Neuromuscular Flashcards

1
Q

Dermatomes

A

TETA (T4) OmbligO (T10), piiiito groin (L1), culo5 (S5)

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2
Q

Radial at axilla

A

crutches/Sat night palsy, post arm numb (post cut nerve), triceps weak

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3
Q

Radial spiral groove

A

fx, triceps ok, post arm sens ok, imp lateral upper arm sens (lat cuta nerv, branch above elbow)

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4
Q

Radial

A

BEST (Brachioradialis [hammer curl], Extensors of forearm, Supination, Triceps) + sens post arm

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5
Q

Ulnar Guyon

A

pinky paresthesia, weak FAbd. Sens hypothenar eminence preserved [palmar cutaneous branch proximal], preserved pinky and ring flex [musc proximal to wrist)

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6
Q

Ulnar elbow

A

+ paresthesia in palm, weak pinky and ring flex

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7
Q

Median function

A

THENAR EMINENCE + pronator teres +prox finger flex. Benedictine (flex ½ digits),

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8
Q

Median ant inteross

A

PURE MOTOR, can’t make OK sign only pulps touch, HD fistula

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9
Q

Pronator teres

A

“golfers elbow” pain in forearm worse with pronation, weak median nerve NOT pronator teres (does the compression

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10
Q

Musculocutaneous

A

shoulder dislocation, brachialis/biceps brachi (EF and supination when flexed only), sens lateral forearm above wrist

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11
Q

Axillary

A

fx surgical neck of humerus, sens tattoo area, deltoid (after 30deg abd, arm flex)

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12
Q

Femoral function

A

HF (before inguinal canal), KE, sens ant/medial thigh

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13
Q

Sciatic function

A

hamstring, KF

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14
Q

Peroneal

A

DF, AEv, sens ant/lat shin/foot

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15
Q

TIbial

A

PF + FIn

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16
Q

Tarsal tunnel

A

tibial burning plantar, sens loss sole, AJ ok, no motor

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17
Q

Foot drop ddx

A

deep peroneal (DF, AEv) [PED = Peroneal Everts Dorsif], L5 (+ AIn and Habd), Sciatic (+AIn +PF)

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18
Q

AMAN

A

GM1, GD1a

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19
Q

MMN

A

GM-1

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20
Q

Familial amyloid

A

transthyretin mut TTR, 1 (autonomic), 2 (CTS), rx. Patisiran (siRNA), inoteseren (ASOligo), tafamidis/difusinal stabilizers

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21
Q

CMT1A

A

demyelinating, Mild, dup PMP22 ch 17

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22
Q

CMT1B

A

demyelinating, Severe, myelin protein 0 mut

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23
Q

CMT3

A

demyelinating, Dejerine Sottas sx, most severe, hypertrophy of nerves

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24
Q

CMT2

A

Axonal- 2A2 optic atrophy, 2B foot ulcers, 2C vocal cord paralysis

25
Q

CMT4

A

combined demylinating and axonal

26
Q

Giant axonal neuropathy

A

GAN gene, intermediate filament, large focal axonal swelling, walking inner edge of feet, curly hair, UMN, optic atrophy

27
Q

Refsums

A

peroxisomal, phytanic acid accumulation, retinitis pigmentosa, cardiomyopathy, skin, diet low in phytanic

28
Q

Tangiers

A

orange tonsils, high TG low LDL/HDL, ABCA1 (ATPase triphosphate cassette transporter protein)

29
Q

HSAN1

A

young adult, disociated sens loss pain/temp>vib

30
Q

HSAN2

A

infant generalized pain loss, mutilation risk

31
Q

HSAN3

A

autonomic

32
Q

HSAN4

A

congenital pain insensitive, cognitive delay

33
Q

IBM

A

asymmetric wrist FF quad and ant tib weak, RIMMED VACUOLES

34
Q

HypoK PP

A

AD, 1 (CACNA1S Ca channel gene mut) 2 (SCN4A Na channel), weakness w/ hyporeflexia, high CK low K, trigger exercise, carb meal, etoh, cold, emotional, given gluc to trigger

35
Q

HyperK PP

A

SCN4A, trigger by rest after exercise and fasting, give glucose for ppx, give K to trigger

36
Q

Centronuclear myopathy

A

floppy baby, PTOSIS OCULOPALSIES, pharynx

37
Q

DM1

A

distal weak, CTG (CUT THE GRIP) exp in myotonic dystrophy protein kinase DMPK, FRONT BALD, temporalis atrophy

38
Q

DM2

A

proximal weak, CCTG expansion in zinc finger 9

39
Q

FSH

A

D4Z4 deletion, delt spared

40
Q

Oculopharyngeal

A

AD, french-canadian “eh” GCX(any) exp in polyA, dysphagia, dysphonia, ptosis, path rimmed vacuoles, intranuclear tubular filaments

41
Q

Myotonia Congenita

A

Thomsens (milder, kids), Beckers (severe, teens), warm up phenomenon (exercise improves), CLCN1 mut, anesthesia prolonged, rx mexiletine

42
Q

Paramyotonia

A

SCN4a channelopathy, exercise accentuates myotonia (“para”-doxical)

43
Q

Nonaka

A

foot drop, rimmed vacuoles, tubular filaments (similar to IBM)

44
Q

Miyoshi

A

dysferlin or ANO5, post distal leg weak

45
Q

Central core

A

Ryanodine receptor mut (malig hyperthermia risk)

46
Q

Emery-Dreifuss

A

Emerin/Lamin a/c, contractures elbows/knees, arms weak first, IQ ok, HEART (conduction)

47
Q

Congenital musc dyst Collagenopathies

A

collagen VI, contractures, distal hyperlaxity, calcanei protrusion
Ullrich’s - Severe
Bethlem - Mild

48
Q

Laminin-a-2

A

Congenital musc dyst Merosinopathies - eyes spared, intelligence ok

49
Q

Congenital musc dyst Dystroglycanopathies

A

path low dystroglycan
Fukuyama congenital musc dystrophy - common in Japan, fukutin mut, eye-brain involved, sz
Muscle-eye-brain
Walker-Warburg - more severe muscle-eye-brain

50
Q

MELAS

A

polymerase gamma mut

51
Q

Neurogenic muscle weakness, ataxia and retinitis pigmentosa

A

adenosine triphosphate 6 mut

52
Q

Kearns Sayre

A

retinitis pigmentosa, ophthalmoplegia, cardiac conduction, myopathy, ataxia, hearing loss

53
Q

Kennedy

A

LMN, X, CAG exp androgen receptor, GINECOMASTIA and hypogonadism/sterile, spinobulbar muscular atrophy, face fasics, tremor

54
Q

SMA

A

ant horn cell degeneration, survival motor neuron 1 mut, 1 infantile (neonatal resp failure, dead by 2), 2 intermediate (1-2yo, tremor, contractures, dont walk), 3 juvenile (5-15yo, proximal weak, dif walking but walk, tremor/fasics), 4 adult onset (QUADRICEPS, proximal, CK high). Path entire groups of fascicle atrophy and neighbor hypertrophy, EMG myopathic, NCS nl.

55
Q

PLS

A

UMN 3y w/o LMN, spastic tetrapresis, can be bulbar too

56
Q

HIV myelopathy

A

vacuolar myelopathy, lateral and post column demyelination

57
Q

HSP

A

pure (only LE spasticity), complicated (optic neurop, deaf, neuropathy, dementia, etc). SPAST (spastin), alastin, paraplegin, spartan, maspardin

58
Q

Watershed cord

A

T4-8