Trigger - PN 2 Flashcards by Billie Roberts

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

GBS

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

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Albuminocytologic dissociation in LP

GBS
also see:

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

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CMV and campylobacter jejuni

MC viruses in GBS

camp is MC

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Thickening and enhancement of intrathecal spine roots and cranial nerve roots

GBS MRI brain/spine findings

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what is the immunotherapy treatement for GBS

plasmapheresis or IVIG

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

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Antibodies destroy ACh receptors and postsynaptic folds.

myasthenia gravis

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

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anti-AChR antibodies (MC)

myesthenia gravis

can also see anti-musk antibodies

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MC seen with thymic disease

myasthenia gravis

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What drugs are known to induce or exacerbate MG?

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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MC presenting symtpom is ptosis, diplopia or weak EOM

myesthenia gravis

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

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

+ is myesthenia gravis

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

MuSK antibodies

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MC confirming test for myesthenia gravis

Repetitive nerve stimulation

+ is smaller EPSP d/t repetitive stimulation

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what is the most SENSITIVE test for myesthenia gravis

single fiber electromyography

+ is increased interval between 1st and 2nd AP

CI in people who have had botox

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pyridostigmine bromide is used when

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

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what are the inheritence type of duchenne and becker muscular dystrophies

X linked recessive

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markedly reduced or absent dystrophin

Duchenne musclular dystrophy

resulting in rapid muscle damage with early use

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normal dystrophin but altered shape

bekcer muscular dystophy

resulting in later onset and milder course

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Gowers sign

Using hand support to get off of the floor.

seen in Duchenne Muscular dystrophy

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which muscular dystophy has more common cardiac complications

BMD

Serum CK 10-20x UNL

Duchenne or Becker muscular dystrophy

is also raised in carriers but only by 3x UNL

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

genetic analysis

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

muscle biopsy findings in muscular dystrophy

mainstay of therapy for MD

glucocorticoids

who is eteplirsen used for

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

who is Golodirsen and Vitolarsen indicated in

Duchenne muscular dystrophy pts with mutation leading to exon 53 skipping

who is Casimersen indicated in

Duchenne muscular dystrophy patients who have exon 45 skipping

who is delandistrogene moxeparvovec used in

duchenne muscular dystrophy boys aged 4-5 years creates microdystrophin

annual Echo and cardiac MRI

Muscular dystrophy Cardiac MRI doesnt start till age 7

ACE/ARB use

At 10 years old to reduce HF progression in muscular dystophy

who takes IV bisphosphonates, and what is the caveat

muscular dystrophy patients must be able to stand upright for 30 min to take (also take calcium and vit D)

Spectrum of disorders due to mutation in myelin gene

charcot-marie-tooth MC type of hereditary neuropathy

autosomal dominant

charcot-marie-tooth MC in males

presents with pes cavus and foot deformities

charcot-marie-tooth leads to foot drop and slow progressive distal leg weakness

inverted champagne bottle legs or stork leg deformities

charcot-marie-tooth

what confirms dx of charcot-marie-tooth

genetic testing

Stocking-glove pattern of neuropathy with ABSENT ankle DTRs

distal symmetric polyneuropathy (MC form of DM neuropathy)

Amitriptyline Duloxetine Pregabalin Gabapentin are all treatments

distal symmetric polyneuropathy

Primary symptom is GI dysfunction leading to gastroparesis

autonomic neuropathy

when is bethanchol used

bladder dysfunction/urinary retention in autonomic neuropathy

when is fludrocortisone and midodrine used?

autonomic neuropathy

where does alcoholic neuropathy originate in the body

the feet and toes

thiamine supplementation with low doses of TCAs or gabapentin

alcoholic neuropathy

Chronic alcohol use is the MC risk factor in which disease

dry beriberi also inactive patients with low caloric intakes

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

dry beriberi

Wernicke-Korsakoff syndrome Wernicke encephalopathy Korsakoff syndrome are all assocaited with what disease

beriberi

lack of what nutrient causes damage to the myelin sheath

vit b12

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

pernicious anemia

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

parenteral vitamin supplementation especially thiamine.