or weak EOM

A

myesthenia gravis

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11
Q

what is a positive ice pack test and what does it imply

A

Ice-pack onto closed lid for 2 minutes
Positive response would improve the ptosis.

+ is myesthenia gravis

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12
Q

which antibody suggests a patient is less likely to have thymic disease

A

MuSK antibodies

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13
Q

MC confirming test for myesthenia gravis

A

Repetitive nerve stimulation

+ is smaller EPSP d/t repetitive stimulation

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14
Q

what is the most SENSITIVE test for myesthenia gravis

A

single fiber electromyography

+ is increased interval between 1st and 2nd AP

CI in people who have had botox

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15
Q

pyridostigmine bromide is used when

A

trreatment of myesthenia gravis before eating or prior to muscle fatiguing activities

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16
Q

what are the inheritence type of duchenne and becker muscular dystrophies

A

X linked recessive

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17
Q

markedly reduced or absent dystrophin

A

Duchenne musclular dystrophy

resulting in rapid muscle damage with early use

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18
Q

normal dystrophin but altered shape

A

bekcer muscular dystophy

resulting in later onset and milder course

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19
Q

Gowers sign

A

Using hand support to get off of the floor.

seen in Duchenne Muscular dystrophy

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20
Q

which muscular dystophy has more common cardiac complications

A

BMD

21
Q

Serum CK 10-20x UNL

A

Duchenne or Becker muscular dystrophy

is also raised in carriers but only by 3x UNL

22
Q

What is the first-line diagnostic test for muscular dystrophy?

A

genetic analysis

23
Q

Isolated “opaque” hypertrophic fibers
Replacement of muscle by fat and CT

A

muscle biopsy findings in muscular dystrophy

24
Q

mainstay of therapy for MD

A

glucocorticoids

25
Q

who is eteplirsen used for

A

patients with Duchenne Muscular dystrophy who have mutation leading to exon 51 skipping

26
Q

who is Golodirsen and Vitolarsen indicated in

A

Duchenne muscular dystrophy pts with mutation leading to exon 53 skipping

27
Q

who is Casimersen indicated in

A

Duchenne muscular dystrophy patients who have exon 45 skipping

28
Q

who is delandistrogene moxeparvovec used in

A

duchenne muscular dystrophy boys aged 4-5 years

creates microdystrophin

29
Q

annual Echo and cardiac MRI

A

Muscular dystrophy

Cardiac MRI doesnt start till age 7

30
Q

ACE/ARB use

A

At 10 years old to reduce HF progression in muscular dystophy

31
Q

who takes IV bisphosphonates, and what is the caveat

A

muscular dystrophy patients

must be able to stand upright for 30 min to take

(also take calcium and vit D)

32
Q

Spectrum of disorders due to mutation in myelin gene

A

charcot-marie-tooth

MC type of hereditary neuropathy

33
Q

autosomal dominant

A

charcot-marie-tooth

MC in males

34
Q

presents with pes cavus and foot deformities

A

charcot-marie-tooth

leads to foot drop and slow progressive distal leg weakness

35
Q

inverted champagne bottle legs or stork leg deformities

A

charcot-marie-tooth

36
Q

what confirms dx of charcot-marie-tooth

A

genetic testing

37
Q

Stocking-glove pattern of neuropathy with ABSENT ankle DTRs

A

distal symmetric polyneuropathy

(MC form of DM neuropathy)

38
Q

Amitriptyline
Duloxetine
Pregabalin
Gabapentin

are all treatments

A

distal symmetric polyneuropathy

39
Q

Primary symptom is GI dysfunction leading to gastroparesis

A

autonomic neuropathy

40
Q

when is bethanchol used

A

bladder dysfunction/urinary retention in autonomic neuropathy

41
Q

when is fludrocortisone and midodrine used?

A

autonomic neuropathy

42
Q

where does alcoholic neuropathy originate in the body

A

the feet and toes

43
Q

thiamine supplementation with low doses of TCAs or gabapentin

A

alcoholic neuropathy

44
Q

Chronic alcohol use is the MC risk factor in which disease

A

dry beriberi

also inactive patients with low caloric intakes

45
Q

Symmetric peripheral sensorimotor neuropathy with loss of reflexes, MC in alcoholics

A

dry beriberi

46
Q

Wernicke-Korsakoff syndrome
Wernicke encephalopathy
Korsakoff syndrome

are all assocaited with what disease

A

beriberi

47
Q

lack of what nutrient causes damage to the myelin sheath

A

vit b12

48
Q

antiparietal cell antibodies and intrinsic factor antibodies are seen in which disease

A

pernicious anemia

49
Q

rapid significant weight loss with recurrent vomiting and numbness to the feet after surgery is treated with WHAT

A

parenteral vitamin supplementation especially thiamine.