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Laughing Neuro > Neuromuscular > Flashcards

Neuromuscular Flashcards

0
Q 
What syndrome / nerve involvement?
pain in arm/forearm
weak thumb, 2nd, 3rd finger flexion
NORMAL sensation
Can't make "ok" sign
A

anterior interosseus syndrome (pure motor nerve branch of median)

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1
Q

What sensory area should be spared in carpal tunnel?

A

sensation over thenar eminence b/c palmar cutaneous branch arises proximal to tunnel.

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2
Q

what might be prolonged on nerve conduction studies for carpal tunnel?

A

median sensory study distal latency (before motor NCS abnormal)
*Both may be normal on routine NCS

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3
Q

Muscle to test to ddx ulnar neuropathy from T1 radiculopathy and why? What movement?

A

test abductor pollicis brevis b/c its innervated by median nerve and would be involved in T1 radiculopathy but not ulnar neuropathy.

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4
Q

What are the 4 branches of the posterior cord?

A

STAR: Subscapular, Thoracodorsal, Axillary, Radial

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5
Q

What does a lesion of the posterior cord cause?

A

sensory loss of posterior arm/hand, weak shoulder abduction, weak extension fingers, wrist, elbows

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6
Q

what nerve is responsible for supination of forearm, brachioradialis reflex and extension of thumb, fingers, wrist, elbow?

A

radial nerve

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7
Q

extensor carpi ulnaris is innervated by what?

A

Radial: all extension! (even though ulnaris is in the name.)

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8
Q

Humerus fracture can affect what nerve?

A

radial

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9
Q

Saturday night palsy affects what?

A

radial neuropathy at spiral groove

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10
Q

What does radial palsy from spiral groove look like?

A

wrist and finger drop, weak supination, lateral dorsal hand sensory loss, SPARED elbow extension b/c triceps innervated before the spiral groove

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11
Q

Ddx radial neuropathy at axilla vs spiral groove

A

axilla will involve the triceps and will cause sensory loss of posterior forearm and arm vs radial groove which spares triceps, and causes sensory loss of hand only.

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12
Q

anterior and posterior interosseus neuropathy have what feature that distinguishes from median/radial neuropathy

A

No sensory loss (motor nerves only)

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13
Q

what does the axillary nerve supply? What functions?

Injured by what?

A

deltoid and teres minor, upper lateral arm sensory

Injured with humerous frx and also shoulder dislocation

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14
Q

nerve responsible for cremasteric reflex, and the roots?

A

genitofemoral L1-L2

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15
Q

what is compressed in meralgia paresthetica?
What does it cause
What causes it

A

lateral femoral cutaneous nerve
causes paresthesias of lateral thigh
from tight clothes, pregnancy, obesity, weights at waist

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16
Q

nerve injured in childbirth and what does it cause?

A

loss of sensation and adduction of medial thigh
Obturator: AAP GOP
Adductor brevis, longus, magnus
Gracilis, obturator externus, pectineus

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17
Q

What sx of complete sciatic paralysis?

A

knee flexion, ankle, toe movement
sensation lateral knee, lateral/post calf, lateral foot, dorsum/sole of foot
ankle reflex

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18
Q

hip surgery, dislocation, fracture, or intramuscular buttock injection can injure what nerve?

A

sciatic

-prob w/ hamstrings, adduction of hip, posterior and lateral sensation

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19
Q

what is piriformis syndrome?

A

sciatic compressed against piriformis and causes buttock pain

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20
Q

what nerve allows you to tiptoe?

A

tibial nerve

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21
Q

What is tarsal tunnel syndrome
What nerve
what sx

A

tibial nerve neuropathy or branches in the tarsal tunnel

Causes burning pain in ankle and foot, and perimalleolar pain worse with weight bearing and at night

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22
Q

crossing legs can result in what nerve injury?

A

superficial common peroneal nerve at fibular head

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23
Q

Which type of muscle are fast, fatigue resistant?

A

2a

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24
Q

which type of muscle fiber are slow and use oxidative metabolism for steady contraction?

A

type 1

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25
Q

What type of muscle fibers are fast and fatiguable but provide big force/

A

2b

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26
Q

episodes of weakness lasting hours or days triggered by large carbo load or exercise is caused by what gene mutation usually and what is the inheritance and name of dz?

A

hypokalemic periodic paralysis
1q31 mutation in Ca channel
AD

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27
Q

what is most likely genetic cause of hyperkalemic periodic paralysis

  • what channel and gene
  • what inheritance
  • when does it present?
A

SCN4A alpha subunit of Na channel on skeletal muscle
periodic paralysis in kids <10y, symmetric lasting min-hrs triggered by rest AFTER exercise or fasting; relieved by carbs and lt exercise
Chr 17q23

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28
Q

hypoK/hyperK match:
better with carbs/after light exercise
worse with carbs/exercise

Na channel
Ca channel

Chr 1
Chr 17

A

HypO K: worse with carbs and with exercise, chr 1q31 for Ca channel

HypER K: beTTER with carbs/REst after exercise, chr 17q23 SCN4A, Na channel

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29
Q

Mutation and channel defect in myotonia congenita

A

muscle chloride channel CLCN1, Chr 7q

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30
Q

Disease triggered by cold with myotonia?

A

paramyotonia congenita

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31
Q

what makes paramyotonia congenita para?

A

it actually worsens with exercise as opposed to the myotonia congenitas that improve with repeated muscle activity
(often eyelids don’t open after first strong blink.)

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32
Q

newborn with hip dislocation, clubfoot, hypotonia, weakness?

Dx and likely genetics/inheritance

A

Central Core disease congenital myopathy

-AD, RYR1 chr 19q13. (also cause malignant hyperthermia)

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33
Q

Patient with hypotonia, muscle weakness, ptosis and extraocular muscle weakness

A

centronuclear myotubulular myopathy (CN- includes CN)

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34
Q

Dx w/ resp distress, severe hypotonia at birth, high arched palate, narrow face?
Path?

A

Nemaline rod myopathy

0muscle EM: rods originate from Z discs

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35
Q

limb girdle weakness and purple eyelid rash and periorbital edema

  • what antibodies
  • what classic path
  • what tx?
A

anti Jo-1 Ab
perifascicular atrophy
steroids tx

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36
Q

what immune systems mediate dermatomyositis and polymyositis respectively?

A

dermato: humoral
poly: T cells

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37
Q

Pt w/ weakness in finger flexors and quads, slightly elevated CK, older man?

A

inclusion body myositis

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38
Q

Classic pathology in inclusion body myositis?

A

endomysial inflammation, eosinophilic cytoplasmic inclusions, rimmed vacuoles**, amyloid deposition

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39
Q

Tx for inclusion body myositis?

A

does NOT respond to immunomodulatory meds

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40
Q

3 classic EMG findings in myositis:

A
  1. incr insertional activity
  2. low amp short duration motor unit action potentials
  3. early recruitment of motor unit action potentials
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41
Q

rimmed vacuoles on muscle bx EM?

A

inclusion body myositis

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42
Q

the muscle disease that affects fingers, distal ue muscles uniquely and quads.

A

IBM (sitting at computer) inclusion body myositis

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43
Q

the myopathy not associated with increased insertional activity on EMG?

A

steroid myopathy (type 2 fiber atrophy)

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44
Q

med that can cause a mitochondrial myopathy with ragged red fibers?

A

AZT/Zidovudine

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45
Q

mild proximal weakness with muscle cramps, myoedema, delayed reflexes, muscle enlargement, myokymia, very elevated CK

Dx?

A

hypothyroid myopathy

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46
Q

characteristics of icu/critical illness myopathy

A

pts on steroids/nmj blockers get flaccid paralysis, areflexia, vent-dependent, loss of myosin, SPARED extraocular muscles and sensation

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47
Q

Difference in bx results in steroid myopathy vs critical illness myopathy

A

steroid: loss of 2b fibers (*see dark type 1 fibers, not light 2 fibers)
critical illness: loss of myosin

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48
Q

Inheritance for congenital muscular dystrophy?

A

AR

49
Q

Inheritance for Facioscapulohumeral muscular dystrophy

A

AD

50
Q

Inheritance for Emery-Dreifuss muscular dystrophy

A

X-linked recessive, AD, or AR (think X linked most likely)

51
Q

Inheritance for oculopharyngeal muscular dystrophy?

A

AD

52
Q

Inheritance for Limb girdle muscular dystrophy

A

AD or AR

53
Q

Differentiate two forms of congenital muscular dystrophy: Merosin positive and negative

A

+: less common, less severe, may ambulate, no white matter abnormalities on T2brain MRI, Chr 6 (MeroSIX)

-: more common, more severe, no walking, progressive contractures

54
Q

Syndromic congenital muscular dystrophy with chromosome 9q31-33 mutation?

A

Fukuyama disease

55
Q

syndromic muscular dystrophy in Finland with defect in POMGnT1 on Chr 1p32?

A

Muscle-eye-brain disease

56
Q

Disease with POMT1 mutation on chr 9q34?

A

Walker Warburg syndrome

57
Q

In general, the syndromic congenital muscular dystrophies are due to what?

A

hypoglycosylated alpha-dystroglycan

58
Q

Distal myopathy which affects gastroc and soleus involving mostly posterior compartment of legs, spares extensor digiti minimi but has very high CK? Genetics?

A

Miyoshi myopathy with defect on chr 2p13

My sushi is a gastronomical delight

59
Q

This muscle disorder results in early elbow and ankle contractures, toe walking, and cardiac abnormalities that can lead to sudden death

A

Emery Dreifuss Muscular dystrophy

60
Q

Humeroperoneal distribution of weakness and heart prob?

A

Emery Dreiffus

61
Q

Popeye effect? Seen in what dz?

A

weak bicep/tricep but strong and big deltoid and distal arm muscles seen in FSHD: fascioscapulohumeral muscular dystrophy

62
Q

FSCH usually presents at what age

A

by age 20y

63
Q

Asymmetric weakness of face, scapula muscles, proximal upper extremities, and foot dorsiflexion.
Can’t whistle, use a straw, close eyes tightly.

A

FSHD

64
Q

Think of marching band guy who can’t hear, scapula wing, he can’t march, he may wear a marching band coat, and he can’t play the trumpet anymore. What disease and genetics?

A

FSHD: scapular winging, hearing loss, AD chr 4q D4Z4 deletion.
Weak mouth/face, Popeye effect, and may be assoc w/ COATS disease: exudate telangiectasia and retinal detachment

65
Q

Autosomal dominant vs recessive limb girdle muscular dystrophy

A

AD: type 1
AR: type 2

66
Q

DX with myotonia, facial and distal weakness, multiple organs involved including apathy, christmas tree cataracts, heart probs, GI / swallow probs, frontal balding, hypersomnia, hypogonadism, hypoventilation, insulin resistance

Genetics?

A

myotonic dystrophy 1: CTG repeat in DMPK on chr 19q13.3 (AD)

67
Q

EMG in myotonic dystrophy?

A

myotonic discharges, myopathic muscle fiber action potentials

68
Q

path in myotonic dystrophy

A

type 1 fiber atrophy, increased central nuclei

69
Q

likely dx w/ neck weakness, temporal/masseter weakness, ptosis, dysphagia, weak eye and mouth closure, other organs involved

A

myotonic dystrophy type 1

70
Q

What is the most common myopathic disorder to present in neonatal period and what is the genetics?

A

congenital myotonic dystrophy with >1000 CTG repeats on chr 19q13 due to anticipation. Mom might have myotonic grip

71
Q

Neonatal EMG in congenital myotonic dystrophy

A

does not yet show myotonia

72
Q

What distinguishes myotonic dystrophy type 1 from type 2?

A

Type 2 is known as proximal myotonic myopathy (PROMM) and is primarily proximal and can have calf pseudohypertrophy. LEss cardiac/lung involvement, less atrohphy. Is a tetranucleotide repeat of CCTG (extra C: closer to proximal)

73
Q

Asymmetric ptosis, progressive extraocular muscle weakness without diplopia and mild neck/prxoimal limb weakness.

A

oculopharyngeal muscular dystrophy

74
Q

genetics of oculopharyngeal muscular dystrophy

Muscle path?

A

autosomal dominant GCG repeat in PAPB2 on chr 14q11.
(GCG: gag choke gag from dysphagia)

bx shows rimmed vacuoles and fiber size variation

75
Q

episodes like Reye’s syndrome, progressive weakness, cardiomyopathy
Dx and what does the muscle bx show?

A

primary systemic carnitine def.

bx shows lipid storage

76
Q

most common metabolic cause of recurrent myoglobinuria?

A

CPT-II deficiency on chrom 1

see pee tea with too much exercise

77
Q

The glycogen storage diseases all have what inheritance and what is the exception?

A

all autosomal recessive except phosphoglycerate kinase deficiency which is X-linked recessive

78
Q

What is another name for acid maltase deficiency?

A

Pompe disease / Glycogen storage disease type 2

79
Q

This disease starts in 20-30s and p/w fatigue, leg/trunk weakness and respiratory failure and myotonia in paraspinal muscles
Dz, CNS risk, and bx findings?

A

Acid maltase deficiency, intracranial aneurysms due to glycogen deposition, vacuolar myopathy on bx

80
Q

second wind phenomenon?

A

McArdle’s disease Glycogen storage disease type V

81
Q

2 diseases with exercise intolerance and myoglobinuria?

How to distinguish?

A

Mcardle dz AND

TArui disease / phosphofructokinase def GSD VII and abnl FILT test, but NO second wind phenomenon

82
Q

Classic emg / ncs finding in infantile botulism

A

posttetanic facilitation and absence of posttetanic exhaustion

83
Q

repetitive stim at what Hz causes potentiation in botulism?

A

20-50 (Hi freq)

84
Q

dz w/ dry mouth and proximal muscle weakness in which strength temporarily increases with exercise?

A

LEMS

85
Q

CMAP decrement with repetitive stimulation at 2-5Hz?

A

Seen in Myasthenia

86
Q

increased jitter and blocking on single fiber EMG in what?

A

Myasthenia

87
Q

what major meds should be avoided in myasthenia?

A

5 major: aminoglycosides, procainamide, quinine/quinidine, magnesium, beta blockers

88
Q

pts w/ anti-MUSK instead of anti-ACHR Ab in myasthenia have what treatment features?

A

less response to anticholinesterase meds and htymectomy less helpful

89
Q

In a pt w/ GBS looking picture but Miller fisher variant with ophthalmoplegia but NORMAL CSF, think what?

A

tick paralysis

90
Q

dz with hypohidrosis, decreased tears and saliva, GI issues and impotence and pain
Dx and defect?

A

Fabry’s alpha-galactosidase def

91
Q

syndrome that may be assoc w/ carpal tunnel that also involves heart probs and lower limb pain, and autonomic difficulties?

A

Familial amyloid polyneuropathy (FAPO) from transthyretin defect

92
Q

CMT 1: type of neuropathy and inheritance?

A

demyelinating and AD

93
Q

CMT II: type of neuropathy and inheritance

A

Axonal AR

94
Q

Inheritance and type of neuropathy of CMT III

A

Demyelinating AR

95
Q

Deletion vs duplication of CMT I and HNPP

A

Deletion of PMP-22 in HNPP
Duplication of PMP-22 in CMT IA

(Duplication: 2 of them, two abnormal pes cavus feet)

96
Q

Multifocal motor neuropathy with conduction block is known fo what clinical feature presentation and what lab test?

A

asymmetric distal weakness and atrophy in UE

Anti-GM2 antibodies

97
Q

What is POEMS?

A 
Polyneuropathy
Organomegaly
Endocrinopathy
M protein (monoclonal gammopathy)
Skin changes
98
Q

What is the deficiency in Tangier disease and what is the genetics? What are the sx? What do labs / bx show?

A

ABCA1 on chr 9q31, deficiency of alpha lipoprotein (dx w/ very low HDL)
sensory neuropathy of distal upper extremities and atrophy of intrinsic hand muscles, large orange tonsils, LAD/splenomegaly
(hold a tangerine in your hand where atrophy occurs)
Path: macrophages w/ cholesterol esters

99
Q

upper trunk plexopathy? presentation?

A

Erb-Duchenne, waiter tip

100
Q

Lower trunk plexopathy? and sx?

A

lower trunk rare plexus injury with claw like deformity and possible Horner’s

101
Q

What are the features (2) of radiation induced plexopathy?

A

prominent pain and myokymia

102
Q

First EMG finding in AIDP?

A

LOss of F waves

103
Q

What is a poor prognosis AIDP, and what are the markers and associations?

A

AMAN: acute motor axonal neuropathy. Assoc w/ Campylobacter, anti-GD1 and anti-GM1 ABs

104
Q

What is the Miller Fischer variant of AIDP/GBS?

A

triad of opthalmoparesis, ataxia, areflexia

105
Q

Dx in patient with significant weight loss, pain, asymmetric proximal weakness of quads and psoas predominantly

A

diabetic amyotrophy

106
Q

Characteristic EMG of radiculopathy?

A

even though sensory complaints in dermatomal distribution, the SNAPs are normal on EMG/NCS w/ lesions proximal to the =DRG

107
Q

Acute radiculopathy might show what in the first 2 weeks only on EMG/NCS

A

fibrillations in paraspinal muscles

108
Q

UMN/LMN disease SPARING extraocular muscles and sensation?

A

ALS

109
Q

What do you see on pathology of ALS?

A

loss of motor neurons in cord, brainstrem sparing extraocular; loss of Betz cells, fiber-type grouping and muscle atrophy, atrophy of motor and anterior roots of spinal cord

110
Q

Bunina bodies are what and seen in what?

A

eosinophilic cytoplasmic inclusions in ALS

111
Q

Med for ALS and what needs to be monitored?

What is proposed mechanism?

A

Riluzole, watch LFTs

inhibits relase of glutamate, and there are high glutamate levels in CSF in ALS

112
Q

Gene for familial ALS?

A

SOD1 gene on chr 21q22.1

113
Q

What is Fazio-Londe syndrome? features?

A

juvenile progressive bulbar palsy with facial weakness, dysarthria, dysphagia, lingual fasciculations (think of face fonze / londe: funny speech in london)

114
Q

Another name for spinobulbar atrohpy?

A

Kennedy’s X-linked spinobulbar muscular atrophy

115
Q

What causes Kennedy’s disease genetics, and what is the clinical presentation? major DDX?

A

CAG expansion of Androgen receptor gene
muscle cramps, face weakness/fasciculations, proximal limb weakness, dysarthria/swallowing issues, decr reflexes, hormonal issues.
DDX ALS but NO UMN signs

116
Q

Gene for SMA?

A

5q11

117
Q

SMA that doesn’t walk but sits?

A

SMA II

118
Q

Type of SMA with walkers, when do they present?

A

after 18 mos, often get scoliosis

119
Q

Most common cause of recurrent myoglobinuria?

A

Carnitine palmitoyltransferase deficiency

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