neuromuscular disease and disease of the peripheral nervous system Flashcards

(71 cards)

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
type grouping muscle fiber atrophy is a result of TQ
loss of innervation with reinnervation
26
describe type grouping
loss of checkerboard pattern of type 1 and type 2 myofibers TQ
27
Occurs when a type group becomes denervated
Group atrophy
28
what is the rate at which nerves sproat
2mm per day thus very slow
29
tq clincal course of Gullain-Barre
ascending paralysis, increased CSF protein
30
what is hereditary motor and sensory neuropathy 1 known as
charcot-marie-tooth disease
31
mode of inheritance of HMSN1
AD
32
tq symptoms of HMSN 1
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
Progressive muscular atrophy of the calf | peroneal muscular atrophy
HMSN I
34
Presents as distal muscle weakness, atrophy of the calf, or secondary orthopedic problems of the foot (pes cavus)
HMSN I
35
What is the only HMSN that doesnt have onion bulbs
type II, | both type I and III have onion bulbs
36
what is degenerated in hmsn 1
posterior columns of spinal cord
37
what is the charcot-marie tooth diease, but the NEURONAL FORM
HMSN II
38
Charcot-marie-tooth diease HYPERTROPHIC form
HMSN I
39
Autosomal recessive HMSN
III
40
Dejerine-Sottas Disease
HMSN III
41
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
HMSN III
42
Peripheral Neuropathy (Distal/proximal) symmetric sensory or sensorimotor neuropathy is most common
distal
43
what is more common mononeuropathy or polyradiculopathy in DM
polyradiculopathy
44
what is the most effected cranial nerve in monmoneuropathy
occulomotor nerve
45
tq 65% of predialysis patients have evidence of a
uremic neuropathy
46
TQ uremic neuropathy is a (proximal or distal), (symmetric or asymmetric) neuropathy
Distal, symmetric neuropathy
47
what vitamins can cause axonal neuropathys
thiamine deficiency (b3), B12, B6, E
48
what will a thiamin dief cause as far as peripheral neruopathies go
dry beriberi
49
what three antimircobial can cause peripheral neuropathies
isoniazid, chloramphenicol, linezolid
50
what cardiac drugs can cause peripheral neuropathy
amodarone, hydralazine
51
what antineoplastic drugs can cause peripheral neuropathies
cisplatin, carboplatin, vincistine
52
what is the most common site for a Mortans neuroma
Third MTP interspace
53
who is more likely to get a a morton neuroma
females as they were high-heeled shoes
54
mulder click used for
mortan neuroma
55
does a mortans neurom effect a dorsal or plantar nerve
plantar
56
walking on a pebble
mortan neuroma
57
what is the most common cause of carpal tunnel syndrome
compression of the median nerve at the level of the wrist
58
where will you experiance numbness and parethesias in carpal tunnel syndrome
the thumb and first two digits as the median nerve has been compressed at the wrist
59
predisposing factor to carpal runnel syndrome
excess use of the wrist, DJD, hypothriodism, amyloidosis
60
what nerve is typically impinged in a mortan normal
plantar common digital nerve of the 3 webspace
61
Benign encapsulated nerve sheath tumors | composed of cells derived from Schwann cells
Schwannoma
62
Schwannoma | most are genetic or sporadic
sporadic
63
TQ what are schwannomas asscociated with
neurofibromatosis type 2
64
Neurofibromatosis type 2
Autosomal dominant disorder characterized by multiple tumors: schwannoma, meningioma, glioma, ependymoma
65
Association with Neurofibromatosis type 2
Schwannoma
66
(Verocay bodies
• Antoni A which is part of schwannoma
67
hypocellular areas with cells | loosely arranged in a myxoid stroma
Antony B regions
68
Schwannoma: Clinical Features
• Tinnitus, hearing loss
69
Schwannoma • Treatment:
surgical excision
70
When is recurrence common after surgical excision of a schwannoma
if you have NF2
71
When is recurrence common after surgical excision of a schwannoma
if you have NF2