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Neurology > Peripheral NS Disorders > Flashcards

Peripheral NS Disorders Flashcards

1
Q

Wallerian degeneration

A

disintegration of myelin or axons distal to site of injury

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2
Q

neuronal/axonal degeneration

A

distal dying of axons and loss of myelin after damage to cell body of neurons

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3
Q

demyelination

A

loss of myelin sheath

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4
Q

symptoms assoc. with small nerve fibers

A

neuropathic pain
disturbance of temp. sensation
autonomic dysfunction

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5
Q

symptoms assoc with large nerve fibers

A

loss of vibration/joint sense
weakness
fasciculations
loss of DTRs

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6
Q

symmetric proximal and distal weakness with sensory loss

A

GBS, CIDP

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7
Q

symmetric distal weakness with sensory loss

A

drug-induced, toxic and metabolic, hereditary, amyloidosis

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8
Q

asymmetric distal weakness with sensory loss; multiple nerves

A

vasculitis, HNPP, infections (leprosy, sarcoid, Lyme, HIV)

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9
Q

single nerve signs

A

compressive mononeuropathy or radiculopathy

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10
Q

asymmetric distal weakness WITHOUT sensory loss

A

motor neuron disease

multifocal motor neuropathy

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11
Q

asymmetric proximal and distal weakness with sensory loss

A 
polyradiculopathy or plexopathy
meningeal carcinomatosis
lymphomatosis
HNPP
hereditary neuropathy
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12
Q

symmetric sensory loss w/o weakness

A

cryptogenic sensory neuropathy
metabolic, drug-induced, toxic
leprosy

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13
Q

asymmetric proprioceptive sensory loss w/o weakness

A

sensory neuronopathies

consider paraneoplastic, Sjogrens, vitamin B6 tox, HIV, cisplatinum toxicity

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14
Q

autonomic symptoms

A

DM, amyloidosis, GBS, vincristine, porphyria, HIV, idiopathic pandysautonomia

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15
Q

first line tests for PNS disorders

A 
CBC
ESR/CRP
glucose, HbA1C or 2 hr OGTT
rheumatoid - ANA, anti-ENA, RF
renal and LFTs
vit B12 and MMA levels
TFTs
serum and urine protein electrophoresis
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16
Q

RF for peripheral neuropathy

A

diabetes mellitus
alcoholism
non-alcoholic liver dz
malignancy

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17
Q

VITAMINS mnemonic

A 
Vitamin deficiency/vasculitis
Infections - leprosy, TB
Toxic - amiodarone, lead, vincristine, chemotherapy
A - amyloid
Metabolic - alcohol, DM, porphyria, hypothyroidism, liver/renal
Idiopathic/Inherited
Neoplasms
Systemic - SLE, Sjogrens, RA, MM
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18
Q

polyneuropathy

A

involvement of multiple peripheral nerves, usually legs&raquo_space; arms and distal&raquo_space; proximal

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19
Q

mononeuropathy

A

involvement of single nerve

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20
Q

mononeuropathy multiplex

A

involvement of several individual nerves in a multifocal distribution

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21
Q

MC infections assoc with GBS

A

campylobacter jejuni
CMV
EBV

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22
Q

target of autoimmune response in GBS

A

gangliosides

- anti GM1, anti-GD1a, anti-GQ1b, anti-GD1b

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23
Q

typical presentation of GBS

A

rapidly evolving, ascending areflexic motor paralysis w/ or w/o sensory disturbances; initial symptoms consist of tingling/pins and needles sensations in feet

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24
Q

symptoms of GBS

A

neuropathic pain
areflexic paralysis
bulbar and resp. mm weakness
autonomic dysfunction

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25
Q

Miller-Fisher variant of GBS

A

gait ataxia, areflexia, external ophthalmoplegia usually w/o limb ; nerve conduction studies are normal, anti-GQ1b ab’s

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26
Q

electrodiagnostic findings in GBS

A

prolonged distal latencies
prolonged or absent F waves
conduction block
decreased motor unit recruitment

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27
Q

how do you measure respiratory function in GBS pt

A

measure FVC and negative inspiratory pressure

- if FVC < 15 ml/kg, ICU and intubate

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28
Q

Tx of GBS

A

plasmaphoresis or IVIG

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29
Q

CF of CIDP

A

slowly evolving weakness in legs with widespread areflexia and loss of vibratory sense (large fiber); weakness of neck flexors and painful paresthesias may be present

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30
Q

about 10% of pts with CIDP have associated illness…

A

HIV
monoclonal gammopathy
Hodgkins lymphoma

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31
Q

tx of CIDP

A

periodic IVIG therapy
steroids - 90% improve but 50% relapse
azathioprine

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32
Q

pure motor multiple mononeuropathy with predominance in males that can occur at any age

A

multifocal motor neuropathy

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33
Q

CF in multifocal motor neuropathy

A

slowly progressive, asymmetric, distal limb weakness that begins in the arms (in distribution of individual nerves)

34
Q

dx of multifocal motor neuropathy

A

conduction block in motor nerves in areas not prone to compression
- high IgM anti-GM1

35
Q

Tx of multifocal motor neuropathy

A

IVIG
rituximab
immunosuppressants - cyclophosphamide

36
Q

symmetric sensorimotor neuropathy that affects legs&raquo_space; arms, causing prominent large-fiber sensory loss and sensory ataxia as well as weakness

A

neuropathy due to MM or MGUS

37
Q

Dx of neuropathy due to MM or MGUS

A

significant demyelination and axonal loss

38
Q

anti-MAG neuropathy responds to

A

rituximab

39
Q

chronic progressive distal symmetric diabetic polyneuropathy

A

mixed sensory-autonomic-motor polyneuropathy (can be small fiber, large fibers and autonomic)

40
Q

diabetic proximal motor neuropathy (Diabetic amyotrophy)

A

severe thigh and back pain, followed w/in weeks by mild to severe hip/thigh mm weakness with mm atrophy

41
Q

who is more susceptible to compression neuropathies?

A

diabetics

42
Q

CF in diabetic polyneuropathy

A

neuropathic pain/dysesthesias
distal, symmetric, slowly progressive sensory loss in legs before arms
autonomic insufficiency
weakness

43
Q

symptomatic tx of neuropathic pain

A

TCAs
duloxetine
anticonvulsants - gabapentin, pregabalin, lamotrigine

44
Q

uremic neuropathy

A

symmetric, distally predominant sensorimotor axonal polyneuropathy; foot drop and leg weakness are major manifestations

45
Q

what other neuropathy can mimic GBS with rapidly evolving weakness or paralysis, and areflexia and abdominal pain

A

porphyric neuropathy

46
Q

critical illness neuropathy

A

in 50% of pts with severe medical illness in ICU > 2 weeks; first manifestation could be difficulty weaning from ventilator

47
Q

CF in Charcot-Marie tooth disease

A

presents in adolescence with symmetric, slowly progressive, distal muscular atrophy of legs and feet; pts also have hammer toes and pes cavus

48
Q

CMT-2

A

axonal motor neuropathy

49
Q

MC infectious cause of neuropathy world-wide

A

leprosy

50
Q

CF of leprosy neuropathy

A

mononeuropathy multiplex with predilection for cooler areas (nose, ears, distal limbs); DTRs are usually preserved with nerve hypertrophy which is palpable

51
Q

pandysautonomia

A

acquired disorder, usually immune in nature that follows a viral infection, in which both PNS and SNS are affected

52
Q

CF of charcot-marie tooth disease

A
  • AD
  • atrophy of calves (champagne bottle)
  • peripheral nerves palpably enlarged
  • large fiber sensory loss
  • absent ankle jerk reflexes
53
Q

Dejerine-Sottas disease (HSMN III)

A

rare, pediatric dz with AR inheritance

  • severe weakness/numbness
  • markedly enlarged peripheral nerve with onion bulb formation
  • slowed conduction velocities
54
Q

triad of peripheral neuropathy, retinitis pigmentosa and dry/scaly skin

A

Refsum’s dz - HSNM IV

- AR disorder with accumulation of phytanic acid

55
Q

Tx of Refsum’s dz

A

dietary restriction of phytanic acid and plasmaphoresis

56
Q

what cancer causes purely sensory neuropathy

A

small cell ca of lung

57
Q

four systemic infections w/ high incidence of neuropathy

A

leprosy
syphillis
HIV
diphtheria

58
Q

diabetic amyotrophy

A

ischemic infarction of lumbosacral plexus

59
Q

cause of mononeuropathy mutliplex

A

ischemic infarction of vasa nervorum due to vasculitis (ie. SLE, RA, PAN, DM)

60
Q

patho of distal axonopathy

A

metabolic abnormality causes failure of protein synthesis and axonal transport, resulting in degeneration of distal regions of axons

61
Q

what axons are most susceptible to metabolic injury?

A

small diameter axons - initial sx. incl autonomic dysfunction and small fiber sensory modalities (pain/temp)

62
Q

2 MC forms of demyelinating neuropathy

A

GBS

CIDP

63
Q

general characteristics of demyelinating neuropathy

A

affect large diameter myelinated axons

  • significant motor weakness
  • large fiber sensory loss (incl loss of vibration and proprioception)
64
Q

diphtheric neuropathy

A

toxin inhibits Schwann cell production of myelin, prod. severe weakness and large fiber sensory loss

65
Q

somatic motor neuronopathies

A

isolated involvement of ant. horn cells

- ALS and spinal muscular atrophy

66
Q

somatic sensory neuronopathies

A

disruption of metabolism of sensory nerve cell bodies, followed by degeneration of their processes
- ex. paraneoplastic, Sjogren’s

67
Q

autonomic neuronopathy

A

post-ganglionic autonomic neurons –> causes idiopathic orthostatic hypotension

68
Q

NCS results for demyelinating neuropathies

A
  • slowed conduction velocity
  • prolonged distal latency
  • dispersion of evoked compound APs
  • conduction block
69
Q

NCS findings in axonal neuropathies

A

reduced amplitude of compound motor or sensory APs

70
Q

EMG findings in acute denervation

A

fibrillations

positive waves

71
Q

EMG findings in chronic denervation

A

large amplitude, long duration and polyphasic voluntary motor unit potentials
- decreased recruitment of motor units

72
Q

EMG findings in demyelination

A

decreased recruitment pattern

- voluntary motor unit potentials are normal

73
Q

general indication for nerve biopsy

A

asymmetric, multi-focal neuropathies

- can dx. vasculitis, amyloidosis, leprosy and sarcoidosis

74
Q

what nerves are taken for nerve biopsy

A

sural nerve

superficial radial N (UE)

75
Q

what neuropathies are steroids recommended?

A

vasculitic neuropathies

CIDP

76
Q

role of azathioprine in neuropathies

A

used in combo with steroids to tx. autoimmune neuropathies; allows steroids to be tapered more quickly

77
Q

3 MOA of IVIG

A
  • anti-idiotypic abs may neutralize auto-abs
  • reduced complement med. damage
  • down regulate immune production
78
Q

in which neuropathies is IVIG beneficial

A

Guillan Barre
CIDP
polyneuropathy due to paraproteins

79
Q

s/e of IVIG

A

fever, myalgia, headache, rash, aseptic meningitis and renal failure

80
Q

what neuropathies are TCAs useful for

A

peripheral neuropathies with burning dysesthetic pains; these drugs are sedating, therefore, if taken at bedtime promote sleep

81
Q

what anticonvulsants are commonly used for neuropathic pain?

A

carbamazepine, phenytoin, gabapentin and lamotrigine

- stabilize neuronal membranes

82
Q

what type of neuropathic pain benefits from anti-convulsants

A

lancinating pain i.e. trigeminal neuralgia

Neurology (15 decks)

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