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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
Miller-Fisher variant of GBS
gait ataxia, areflexia, external ophthalmoplegia usually w/o limb ; nerve conduction studies are normal, anti-GQ1b ab's
26
electrodiagnostic findings in GBS
prolonged distal latencies prolonged or absent F waves conduction block decreased motor unit recruitment
27
how do you measure respiratory function in GBS pt
measure FVC and negative inspiratory pressure | - if FVC < 15 ml/kg, ICU and intubate
28
Tx of GBS
plasmaphoresis or IVIG
29
CF of CIDP
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
30
about 10% of pts with CIDP have associated illness...
HIV monoclonal gammopathy Hodgkins lymphoma
31
tx of CIDP
periodic IVIG therapy steroids - 90% improve but 50% relapse azathioprine
32
pure motor multiple mononeuropathy with predominance in males that can occur at any age
multifocal motor neuropathy
33
CF in multifocal motor neuropathy
slowly progressive, asymmetric, distal limb weakness that begins in the arms (in distribution of individual nerves)
34
dx of multifocal motor neuropathy
conduction block in motor nerves in areas not prone to compression - high IgM anti-GM1
35
Tx of multifocal motor neuropathy
IVIG rituximab immunosuppressants - cyclophosphamide
36
symmetric sensorimotor neuropathy that affects legs >> arms, causing prominent large-fiber sensory loss and sensory ataxia as well as weakness
neuropathy due to MM or MGUS
37
Dx of neuropathy due to MM or MGUS
significant demyelination and axonal loss
38
anti-MAG neuropathy responds to
rituximab
39
chronic progressive distal symmetric diabetic polyneuropathy
mixed sensory-autonomic-motor polyneuropathy (can be small fiber, large fibers and autonomic)
40
diabetic proximal motor neuropathy (Diabetic amyotrophy)
severe thigh and back pain, followed w/in weeks by mild to severe hip/thigh mm weakness with mm atrophy
41
who is more susceptible to compression neuropathies?
diabetics
42
CF in diabetic polyneuropathy
neuropathic pain/dysesthesias distal, symmetric, slowly progressive sensory loss in legs before arms autonomic insufficiency weakness
43
symptomatic tx of neuropathic pain
TCAs duloxetine anticonvulsants - gabapentin, pregabalin, lamotrigine
44
uremic neuropathy
symmetric, distally predominant sensorimotor axonal polyneuropathy; foot drop and leg weakness are major manifestations
45
what other neuropathy can mimic GBS with rapidly evolving weakness or paralysis, and areflexia and abdominal pain
porphyric neuropathy
46
critical illness neuropathy
in 50% of pts with severe medical illness in ICU > 2 weeks; first manifestation could be difficulty weaning from ventilator
47
CF in Charcot-Marie tooth disease
presents in adolescence with symmetric, slowly progressive, distal muscular atrophy of legs and feet; pts also have hammer toes and pes cavus
48
CMT-2
axonal motor neuropathy
49
MC infectious cause of neuropathy world-wide
leprosy
50
CF of leprosy neuropathy
mononeuropathy multiplex with predilection for cooler areas (nose, ears, distal limbs); DTRs are usually preserved with nerve hypertrophy which is palpable
51
pandysautonomia
acquired disorder, usually immune in nature that follows a viral infection, in which both PNS and SNS are affected
52
CF of charcot-marie tooth disease
- AD - atrophy of calves (champagne bottle) - peripheral nerves palpably enlarged - large fiber sensory loss - absent ankle jerk reflexes
53
Dejerine-Sottas disease (HSMN III)
rare, pediatric dz with AR inheritance - severe weakness/numbness - markedly enlarged peripheral nerve with onion bulb formation - slowed conduction velocities
54
triad of peripheral neuropathy, retinitis pigmentosa and dry/scaly skin
Refsum's dz - HSNM IV | - AR disorder with accumulation of phytanic acid
55
Tx of Refsum's dz
dietary restriction of phytanic acid and plasmaphoresis
56
what cancer causes purely sensory neuropathy
small cell ca of lung
57
four systemic infections w/ high incidence of neuropathy
leprosy syphillis HIV diphtheria
58
diabetic amyotrophy
ischemic infarction of lumbosacral plexus
59
cause of mononeuropathy mutliplex
ischemic infarction of vasa nervorum due to vasculitis (ie. SLE, RA, PAN, DM)
60
patho of distal axonopathy
metabolic abnormality causes failure of protein synthesis and axonal transport, resulting in degeneration of distal regions of axons
61
what axons are most susceptible to metabolic injury?
small diameter axons - initial sx. incl autonomic dysfunction and small fiber sensory modalities (pain/temp)
62
2 MC forms of demyelinating neuropathy
GBS | CIDP
63
general characteristics of demyelinating neuropathy
affect large diameter myelinated axons - significant motor weakness - large fiber sensory loss (incl loss of vibration and proprioception)
64
diphtheric neuropathy
toxin inhibits Schwann cell production of myelin, prod. severe weakness and large fiber sensory loss
65
somatic motor neuronopathies
isolated involvement of ant. horn cells | - ALS and spinal muscular atrophy
66
somatic sensory neuronopathies
disruption of metabolism of sensory nerve cell bodies, followed by degeneration of their processes - ex. paraneoplastic, Sjogren's
67
autonomic neuronopathy
post-ganglionic autonomic neurons --> causes idiopathic orthostatic hypotension
68
NCS results for demyelinating neuropathies
- slowed conduction velocity - prolonged distal latency - dispersion of evoked compound APs - conduction block
69
NCS findings in axonal neuropathies
reduced amplitude of compound motor or sensory APs
70
EMG findings in acute denervation
fibrillations | positive waves
71
EMG findings in chronic denervation
large amplitude, long duration and polyphasic voluntary motor unit potentials - decreased recruitment of motor units
72
EMG findings in demyelination
decreased recruitment pattern | - voluntary motor unit potentials are normal
73
general indication for nerve biopsy
asymmetric, multi-focal neuropathies | - can dx. vasculitis, amyloidosis, leprosy and sarcoidosis
74
what nerves are taken for nerve biopsy
sural nerve | superficial radial N (UE)
75
what neuropathies are steroids recommended?
vasculitic neuropathies | CIDP
76
role of azathioprine in neuropathies
used in combo with steroids to tx. autoimmune neuropathies; allows steroids to be tapered more quickly
77
3 MOA of IVIG
- anti-idiotypic abs may neutralize auto-abs - reduced complement med. damage - down regulate immune production
78
in which neuropathies is IVIG beneficial
Guillan Barre CIDP polyneuropathy due to paraproteins
79
s/e of IVIG
fever, myalgia, headache, rash, aseptic meningitis and renal failure
80
what neuropathies are TCAs useful for
peripheral neuropathies with burning dysesthetic pains; these drugs are sedating, therefore, if taken at bedtime promote sleep
81
what anticonvulsants are commonly used for neuropathic pain?
carbamazepine, phenytoin, gabapentin and lamotrigine | - stabilize neuronal membranes
82
what type of neuropathic pain benefits from anti-convulsants
lancinating pain i.e. trigeminal neuralgia

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