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Clin Med MSKII > High Yield periphersal nerve and skeletal muscle path > Flashcards

High Yield periphersal nerve and skeletal muscle path Flashcards

1
Q
  • family (canned goods)
  • descending paralysis
  • obligate anaerobe, gram +, spore former
  • flaccid paralysis
  • Floppy baby –> ingestion of spores from honey
  • adult –> ingestion of preformed toxin in canned goods
A

Clostridium botulinum

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2
Q
  • paralysis of posterior oral pharynx
  • demyelination
  • can spread to other nerves
  • gram + rod
  • pseudomembrane in throat
  • bulls neck
  • Tellurite and loefler’s media
A

Corynebacterium diptheriae

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3
Q
  • Gram neg. rod, comma shaped
  • can cause Guille Barre (demyelination of peripheral nerves . . ascending paralysis)
  • bloody diarrhea
  • lives in gut of poutry
  • oxidase +
  • can also cause reactive arthritis
A

Campylobacter jejuni

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4
Q
  • acid fast
  • symmetric neuropathy in glove and stalking pattern
  • skin plaques on extensor surfaces
  • lionine faces
A

lepromatous mycobacterium

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5
Q
  • Northeast US
  • Ixodes tick
  • spirochete
A

Borrelia . .lyme disease

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6
Q

stages of lyme disease

A

1: bulls eye rash and flu like symptoms
2; heart block and bilateral bell’s palsy
3; migratory polyarthritis of large joints and mild encephalopathy

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7
Q

what type of neuropathy in DM

A

distal symmetric ascending sensorimotor neuropathy

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8
Q

Bells palsy is often associated with what

A
  • URI

- or diabetes

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9
Q

what type of neuropathy is Guillain-Barre syndrome

A

-Acute inflammatory Demyelinating polyneuropathy . . immune mediated

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10
Q

in pacific islands, what has been associated with a relatively high incidence of Guillain Barre syndrome

A

Zika

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11
Q

Clinical features of Guillain Barre

A
  • Mostly motor: ascending paralysis and areflexia, loss of pain sensation
  • CSF: increase in protein and little inflammatory cells
  • Treat with plasmapheresis and IVIG
  • Small % don’t respond to treatment
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12
Q

Chronic inflammatory demyelinating poly(radiculo)neuropathy is symmetrical mixed sensorimotor polyneuropathy that persists for how long

A

> 2 months

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13
Q

onion bulb neuropathy

A

Chronic inflammatory Demyelinating Poly(radiculo)neuropathy

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14
Q

Diptheria produces an acute peripheral neuropathy associated with what

A

prominent bulbar and respiratory muscle dysfunction

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15
Q

compression of brachial plexus

A

Apex of lung Cx

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16
Q

compression of obturator nerve

A

Pelvic neoplasm

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17
Q

polyradiculopathy involving lower extremity . . compression of what

A

cauda equine by meningeal carcinomatosis

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18
Q

paraneoplastic neuropathy

A
  • anti-Hu antibodies
  • Small cell lung cancer
  • CD8 mediated attack of dorsal root ganglion
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19
Q

POEMS syndrome

A
  • polyneuropathy
  • organomegaly
  • endocrinopathy
  • monoclonal gammapathy
  • skin changes
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20
Q

describe Morton neuroma

A
  • w>M
  • compression neuropathy affecting interdigital nerve at intermetatarsal sites
  • leads to foot pain
  • perineural fibrosis
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21
Q

inherited peripheral neuropathies are often present in who

A

adults

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22
Q

what are the 3 charcot-Marie Tooth diseases . . inherited neuropathies

A

CMT1 (2nd decade) . AD
CMTX . X linked . genes encode connexin32
CMT2 (very early in life) . .AD . . MFN2 gene

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23
Q

Describe hereditary neuropathy with pressure palsy

A
  • PMP22 gene

- compression of individual nerves at sites that are prone to entrapment

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24
Q

Describe the morphologic feature of nerves in Hereditary neuropathy with pressure palsy

A

-swollen, bulbous myelin sheaths at the end of internodes . . . Tomaculi architectures

25
Q

Regardless of the cause, disorders that impair the function of neuromuscular junctions tend to present with what?

A

painless weakness

26
Q

epidemiology of myasthenia Gravis disease

A
  • bimodal age distribution
  • female to male, 2:1
  • young adults
  • in older adults there is males predominance
27
Q

strong association b/t myasthenia Gravis and what abnormalities

A

Thymic . . thymoma or thymic hyperplasia

-usually absent if in older patient tho

28
Q
  • fluctuating weakness that worsens with exertion
  • diplopia
  • ptosis
A

Myasthenia Gravis

29
Q

Lambert-Eaton myasthenic Syndrome is an autoimmune disorder caused by antibodies that block what?

A

ACh release by inhibiting a presynaptic Ca channel

30
Q

how do you distinguish Lambert eaton from Myathenic syndrome

A

Lambert eaton: repetitive stimulation increases muscle response rather than causes fatigue

31
Q

about half of lambert Eaton cases associated with what

A

underlying malignancy most often neuroendocrine carcinoma of lung

32
Q

Type II fiber atrophy with sparing of type I fibers is seen with prolonged use of what?

A

corticosteroids

33
Q

what are the 3 inflammatory myopathies

A
  • polymyositis
  • dermatomyositis
  • inclusion body myositis
34
Q

polymyositis and dermatomyositis show typical features of autoimmune inflammatory diseases, including association with what genotype

A

HLA-DR

35
Q

inflammatory myopathy that is a chronic progressive disease of older patients and associated with rimmed vacuoles

A

inclusion body myositis

36
Q

features of Dermatomyositis

A
  • children and adults
  • latter frequently as paraneoplastic disorder
  • immune damage to small blood vessels and perifascicular atrophy
  • helitrope rash
  • scaling erythematous eruption or dusky red patches over knuckles, elbows, and knees (Gattron papules)
37
Q

autoantibodies and specific presentations of Dermatomyositis

A
  • Anti-Mi2: Gattron papules and heliotrope rash
  • Anti-Jo1: interstitial lung disease, skin rash described as “mechanic’s hands”
  • Anti-P155/P140: paraneoplastic and juvenile cases
  • CD4+ cells
38
Q

this inflammatory myopathy is an adult onset myopathy caused by CD8 cells

A

polymyositis

39
Q

what does polymyositis lack that dermatomyositis has

A

cutaneous features

40
Q

Describe inclusion body myositis

A
  • later adulthood . .older than 50. most over 65
  • most severe weakness in quads
  • dysphagia can occur
  • cN1A
  • elevated creatine kinase
  • rimmed vacuoles on Gomori trichrome stain
41
Q

what is the most common complication of statins

what other things cause muscle toxicity

A

myopathy
-unrelated to dose, cumulative dose, or statin type

  • anti-malarials
  • thyroid disease
  • alcohol
42
Q

response of inflammatory myopathies to steroids and immunosuppressive drugs

A
  • dermatomyositis and polymyositis respond

- inclusion body myositis does NOT

43
Q

congenital myopathy that has defect in ryanodine receptor-1 (RYR!)

A

central core disease

44
Q

the 3 diseases that cause “floppy” infant

A
  • central core disease
  • Nemaline myopathy (NEM)
  • Centronuclear myopathy
45
Q

Duchenne and Becker muscular dystrophy are caused by what

A

LOF mutations in dystrophin gene on X-chromosome

46
Q

morphology for muscular dystrophy

A
  • segmental myofiber degeneration and regeneration with admixture of atrophic myofibers
  • fatty replacement
  • variation in size of myofibers
  • absence of normal sarcolemmal staining pattern in Duchenne
  • Reduced staining in Becker
47
Q

Clinical features in Muscular dystrophy

A
  • boys
  • normal at birth
  • walking delayed
  • clumsiness and poor coordination first noted
  • Pseudohypertrophy of calf muscles
  • wheel chair by age 9.5 (average)
  • death by 25-30
  • cardiomyopathies and arrythmias
48
Q

Treatment for duchenne MD

A

supportive care

49
Q

features of myotonic dystrophy

A
  • AD
  • triplet repeat expansion
  • skeletal muscle weakness
  • cataracts
  • endocrinopathy
  • cardiomyopathy
50
Q

this MD is associated with a characteristic pattern of muscle involvement that includes prominent weakness of facial muscles and muscles of the shoulder girdle

A

Fascioscapulohumeral dystrophy

51
Q

chronic progressive external ophthalmoplegia is a common feature of what disorders

A

mitochondrial

52
Q
  • ragged red fibers

- concentric membranous rings (“phonograph record”)

A

mitochondrial myopathy

53
Q

neuropathic disorder in which loss of motor neurons leads to muscle weakness and atrophy . . floppy infant

A

Spinal muscular atrophy (SMA)

54
Q

morphology of SMA

A

rare hypertrophied myofibers admixed with numerous atrophic rounded myofibers

55
Q

pathogenesis of SMA

A
  • Autosomal recessive

- SMN1 gene

56
Q

synonyms for SMA

A

-Werdnig-Hoffmann disease

57
Q

describe subtypes of SMA and age of onset

A
  • SMA 0: extremely weak at birth. immediate ventilation and don’t breath independently
  • SMA 1: onset prior to 6 months (Werdnig Hoffman)
  • SMA 2: prior to age 1. will sit but never walk
  • SMA 3: after 1 will walk then lose ability
  • SMA 4: before age 10
58
Q

clinical manifestations of ion channel myopathies

A

depending on channel affected may include epilepsy, migraine, movement disorders with cerebellar dysfunction, peripheral nerve disease, muscle disease

Clin Med MSKII (16 decks)

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