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Neurology > Neuropathy and Myopathy > Flashcards

Neuropathy and Myopathy Flashcards

1
Q

Riley Day Syndrome

A

Inherited neuropathy, Familial Dysautonomia, mutations in IKBKAP gene on chromosome 9

cyclic vomiting, hyperhidrosis, orthostatic hypotension
no cervical ganglion or lateral horns

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2
Q

Autonomic symptoms

A 
Riley Day
Fabry
Amyloid Neuropathy
Shy Dragger
Lambert Eaton 
Tetraplegia C4-5
Acute Autonomic paralysis (ACTH antibody)
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3
Q

Ondines Curse

A

loss of autonomic breathing in sleep
need to stay awake

Medullar pathology, Listeria, Leigh

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4
Q

Distal Myopathy

A

Miyoshi - Dysferlin (like LGMD 2B) AR
Nonaka - Vacuoles - AR
Welander Distal dystrophy
Desmin Myopathy - Desmin

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5
Q

Congenital MD

A

Merosin Deficiency

Fukutin with IQ def (also as LGMD2I prox

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6
Q

LGMD 1A

A

Myotilin
Distal
Vocal cord
Pharynx

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7
Q

LGMD 1B

A

Lamin

contractures and heart

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8
Q

LGMD 1C

A

Caveolin

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9
Q

LGMD 2A

A 
Calpain
prox dist
CPK
contractures
AD
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10
Q

LGMD 2B

A

Dysferlin like Miyoshi but prox

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11
Q

LGMD 2C-F

A

Sarcoglycan

Becker like with Hypertrophy and heart

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12
Q

Myotonic Dystrophy

A 
CTG repeat DM1
Arrhythmia
distal 
Chr 19
DMPK Gene
Ptosis non ocular
Cataract 
Testicles
balding
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13
Q

PROMM

A

DM2

CCTG repeat prox weakness

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14
Q

Fascioscapular

A 
shorter than the WT
Telomer 4q
D4Z4
prox
facial scapula DELTOID
ant tibial 
hearing
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15
Q

Oculopharyngeal

A

GCG (expension from 10 normally to 11-17
AD
Progressive ptosis (drooping of eyelids) and weakness of the extraocular muscles is the initial clinical finding.. Dysphagia (difficulty swallowing) and proximal limb weakness

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16
Q

Primary Erythromelalgia

A

Red Hands, Nav1.7 mutation hypersensitive

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17
Q

SCN9A mutation

A

painlessness

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18
Q

Familial Amyloid PN

A

AD
Transthyretin - autonomous, GI, Heart, lancinating pains
ApoA1 - early renal involvement
Gelsolin - finish type - corneal clouding and cranial nerves, baggy skin

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19
Q

Painful Neuropathy

A

Toxic: Alcohol, Thallium, Arsen
Immune:GBS, IgM, CASPR2, Sjoegren, Anti-Hu, Cryoglobulin, HIV, Vasculitis
Hereditary: Fabry, GM2, SCN9A, Amyloidosis, Porphyria, Eryhthromelalgia
Metabolic: DM, Pellagra, beriberi,
Polio

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20
Q

Tangiers Disease

A 
Familial alpha-lipoprotein deficiency
ABCA1 transporter CHr. 9
Cholesterol deposits
Yellow tonsils, corneal cloudin, splenomegalie
pain and temp loss polyneuropathy
face arm upper trunk syringomyalia like
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21
Q

GBS

A 
0.4-1.7 per 100000
C jejuni 30%
more females than males
5% respiratory failure
50% pain
urinary retention 15%
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22
Q

AMAN

A 
GBS variant
C jejuni in 67%
more in mexico Japan
Arms more than Legs
Respiratory failure uncommon
No sensory loss
GM1 GM1b GD1
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23
Q

GBS Variants

A

Variants:
AMAN
AMSAN acute MOTOR SENSORY axonal neuropathy
Pharyngeal Cervical Brachial variant and opthalmoplegia
Complete ophthalmoplegia and ataxia and areflexia: Miller Fisher

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24
Q

Miller Fisher

A 
80% Diplopia start, Myalgia, Ataxia
external ophthalmoplegia and pupillary dysfunction 42%
Ataxia
Areflexia
 GQ1b or GT1b Antibodies

if drowsiness and Brainstem or long tracts: BICKERSTAFF

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25
Q

Critical Illness Neuropathy

A

motor type neuropathy
bacterial sepsis
Axonal

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26
Q

Acute Uremic PN

A

subacute acute like subacute GBS

demyelinating

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27
Q

Acute Sensory Ganglionopathy

A 
ataxia, areflexia, numbness, pain
from face to all of the body
CSF protein elevated
- drugs
- autoimmune
- toxic
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28
Q

Diphtheria

A

Exotoxin:: acute pharyngeal and laryngeal
muscles
A and B toxin: inhibits protein synthesis
Neck edema, headache, Laryngitis
Neuropathy 1:
blurred vision: accomodation defect, nasal speech, Diaphragm
very rare extraocular muscle weakness

NP 2: 
8-12 weeks later:
ascending weakness: sensorimotor NP
weakness, paraesthsia, vibration sense loss, 
demyelinating
CSF protein elevated
symptomatic treatment
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29
Q

Porphyric NP

A 
severe rapid symetric motor PN - axonal
abdo pain psychosis
seizures
AD
CSF protein normal
Treatment: GLUCOSE AND HEMATIN
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30
Q

Porphyria Treatment

A

Glucose and Hematin

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31
Q

Thallium NP

A 
GBS like - AXONAL
or acute sensory
abdo pain vomiting diarrhea
tingling areflexia, 
acral pain
cranial nerves
high CSF rotein
Rapid Alopecia
Treatment: KCL
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32
Q

Arsenic

A

Garlic Smell
Neuropathy vomiting GI Tachycardia, 10-20 delayed neuropathy sensory than motor large fibres, axonal
CHELATION

Hyperkeratosis
skin changes MEES LINES

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33
Q

Lead Neuropathy

A 
Axonal degenration radial nerve
motor somtimes with sensory
anemia basophillic stippling
colickly abdominal pain
EDTA
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34
Q

Polyneuropathy Drug induced

1) Anti neoplastic

A

Cisplatin carboplatin: sensory
paclitaxel: sensory - axonopathy
vincritine
thalidomide

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35
Q

Polyneuropathy Drug induced

2) Antibiotics

A 
Isoniazid symetric sensory, aching burning, (help: PYRIDOSIN)
overdose pyridoxin: ganglionpathy
Chloramhenicol: mild sensory
Metronidazole (plus cerebellum)
Dapsone
Nitrofurantoin
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36
Q

Polyneuropathy Drug induced

3) Cardiac Drugs

A

Amiodarone 5% motor sensory neuropathy
Hydralazine
Niacin

37
Q

Polyneuropathy Drug induced

4) others

A 
PYT
Disulfiram
Statin
Colchicine (more myopathy)
Thalidomde
Amitriptyline
trichlorethylen: Cranial nerves
NO
38
Q

Common causes of mononeuritis multiplex (9)

A 
Polyarteritis nodosa
Isolated vasculitis of peripheral nerve
Wegener
Diabetes
HNPP
Cryoglobulinemia
Sarcoidosis
Lyme Disease
HIV
39
Q

Rare causes for Mononeuritis Multilex (8)

A 
paraneoplastic
Amyloidosis
Leprosy
SLE
Rheumatoid Artrhritis
Leukemia-Lymphoma infiltration
Intravascular lymphoma
Sjoegren Disease
40
Q

Polyarteritis Nodosa PN

A

50% clinical and 75% involvement of peripheral nerves
other: abdo pain, hematuria, fever, eosinophilia, HTN, asthma, Glomerulonephritis and hemorrhgaic lung

Multifocal infarcts
abrupt pain numbness
cranial nerves rare
CSF = normal
necrotizing arteritis on biopsy
p-ANCA
Tx: Steroids and Cyclophosphamide
41
Q

Churgh Strauss Hypereosinophilia PN

A 
excess eosinophils
lung and skin
(induced by zafiruklast)
Churgh Strauss: Rhinitis and ASthma for years
c-ANCA
acute painful neuropathy
42
Q

Wegener

A

1) mononeuritis multiplex vascular
2) lower cranial nerves
c-ANCA

43
Q

c-ANCA

A

Churgh Strauss
Wegener
intravascular Lymphoma
(HIV?, TB?)

44
Q

Cryoglobulinemia

A

purpura, glomerulonephritis, athralgia
Polyneuropathy:
slow progressive,
associated with Hepatitis C

45
Q

Rheumatoid Artritis PN

A

1-5% PN

46
Q

SLE

A

Lupus: 10%
sensorimotor symetric
relapsing remitting
axonal commonly

47
Q

Sarcoidosis

A

subacute chronic polyneuropathy
poliradiculopathy
mononeuropaty

FAcial Nerve

48
Q

Lyme Neuropathy

A

1) CN: bilateral facial
- aseptic meningoradiculitis 100 cells CSF
- radiculitis: BANNWARTH SYNDROME with cauda equina involvement, bladder
2) multiple mononeuropathies (foot wrist drop)
3) lumbar/brachial plexopathy
4) generlaized sensory neuropathy with encephalopathy
5) acute GBS

WESTERN BLOT more specific
Ceftriaxone, Doxycycline

49
Q

Sjoegren Neuropathy

A 
chronic slowly progressive, lymphocytic infiltration of glands, arthritis, 
sensory neuropathy
87%
older women: paresthesia
sensory ataxia
ANTO -RO, SS-A
anti -La or SS-B
50
Q

Wartenberg Syndrome

A

Numbness after reaching, stretching

no reflex loss

51
Q

Celiac Sprue Neuropathy

A

Sprue:
cerebellar ataxia
myoclonus

senory neuropathy
Antigliadin AB

52
Q

Polyneuropathy with Paraproteinemia (6)

A

M-spike

1) M-gus
- – MAG
- – POEMS
2) POEMS
3) MM
4) Waldenstroem
5) Cryoglobulinemia
6) Acquired Amyloid neuropathy

53
Q

Monoclonal Gammopathy of undetermined Significance MGUS

A 
non neoplastic IgM monoclonal
males
60s
slow onset
numbness feet then hand
wasting
areflexia
CSF protein 50-100
demyelinating mixed axo-dem
54
Q

Anti MAG

A 
Proprioception, gait imbalance, tremor, romberg
later weakness, atrophy
Myelin-Associated Glycoprotein (MAG)
Demyelinating
No conduction blocks
PE, Rituximab, Cyclophosphamide
55
Q

GALOP

A 
Gait Disorder
Autoantibody
Late-age
Onser
Polyneuropathy

Sensory more than motor IgM versus cetral myelin
Cyclophosphamide

56
Q

POEMS

A 
Osteosclerotic Myeloma
Polyneuropathy
Organomegaly
Endocrinopathy
M protein
Skin changes

CROW FUKASE SYNDROME
males, 60% 5year survival

osteosclerotic lesions, sensory loss, areflexia, HYPERHIDROSIS Demyelinating later axonal
Endocrnology DM, Hypothyroid, Gynecomastia
Disc swelling in 50%

SERUM: VEGF!!!!
High Protein in CSF
HHV8?

57
Q

MMN Multifocal Motor Neuropathy and Multifocal Conduction Blocks

A 
anti GM1
men
acute onset of wrist or foot drop
painless
no sensory symptoms
!! demyelinating: after weeks atrophy and fasciculations
Areflexia
NO BABINSKY
58
Q

MADSAM

A 
multifocal acquired demyelinating sensory and motor neuropathy
Lewis Sumner
NO GM1
Arms more than Legs
Distal more than Prox
Diaphragm
Conduction blocks
CSF high but under 100
NO IgM anti GM1
59
Q

MMN Multifocal Motor Neuropathy and Multifocal Conduction Blocks

A 
anti GM1
men
acute onset of wrist or foot drop
painless
no sensory symptoms
!! demyelinating: after weeks atrophy and fasciculations
Areflexia
NO BABINSKY
60
Q

MADSAM

A 
multifocal acquired demyelinating sensory and motor neuropathy
Lewis Sumner
NO GM1
Arms more than Legs
Distal more than Prox
Diaphragm
Conduction blocks
CSF high but under 100
NO IgM anti GM1
61
Q

Lipid Metabolism with Neuropathy (7)

A

1) MLD - arylsulfatase - demyelinating
2) Krabbe - beta glaktosidase - dem/axonal
3) ALD - ABCD1 - dem/ax
4) Refsum - dem - Phytanic
5) Fabry - axonal - alha galactosidas
6) Tangier - axonal - ABC1
7) Bassen Kornzweig axonal MTP

62
Q

CMTx

A

connexin 32

63
Q

HMSN III CMT 3

A

Dejerine Sotas
PMP22 P0
infant
severe

64
Q

CMT1B

A

MPZ mutation

dominant, Chr. 1

65
Q

HMSN III CMT 3

A

Dejerine Sotas
PMP22 P0
infant
severe

66
Q

Long thoracic Nerve

A 
C5,6,7
serratus anterior
fixating scapula to chest wall
palsy:
- no raising of arm over head
- winging scapula
66
Q

Long thoracic Nerve

A 
C5,6,7
serratus anterior
fixating scapula to chest wall
palsy:
- no raising of arm over head
- winging scapula
67
Q

Suprascapular Nerve

A 
C5,6
M. supra- and infraspinatus
weak external rotation 
weak abduction first 15 degrees
--- herniated C5C6 disc
67
Q

Suprascapular Nerve

A 
C5,6
M. supra- and infraspinatus
weak external rotation 
weak abduction first 15 degrees
--- herniated C5C6 disc
68
Q

Axillary Nerve

A

C5 (6)
teres minor
deltoid muscle
— no lifting between 15-90 Degrees

68
Q

Axillary Nerve

A

C5 (6)
teres minor
deltoid muscle
— no lifting between 15-90 Degrees

69
Q

Musculocutaneous nerve

A 
C5,6
biceps brachii, brachialis, coracobrachialis
weakness of flexion
sensation: forearm reduced
humerus fracture
69
Q

Musculocutaneous nerve

A 
C5,6
biceps brachii, brachialis, coracobrachialis
weakness of flexion
sensation: forearm reduced
humerus fracture
70
Q

Radial Nerve

A

C6,7,8
triceps, brachioradialis, supinator muscle
PIN posterior interossei nerve: extensor of wrist fingers,
no extension of forearm, no suppination, no wrist finger extension, no thumb extension and abduction in palm plane,
sensory: forearms and hand radial side dorsum

crutch palsy in axilla
pressure palsy
fracture humerus

70
Q

Radial Nerve

A

C6,7,8
triceps, brachioradialis, supinator muscle
PIN posterior interossei nerve: extensor of wrist fingers,
no extension of forearm, no suppination, no wrist finger extension, no thumb extension and abduction in palm plane,
sensory: forearms and hand radial side dorsum

crutch palsy in axilla
pressure palsy
fracture humerus

71
Q

Median Nerve

A

C5, 6, 7, 8 Th1
pronators forearm, long finger flexors, abductor opponens pollicis
sensory palmar

no abductor pollicis breavis and flexor, no opponens

71
Q

Median Nerve

A

C5, 6, 7, 8 Th1
pronators forearm, long finger flexors, abductor opponens pollicis
sensory palmar

no abductor pollicis breavis and flexor, no opponens

72
Q

Ulnar Nerve

A

C8, Th1
hypothenar, abductor adductor fingers,
clawhand

79
Q

Ulnar Nerve

A

C8, Th1
hypothenar, abductor adductor fingers,
clawhand

80
Q

Anterior Interosseus Nerve

A

Only Motoric

flexor pollicis longus
pronator quadratus
the radial half of flexor digitorum profundus (the lateral two out of the four tendons).

As opposed to carpal tunnel syndrome, the AIN has no sensory fibers and therefore no numbness associated with the Anterior Interosseous Syndrome.
Kiloh Nevin Syndrome

If asked to make the “OK” sign, patients will make a triangle sign instead. This ‘Pinch-Test’

81
Q

Meralgia Paraestetica

A

L2-3
thigh to knee sensory
DM Obesity Pregnancy

82
Q

Obturator Nerve

A

L3-4
Adductors
Internal and external rotators
injury by fetal head or forceps during birth

83
Q

Femoral Nerve

A 
L2,3,4
knee extension weakness
#1 DM
pelvic tumor
bleeding into iliopsoas retroperoneal
84
Q

differentiate Femoral from L3,4 root lesion:

A

In femoral nerve, hip adduction by obturator is intact

in L3 lesion no hip adduction

85
Q

Sciatic nerve

A

L4,5, S1,2
disc L4-5:
- L5 root; pain in thigh, numbness inner foot, weakness of dorsiflexion,
disc L5-S1:
- S1, pain, numbness lateral foot, weakness of plantar flexion loss of ankle jerk

86
Q

Common peroneal

A

head of fibular:
- superfiscial peroneal:

    • sensory dorsum of foot except between 1-2 finger
    • lateral lower aspect sensory of leg
  • – EVERSION but NOT INVERSION (L5, tibial)

Deep peroneal:

    • dorsiflexion foot and toe
  • – sensory patch between 1-2
87
Q

Tibial Nerve

A

Calf muscles

  • plantar flexion
  • Inversion
    • sensation plantar of foot
88
Q

MFN2

A

CMT2A
Aut Dom
Optic atrophy

Neurology (7 decks)

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