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LECOM PBL spring 2017 > pathology 1227-1246 (peripheral nerves) > Flashcards

pathology 1227-1246 (peripheral nerves) Flashcards

1
Q

what are the 2 main components of peripheral nerves

A

axons and myelin sheaths

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2
Q

what is the name for the spherical structures that occur within a day of axonal injury

A

myelin ovoids

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3
Q

what is the term for a non-neoplastic haphazard whorled proliferation of axonal processes and associated schwann cells that result in a painful nodule

A

traumatic neuroma

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4
Q

what are some insults that may lead to neuronopathies

A

herpes zoster and toxins like platinum compounds

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5
Q

what do peripheral axonopathies preferentially affect

A

distal extremities

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6
Q

what are neuronopathies likely to affect

A

equally proximal and distal parts of the body

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7
Q

what are common causes of mononeuropathy

A

trauma, entrapment, and infections

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8
Q

what is a common cause of mono neuritis multiplex

A

vasculitis

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9
Q

does guillain-barre occur in the PNS or CNS

A

PNS

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10
Q

what is guillain barre characterized by

A

weakness beginning in the distal limbs that rapidly advances to affect proximal muscle functions

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11
Q

what is the dominant histological finding in guillain barre syndrome

A

inflammation of peripheral nerves, manifested as perivenular and endoneurial infiltration by lymphocytes, macrophages, and a few plasma cells

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12
Q

what is the most common chronic acquired inflammatory peripheral neuropathy

A

chronic inflammatory demyelinating poly(radical)neuropathy

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13
Q

what is chronic inflammatory demyelinating poly(radicalo) neuropathy characterized by

A

symmetrical mixed sensorimotor polyneuropathy that persists for 2 months or more

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14
Q

what is the term for a noninfectious inflammation of blood vessels that can involve and damage peripheral nerves

A

vasculitis

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15
Q

what are some of the neurologic manifestations caused by lyme disease in the 3rd and 4th stages of disease

A

polyradiculoneuropathy and unilateral or bilateral facial nerve palsies

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16
Q

what is the most common cause of peripheral neuropathy

A

diabetes

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17
Q

what is the predominant pathologic finding in individuals with symmetric sensorimotor neuropathy

A

axonal neuropathy

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18
Q

what is the primary event in uremic neuropathy

A

axonal degeneration

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19
Q

what is paraneoplastic sensorimotor neuronopathy most commonly associated with

A

small cell lung cancer

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20
Q

what is morton neuroma marked by

A

perineurial fibrosis

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21
Q

what is CMT1A caused by

A

duplication of a region on chromosome 17 that includes PMP22 gene

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22
Q

what is CMT1B caused by

A

mutations in myelin protein zero gene

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23
Q

what is CMT1X caused by

A

mutations in the GJB2 gene

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24
Q

what does GJB2 gene encode

A

connexin32, which is a gap junction component that is expressed in schwann cells

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25
Q

what is CMT2A caused by

A

mutations in the MFN2 gene

26
Q

what gene causes hereditary neuropathy with pressure palsy

A

PMP22

27
Q

what is lambert-eaton syndrome caused by

A

antibodies that block ACh release by inhibiting a presynaptic calcium channel

28
Q

what is the most common loss of function mutation in congenital myasthenia syndromes

A

gene encoding the epsilon-subunit of the ACh receptor

29
Q

how does botox act

A

by blocking the release of ACh from presynaptic neurons

30
Q

what does Curare block

A

Each receptors

31
Q

what does curare use result in

A

flaccid paralysis

32
Q

what is type 2 fiber atrophy with sparing of type 1 fibers seen with

A

prolonged corticosteroid therapy or disuse

33
Q

when is segmental myofiber degeneration and generation seen

A

when only part of a myofiber undergoes necrosis

34
Q

what is an immunologic disease in which damage to small blood vessels contributes to muscle injury

A

dermatomyositis

35
Q

what is another name for the dilated capillary looks seems in dermatomyositis

A

telangiectasias

36
Q

what do anti-Mi2 antibodies direct against

A

helicase implicated in nucleosome remodeling

37
Q

what do anti-Mi2 antibodies show a strong prominent associated with

A

prominent gottron papules and heliotrope rash

38
Q

what are anti-Jo1 antibodies associated with

A

interstitial lung disease, non erosive arthritis, and skin rash (mechanics hands)

39
Q

what are anti-Jo1 antibodies direct against

A

the enzyme histidyl t-RNA synthetase

40
Q

what are anti-P155/P140 antibodies associated with

A

paraneoplastic and juvenile cases of dermatomyositis

41
Q

what is the most common inflammatory myopathy in children

A

dermatomyositis

42
Q

what is the name for an adult-onset inflammatory myopathy that shares myalgia and weakness with dermatomyositis but lacks its distinctive cutaneous features

A

polymyositis

43
Q

what is a prominent part of the inflammatory infiltrate of the affected muscle in polymyositis

A

CD8-positive cytotoxic T cells

44
Q

where are mononuclear inflammatory cell infiltrates usually located with polymyositis

A

endomysial in location

45
Q

what is the most common inflammatory myopathy in patients older than 65 years

A

inclusion body myositis

46
Q

what is the first line of defense for polymyositis and dermatomyositis

A

corticosteroids

47
Q

what are the leading culprit for toxic myopathies

A

statins

48
Q

what are some common examples of conditions with defects in ECM surrounding myofibers

A

ullrich congenital muscular dystrophy and merosin deficiency

49
Q

what is UCMD characterized by

A

hypotonia, proximal contractors and distal hyperextensibility

50
Q

what is emery-dreifuss muscular dystrophy caused by

A

mutations in genes that encode nuclear lamina proteins

51
Q

what is the triad the EMD is marked by

A

slowly progressive humeroperoneal weakness, cardiomyopathy associated with conduction defects, and early contractures of the achilles tendon, spine and elbows

52
Q

what gene is over expressed in fascioscapulohumeral dystrophy

A

DUX4

53
Q

what is the most common disorder of lipid metabolism to cause episodic muscle damage with exercise or fasting

A

carnitine palmitoyltranferase II deficiency

54
Q

what does the carnitine palmitoyltranferase II deficiency cause impairment of

A

transport of free fatty acids into mitochondria

55
Q

what does myophosphorylase deficiency/ mcArdle disease result in

A

episodic muscle damage with exercise

56
Q

what does acid maltase deficiency impair

A

lysosomal conversion of glycogen to glucose

57
Q

what do mutations affecting KCJN (potassium channel) cause

A

anderson-twail syndrome

58
Q

describe anderson-twail syndrome

A

autosomal dominant disorder associated with periodic paralysis, heart arrhythmia, and skeletal abnormalities

59
Q

what do mutations affecting SCN4A (sodium channel) cause

A

several autosomal disorders with presentations ranging from myotonia to periodic paralysis

60
Q

what is the most common cause of hypokalemic paralysis

A

missense mutations in CACNA1S

61
Q

what do mutations of CLC1 cause

A

myotonia congenita

LECOM PBL spring 2017 (16 decks)

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