neuromuscular disease and disease of the peripheral nervous system Flashcards

1
Q

medial nerves of the motor unit innervate

A

proximal muscles

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2
Q

lateral nerves of the motor unit innervate

A

distal muscles

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3
Q

what makes mylein in the peripheral nervous system

A

schwann cells

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4
Q

how does myelin in the pns differ from that of the CNS

A

pns myelin contain a high proportion of sphingomyelins and glycoproteins

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5
Q

what to myelin products are specfic to the pns

A

myelin protein zero, and myelien basic protein

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6
Q

in the pns what is the ratio of schwann cells to myelinated axon internodal segments

A

1 to 1

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7
Q

what is the ratio of schwanna cells to unmyelinated axons

A

5-20 per unmyelinated axon

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8
Q

connective tissue enclosing the entire nerve contains blood vessels

A

epineruium

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9
Q

encloses each nerve fascicle form a tight blood nerve barrier

A

perineruium

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10
Q

encloses individual nerve fibers

A

endoneurium

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11
Q

Tight junctions between perineural cells

A

perineural barrier

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12
Q

Tight junctions between cells that form the outer layer of
the arachnoid membrane which fuse with the perineurium
of the roots and cranial nerves as they leave the arachnoid
space

A

brain csf barrier

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13
Q

Tight junction formed by endothelial cells in capillaries

derived from vasa nervorum

A

blood- brain barrier

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14
Q

how to axons get protein

A

Protein synthesis does not occur in the axon
• Axoplasmic flow delivers proteins and other
substances from the cell body

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15
Q

what allows your body to be so warm

A

skeletal muscle provides us with much of our heat

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16
Q

Slow-twitch

A

type 1

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17
Q
  • High in myoglobin and oxidative enzymes
  • Many mitochondria
  • Fatigue resistant
  • Red
  • Scant glycogen, abundant lipids
A

Type 1

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18
Q

type one action

A

sustained force, weight bearing

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19
Q

TQ Increase with androgenic steroids

type one or two muscle

A

type 2

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20
Q

fast twich

A

type 2

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21
Q

what determines the myofiber type

A

the motor neuron

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22
Q

Sequential episodes of demyelination and
remyelination leads to a concentric layering of
schwann cell processes (onion bulb formation)

A

segmental demyelination TQ

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23
Q

what happens when skeletal muscle becomes separated from

the LMN

A

atophy

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24
Q

tq what happens in atrophy

A

Myofibers become angular, compressed by surrounding

normal myofibers

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25
Q

type grouping muscle fiber atrophy is a result of TQ

A

loss of innervation with reinnervation

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26
Q

describe type grouping

A

loss of checkerboard pattern of type 1 and type 2 myofibers TQ

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27
Q

Occurs when a type group becomes denervated

A

Group atrophy

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28
Q

what is the rate at which nerves sproat

A

2mm per day thus very slow

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29
Q

tq clincal course of Gullain-Barre

A

ascending paralysis, increased CSF protein

30
Q

what is hereditary motor and sensory neuropathy 1 known as

A

charcot-marie-tooth disease

31
Q

mode of inheritance of HMSN1

A

AD

32
Q

tq symptoms of HMSN 1

A

progressive muscular atrophy of the calf (peroneal muscular atrophy)

Presents as distal muscle weaknes, atrophy of the calf, or secondary orthopedic problems of the foot (pes cavus)

33
Q

Progressive muscular atrophy of the calf

peroneal muscular atrophy

A

HMSN I

34
Q

Presents as distal muscle weakness, atrophy of
the calf, or secondary orthopedic problems of
the foot (pes cavus)

A

HMSN I

35
Q

What is the only HMSN that doesnt have onion bulbs

A

type II,

both type I and III have onion bulbs

36
Q

what is degenerated in hmsn 1

A

posterior columns of spinal cord

37
Q

what is the charcot-marie tooth diease, but the NEURONAL FORM

A

HMSN II

38
Q

Charcot-marie-tooth diease HYPERTROPHIC form

A

HMSN I

39
Q

Autosomal recessive HMSN

A

III

40
Q

Dejerine-Sottas Disease

A

HMSN III

41
Q

HMSN that Begins in EARLY childhood
– Delay in acquisition of motor skills
– Distal weakness is accompanied by truncal weakness
– Areflexia
– Enlarged, often palpable, peripheral nerves

A

HMSN III

42
Q

Peripheral Neuropathy
(Distal/proximal) symmetric sensory or sensorimotor
neuropathy is most common

A

distal

43
Q

what is more common mononeuropathy or polyradiculopathy in DM

A

polyradiculopathy

44
Q

what is the most effected cranial nerve in monmoneuropathy

A

occulomotor nerve

45
Q

tq 65% of predialysis patients have evidence of a

A

uremic neuropathy

46
Q

TQ uremic neuropathy is a (proximal or distal), (symmetric or asymmetric) neuropathy

A

Distal, symmetric neuropathy

47
Q

what vitamins can cause axonal neuropathys

A

thiamine deficiency (b3), B12, B6, E

48
Q

what will a thiamin dief cause as far as peripheral neruopathies go

A

dry beriberi

49
Q

what three antimircobial can cause peripheral neuropathies

A

isoniazid, chloramphenicol, linezolid

50
Q

what cardiac drugs can cause peripheral neuropathy

A

amodarone, hydralazine

51
Q

what antineoplastic drugs can cause peripheral neuropathies

A

cisplatin, carboplatin, vincistine

52
Q

what is the most common site for a Mortans neuroma

A

Third MTP interspace

53
Q

who is more likely to get a a morton neuroma

A

females as they were high-heeled shoes

54
Q

mulder click used for

A

mortan neuroma

55
Q

does a mortans neurom effect a dorsal or plantar nerve

A

plantar

56
Q

walking on a pebble

A

mortan neuroma

57
Q

what is the most common cause of carpal tunnel syndrome

A

compression of the median nerve at the level of the wrist

58
Q

where will you experiance numbness and parethesias in carpal tunnel syndrome

A

the thumb and first two digits as the median nerve has been compressed at the wrist

59
Q

predisposing factor to carpal runnel syndrome

A

excess use of the wrist, DJD, hypothriodism, amyloidosis

60
Q

what nerve is typically impinged in a mortan normal

A

plantar common digital nerve of the 3 webspace

61
Q

Benign encapsulated nerve sheath tumors

composed of cells derived from Schwann cells

A

Schwannoma

62
Q

Schwannoma

most are genetic or sporadic

A

sporadic

63
Q

TQ what are schwannomas asscociated with

A

neurofibromatosis type 2

64
Q

Neurofibromatosis type 2

A

Autosomal dominant disorder characterized by
multiple tumors: schwannoma, meningioma, glioma,
ependymoma

65
Q

Association with Neurofibromatosis type 2

A

Schwannoma

66
Q

(Verocay bodies

A

• Antoni A which is part of schwannoma

67
Q

hypocellular areas with cells

loosely arranged in a myxoid stroma

A

Antony B regions

68
Q

Schwannoma: Clinical Features

A

• Tinnitus, hearing loss

69
Q

Schwannoma • Treatment:

A

surgical excision

70
Q

When is recurrence common after surgical excision of a schwannoma

A

if you have NF2

71
Q

When is recurrence common after surgical excision of a schwannoma

A

if you have NF2