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Neuro > Neuromuscular > Flashcards

Neuromuscular Flashcards

0
Q

myopathy

A

condition that is damaging muscles

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1
Q

neuropathy

A

condition that is damaging nerves

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2
Q

neuromuscular junction (myoneural) disease

A

condition that is damaging the neuromuscular junction at the motor end-plate on muscles

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3
Q

dying back

A

refers to a process in which the longest nerve fibers are injured and die

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4
Q

demyelinative

A

refers to a process that damages the myelin sheath

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5
Q

wallerian degeneration

A

happens to an axon after it has been cut off from its cell body
distal end of the cut axon degenerates

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6
Q

epineurium

A

refers to connective tissue wrapping the nerve

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7
Q

perineurium

A

refers to CT wrapping the fasicules within the nerve

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8
Q

endoneurium

A

connective tissue wrapping each individual axon within the nerve

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9
Q

myelin

A

wrapping of cell membranes around an axon that speeds up conduction

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10
Q

entrapment neuropathy

A

condition where nerve is caught between tougher tissues, contributing to chronic irritation and damage

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11
Q

carpal tunnel

A

nerve entrapment of the wrist is the most common site (median nerve)

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12
Q

schwann cells

A

cells that make myelin in the peripheral nervous system

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13
Q

meralgia paresthetica

A

entrapment of the lateral femoral cutaneous nerve at the level of the inguinal ligament

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14
Q

polyneuropathy

A

generalized damage to peripheral nerves; usually effect longest nerve first

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15
Q

charcot marie tooth

A

family of hereditary neuropathies that usually begin to show clinical signs in late childhood and adolecence and progresses slowly

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16
Q

lambert-eaton myasthenic syndrome

A

autoimmune (and often paraneoplastic) condition that produces weakness

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17
Q

paraneoplastic syndrome

A

refers to remote effects of a tumor; most often it represents a condition in which antibodies directed at a tumor damage other tissues of the body

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18
Q

myasthenia gravis

A

condition in which there is antibody-mediated destruction of acetylcholine receptors producing weakness and fatigability of muscle

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19
Q

nerve conduction study

A

test of speed and amplitude of conduction of peripheral nerve fibers

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20
Q

electromyography

A

needle study in which the electrical activity of muscle fibers is recorded; it can detect damage to muscles and is sensitive to muscle fibers that have been disconented from their nerves

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21
Q

sensory receptors

A

transduction

elementary sensory deficit–ataxia, hypesthesia, paresthesia pain

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22
Q

nerve fibers

A

conduction

sensory motor defects/weakness

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23
Q

motor end-plants

A

neuromuscular transmission

simple motor defect- weakness, wasting, muscle twitching, cramps, myotonia

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24
Q

striated muscle fibers

A

contraction

motor defect- weakness, wasting, occasionally pseudohypertrophy

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25
Q

cell bodies of sensory nerve fibers

A

DRG close to spinal cord

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26
Q

somatic motor nerve fibers originate frm

A

motor neurons in ventral horn of spinal cord

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27
Q

where do somatic motor nerve fibers termintae

A

muscle fibers at NMJ or on muscle spindles

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28
Q

why are autonomic nerve fibers unique

A

there are 2 neurons in sequence

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29
Q

where do autonomic motor nerve fibers terminate

A

glands, organs, smooth muscle fibers

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30
Q

fiber Aa

A

large (est) motor axons
muscle strestch and tension sensation
60-100 m/s
15 microns

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31
Q

Ab fibres

A

touch, pressure, vibration, jt position sensation
30-60 m/s
12-14 microns

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32
Q

Ay fibers

A

gama efferent motor axons
15-30 m/s
8-10 microns

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33
Q

Ad fibers

A

sharp pain, very light touch, temperature sensation
10-15 m/s
6-8 microns

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34
Q

B fibers

A

symp preganglionic motor axons
3-10 m/s
2-5 microns

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35
Q

C fibers

A

dull, aching, burning pain and temp sensation

<1 micron

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36
Q

size of axon correlates positively with

A

speed

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37
Q

B and C fibers are not

A

myelinated

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38
Q

all proteins come from the

A

cell body

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39
Q

trophic factors

A 
class of protein compounds that travel in nerve; critical to health of innervated tissues
-- loss of trophic factors; either the death of neurons or atrophy of peripheral tissues
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40
Q

motor unit

A

physiological unit of motor neuron and all the fibers it innervates

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41
Q

fasiculations

A

spontaneous discharge of indiviudal motor axons and contraction of the motor unit from irritation of axons; visible on skin surface as random, involuntary twitches
often normal because of temporary irriation of motor nerve fibers, but persistent fasciculations indicate DAMAGE TO ANTERIOR HORN CELLS OR AXONS

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42
Q

fibrillation

A

spontaneous contraction of individual muscles that is not visible from the skin surface; need electromyography to detect- fairly eliable sign of damage to motor nerver fiebers OR muscle disease

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43
Q

conditions that damage motor units usually result in

A

overall muscle weakness + high firing rates of individual motor units that are still intact

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44
Q

damage to upper motor neurons makes it impossible to

A

reach high firing rates of motor units during voluntary contraction

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45
Q

motor neuron disease

A

general term of conditions that result from degeneration of anterior horn cells (lower motor neurons) and upper motor neurons of the cerebral cortex that give rise to descending tracts taht control movement

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46
Q

upper motor neuron signs

A

spasticity
increased reflexes
upgoing toes

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47
Q

lower motor signs

A

atrophy
fasciculations
decreased reflexes

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48
Q

signs of both upper nad lower motor neuron disease

A

ALS

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49
Q

ALS mainly affects adults

A

40-60 years old

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50
Q

patho of ALS

A

degeneration of anterior horn cells in spinal cord, CN motor nuclei

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51
Q

what is spared in ALS

A

EYE MOVEMENTS

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52
Q

in ALS arms show___ while legs show____

A

LMN signs

UMN signs

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53
Q

prognosis ALS

A

3-5 years

death via weakness of resp musculature + infection

54
Q

primary lateral sclorosis

A

signs only of UMN

55
Q

progressive muscular atrophy

A

signs of only LMN–>over time normal ALS

56
Q

if signs only of cranial musculature

A

progressive bulbar palsy

57
Q

absence of this gave a certain family ALS

A

superoxide dismutase

58
Q

Rilzule

A

slightly delays progression of disease by decreasing glutamate neurotransmission, decreases fast Na channel act

59
Q

Werdnig-Hoffman disease

A

inheritied condition of infants; anterior horn cell degeneration only

60
Q

Kugelberg-Welander

A

inherited condition of children and young adults; anterior horn cell only

61
Q

general symptoms of peripheral neuropathy

A

positive- pain/synesthesia
negative- loss of sensation, weakness, reflexes
irritative- fasciculations/paresthesias

62
Q

etiology of mononeuropathy

A

usually trauma- acute or chronic

63
Q

most common mononeuropathies due to

A

entrapment of nerves at anatomically vulnerable sites

64
Q

Tinel sign of mononeuropathy

A

nerve becomes very mechnically sensitive at site of injury that can be elicited by gentle tapping over the site of injury

65
Q

where does ulnar nerve entrapment happen?

A

elbow

66
Q

ulnar entrapment symptoms

A

weakness and atrophy of the intrinsic hand muscles common

may be sensory loss over the small digits and the ulnar side of the hand on the palmar and dorsal side

67
Q

Signs and symptoms of radial nerve entrapment

A

main part of radial nerve injured–>wrist drop; diminished sensation of hand
superficial branch–>sensory loss on dorsum of hand
deep branch–>weakened extensors of fingers

68
Q

THoracic outlet syndrome

A

heterogenous group of disorders that involve some mechanical compression of structures in upper thorax
subclavian artery/axillary artery–>vascular TOS
lower brachial plexus–>neurogenic TOS

69
Q

etiology of radiculopathy

A

younger indiv- interverebtral disc herniation

older indiv- more often due to degen change sin disc, bone and joints (thickening of tissues, thinning of discs)

70
Q

s/s radiculopathy

A

pain along distribution of nerve root
deeper, constant aching pain
weakness and reflex loss

71
Q

radiculopathy should be considered when

A

symptoms follow a well-defined nerve root dist and whenever distal symptoms are coupled iwth pain int he bacj or neck

72
Q

mononeuropathy multiplex

A

involvement of several isolated nerves

DM polyneuropathy is most common, or any condition causing systemic vasculitis (SLE, polyarteritis, nodsa, lyme disease)

73
Q

Neuralgic amyotrophy

A

aka idiopathic brachial neuralgia, parsonage-turner syndrome

idiopathic conditiosn affecting brachial plexus; need specialty eval if etiology is not clear

74
Q

treatment diabetic poly neuropathy

A

control diabetes

IVIG, steroids

75
Q

polyneuropathy causes

A

DM
high alc–vit B12 def
excess pyridoxine
heavy metals, organic solvents, industrial exposures
chemo, aed, haart
SLE, sjorgen, wegner, polyarteritos (autoimmune)
chronic conditions- tertiary syphillis, lyme disease, hiv
leprosy
metabolic
abnormal blood proteins

76
Q

which nerves affected first

A

longest

77
Q

Guilliane Barrer syndrome

A

immune attack on myelin; may follow viral or diarrheal illness by 1-2 weeks
ascending weakness, rapid course

78
Q

diagnosis GB

A

elevated protein in CSF, no white cells CSF

79
Q

treatment

A

IVIG may shorten clinical course

80
Q

chronic inflammatory demyelinating polyradiculoneuropathy

A

chronic immune-mediated neuropathy
asymmetric weakness and sensory loss
high protein CSF
relapsing.progress poly neuropathy

81
Q

symptom of charcot marie tooth disease

A

high arches/curled toes

82
Q

hereditary motor sensory neuropathy

A

clear pattern of inheritance

83
Q

HMSN 1

A

demyelinative form

84
Q

HMSN II

A

axonal form

85
Q

end plate

A

each muscle has one and only one;a cts as a plug for a nerve terminal branch
Ach is the NT

86
Q

minature end plate potentials

A

intermittent discharges at end-plate region of fairly constant amplitude and duration; not capable of triggering APs

87
Q

Myasthenia Gravis highest incidence in

A

young adult women

small spike in late middle aged men who have thymic tumors

88
Q

pathophys of MG

A

antibodies against nAChRs of NMJ
thymic epithelial cells have surface proteins with epitopes that cros react with AChR at NMJ
decreases post syn AChRs–>decrease safety factor–>APs less likely to fire

89
Q

signs of myasthenia gravis

A

ptosis/diplopia
problems speaking and swalloing
repetitve or sustained use of muscle**–>depeletion Ach–>ultimate failure of conductio

90
Q

progressive generalized weakness in the

A

first two years

91
Q

treatment of MG

A 
thymectomy
corticosteroids
immunosuppressive drugs
human immunoglobulin
plasma exchange
92
Q

Myasthenic syndrome/lambert eaton syndrome caused by

A

MAYBE a carcinoma (bronchogenic small-cell carcinoma)

93
Q

pathophys MS/lambert eaton syndrome

A

nerve impulse–>defective Ca channel fx in presyn membrane–>reduced # quanta release from nerve

94
Q

symptoms MS/Lambert eaton syndrome

A

weakness that tends to improve with exercise–>repetitive stimylation causes facilitation rather than the fatigue seen with MG

95
Q

treatment Ms/LE

A

remove tumor

4-aminipyridine (acetylcholine releasing agents)

96
Q

botulism

A

another well-known and fortunately rare disorder of transmitter release blockade

97
Q

general s/s of myopathies

A

symmetrical, proximal muscle weakness
possible cramping of muscles (with exercise)
rarely symmetrical, aching discomfort in muscles

98
Q

enzyme that proves thereis a muscle disease

A

CK levels

99
Q

other tests

A

EMG

muscle bx

100
Q

muscular dystrophies

A

noninflammatory degenerative conditions of muscles that are genetically determined, not effectively curable, and progressive; classified according to inheritance and distribution of weakness

101
Q

epi of duchenne muscular dystrophy

A

most common muscular dystrophy

sex-linked; affects young boys

102
Q

pathophys MD

A

mutation in gene for the protein named dystrophin

103
Q

Becker dystrophy

A

less severe for of MD; later onset

104
Q

prognosis MD

A

children usually confined to wheel chair by age 10

usually die in second to third decade

105
Q

limb girdle muscular dystrophy

A

heterogenous group of disorders that has proximal leg weakness that usually progresses slowly but may arrest spontaneously
usually appears in adolescence or aduly life with proximal limb weakness

106
Q

Facioscapulohumeral muscular dystrophy

A

weakness and wasting; appears in adolecence or very eary life with weakness of face and muscles attached to scapula nad prox upper limb

107
Q

prognosis facioscapulohumeral muscular dystrophy

A

weakness usually progresses slow, normal life expectancy, mental retardation

108
Q

myotonic dystrophy

A

due to repeats in the sequence of myotonic protein kinase gene
repeats get longer in sequential generations, with earlier onset of symptoms
abnormality of membranes that is not restricted to muscle

109
Q

s/s myotonic dystrophy

A

delayed relaxation of muscles comvined with muscle atrophy
childhood/young adult life
distal
facial and neck early
–>frontal balding, testicular atrophy, diabetes, cardia arrhythmias

110
Q

prognosis myotonic dystrophy

A

progresses slowly

many victims succumb to resp failure and superimposed infection

111
Q

metabolic myopathies-acquired

A

hyper and hypothyroidism

medications-statins, hydroxychloroquine, colchicine, etoh

112
Q

genetic metabolic myopathies

A

difficulties metabolizing carbs or fat (intracellular inclusions)
poorly defined conditions with occasionally myoglorinuria
nemaline myopathy, myotubular myopathy
mitochondiral myopathies

113
Q

mitochondrial myopathies

A

heterogenous group of muscle diseases associated with excessive replication of somewhat defective mito that accumulate in cells
-tend to affect muscle, cns, heart

114
Q

acquired inflammatory myopathy

A

sarcoidosis

certain infectious condtions (HIV/AIDS)

115
Q

autoimmune inflammatory myopathy

A

polymyositis
dermatomyositis
inclusion body myositis

116
Q

polymyositis

A

30-60 years old
insidious onset of symmetrical, proximal muscle weakness, accompanied by muscle soreness

difficulty climb stairs and lift things

117
Q

dx polymyositis

A

elevated inflammatory markers (ESR, CRP)
elevated muscle damage enzymes (CPK)
bx diagnostic (these tests can be normal!)

118
Q

dermatomyositis

A

children 5-15 then later in adulthood (50, 60s)

insidious weakness proximal muscles
red/purple rash–mostly over eyes (heliotrope) and extensor parts of joints (knuckles, elbows, knees, tows)–may also include face, chest, elsewhere

119
Q

treatment dermato and poly

A

immunosuppression

120
Q

inclusion body myositis

A

late middle aged and older individuals

slowly progressive symmetrical myopathy of proximal muscles (cant climb stairs, flexors of fingers and wrist)–very insidious

121
Q

inclusion body myositis dx

A

elevated CK levels
sed rate elevated
muscle bx-cytoplasmic inclusions on muscle biopsy

122
Q

tx inclusion body myositis

A

does not usually respond to immunosuppression (none)

123
Q

2 functional defects with little wasting

A

myotonic disorders

perioidc paralysis

124
Q

myotonic disorders

A

congenital myotonia- Cl transport abnoramlity; symptoms improve with exercise
Paramyotonia- Na channelopathy; worsens with exercise and with cold

125
Q

dx myotonic disorders

A

needle insertion into muscles–>usually easily identified myotonic potentials

126
Q

perioic paralysis

A

thyrotoxic perioidc paralysis
familial perioid paralysis
-generalized/temporary weakness after meals or exercise

127
Q

dx perioid paralysis

A

can be made by provoking weakness with glucose load and exercise

128
Q

large peripheral nerve fibers

A

well localized touch
pressure
vibration
jt position sense

129
Q

small fibers

A

pain
temperature
very light touch
autonomic

130
Q

polyneuropathy

A

distal, symmetrical sensory loss

ankle jerk reflexes effected eraly

131
Q

myopathy

A

proximal
symmetrical
reflexes preserved until late
no sensory loss

132
Q

lambert eaton

A

weakness of hip girdle muscles with decreased patellar reflexes
often with small cell cancer, but can be idiopathic
antibody against voltage-gated Ca channels

Neuro (23 decks)

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