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Joe's Test Class > Ch 5 - EDX: Peripheral Polyneuropathy > Flashcards

Ch 5 - EDX: Peripheral Polyneuropathy Flashcards

1
Q

What can be affected in peripheral polyenuropathies?

A

Myelin and/or axons of peripheral nerves

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2
Q

What is the presentation of a diffuse peripheral polyenuropathy?

A

Essentially involves all nerves in a length-dependent fashion to a relatively equal extent

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3
Q

What is the presentation of a multifocal peripheral polyenuropathy?

A

Involves one or multiple nerves in an asymmetric or patchy distribution

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4
Q

How are peripheral polyneuropathies classified?

A

Acquired or Inherited

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5
Q

What are categories of inherited peripheral polyneuropathies?

A

– Hereditary Motor and Sensory Neuropathies (HMSN)
– Hereditary Sensory and Autonomic Neuropathies (HSAN)
– Hereditary Motor Neuropathies (HMN)

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6
Q

What is the MC inherited peripheral polyneuropathy?

A

HMSN (Charcot-Marie Tooth) disease

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7
Q

What is HMSN (Charcot-Marie Tooth) disease caused by?

A

Duplication mutation of the PMP-22 gene

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8
Q

What are MCC of acquired peripheral polyneuropathies?

A

DM (MC)
AIDP
Medications

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9
Q

What is the classic triad of clinical presentation of general peripheral polyneuropathies?

A

– Sensory changes in a stocking/glove distribution
– Distal weakness
– Diminished/absent MSR

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10
Q

How do inherited peripheral polyneuropathies typically present?

A

– Sensory loss
– Ataxia
– Increased incidence of muscle cramping

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11
Q

How do acquired peripheral polyneuropathies typically present?

A

– Burning
– Pain
– Paresthesias

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12
Q

What will be seen on NCS in demyelinating injury?

A

Prolonged distal latency
Slowed conduction velocity
Conduction block
Increased temporal dispersion

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13
Q

What will be seen on EMG in demyelinating injury?

A

No fibrillation potentials or positive sharp waves
Myokymic discharge
Decreased recruitment

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14
Q

What will be seen on NCS in axonal injury?

A

Reduced SNAP/CMAP amplitude
Near-normal distal latency
Near-normal conduction velocity

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15
Q

What will be seen on EMG in axonal injury?

A 
(+) Fibrillation potentials
(+) Positive sharp waves
Decreased recruitment
Increased duration and amplitude
Polyphasic potentials
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16
Q

What are NCS findings in acquired neuropathy?

A

+ Conduction block
Focal CV slowing
Inc temporal dispersion

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17
Q

What are NCS findings in hereditary neuropathy?

A
  • Conduction block
    Diffuse CV slowing
    Normal temporal dispersion
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18
Q

What are systemic sx of small fiber neuropathy?

A 
Orthostatic BP
Dry scaly skin
Dry eyes
Dry mouth
Burning pain in extremities
ABN pinprick sensation
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19
Q

Norepinephrine is the primary neurotransmitter for ____.

A

Postganglionic sympathetic adrenergic nerves

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20
Q

What is the sympathetic skin response used for?

A

Evaluating the unmyelinated, sympathetic nerve fibers of the PNS

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21
Q

What does loss of sinus arrhythmia with cardiovagal innervation studies mean?

A

Represents denervation process

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22
Q

What are types of demyelinating motor PN?

A

Multifocal motor neuropathy

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23
Q

What is the pattern of inheritance for CMT?

A

Autosomal dominant

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24
Q

What is the age of onset for CMT?

A

Early childhood in first 2 years

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25
Describe the motor loss in CMT.
- Slow progressive distal motor>sensory ABN - Distal>proximal weakness - Intrinsic foot and lower anterior leg
26
Describe the sensory loss in CMT.
Lower>upper limbs ABN vibration and proprioception Stocking/glove
27
Describe clinical characteristics of CMT
Pes cavus Hammer toes Champagne bottle leg Hypertrophy of peripheral nerves (greater auricular nerve)
28
What is Roussy-Levy syndrome?
CMT associated with an essential tremor
29
What is seen on nerve biopsy in CMT?
Onion bulb formation from focal demyelination, then remyelination
30
What is seen on NCS in CMT?
``` SNAP: ABN CMAP: ABN, CV decreased 70% No temporal dispersion or conduction block ```
31
What is the etiology of AIDP/GBS?
Possible viral attack on the myelin and Schwann cells
32
What is the onset of AIDP/GBS?
1–4 weeks post illness, vaccination, or surgery
33
What is the 1st sign of AIDP/GBS?
Ascending sensory abnormalities
34
Describe CN involvement in AIDP/GBS.
Most common: CN VII | CN I and II unaffected
35
What are variants of AIDP/GBS?
Miller-Fisher syndrome | Pure sensory
36
What are signs of AIDP/GBS?
Ascending sensory ABN and weakness ABN reflexes Resp/autonomic failure
37
What is the first EDX sign of AIDP/GBS?
ABN F wave
38
What EDX findings are associated with poor prognosis of AIDP/GBS?
``` CMAP: Amp <20% of normal NCV <40% of normal F wave: Absent EMG: ABN activity (axonal involvement) ```
39
What is the etiology of CIDP?
Possible immune mediated response
40
What is the onset of CIDP?
Any age, peaks at 50 to 60 years of age
41
What is the clinical presentation of CIDP?
``` Relapsing/remitting Sensory ABN Symmetric proximal>distal weakness ABN reflexes Less CN involvement ```
42
What is the etiology of Hansen's disease?
Mycobacterium Leprae
43
What is the MC neuropathy world-wide?
Hansen's disease (Leprosy)
44
What is the clinical presentation of Hansen's disease (Leprosy)?
Sensory ABN Wrist drop Foot drop Facial palsy
45
What is seen on nerve biopsy in Hansen's disease (Leprosy)?
Foamy histiocyte invasion
46
What is the etiology of porphyria?
Defect heme | synthesis
47
What is the clinical presentation of porphyria?
``` Female>male Lower limb pain/weakness Back/ABD pain Seizures Mental status changes Reaction to barbiturates, sulfa ```
48
What are lab features of porphyria?
Deep red urine
49
What is the clinical presentation of lead toxicity?
``` Progressive UE weakness Radial neuropathy ABD discomfort Blue lines in gums Blindness Epilepsy Encephalopathy (child) ```
50
What are lab features of lead toxicity?
Blood/urine: Lead Basophilic stippling in RBCs x-ray lead lines
51
What is the clinical presentation of lead toxicity?
Lower limb paresthesias and weakness ABN MSR
52
What is the clinical presentation of dapsone toxicity?
Ascending foot and hand neuropathy Methemoglobinemia
53
What is axonal AIDP associated with?
CMV and C. jejuni | infection
54
Which form of AIDP has a poor prognosis?
Axonal worse than demyelinating
55
What is the inheritance pattern of HMSN II and CMT II?
Autosomal dominant
56
What is the clinical presentation of HMSN II and CMT II?
``` Onset 2nd decade Weakness ABN MSR Less foot involvement Tremor Ataxia ```
57
What is seen on nerve biopsy in HMSN II and CMT II?
No onion bulb formation
58
What is the clinical presentation of Cis-platinum?
``` Painful parasthesia in hands and feet Nephrotoxicity Ototoxicity Myelosuppression GI complaints ```
59
What is seen on nerve biopsy in Cis-plantinum, Friedreich's ataxia, Sjogren's syndrome and pyirdoxine toxicity?
Abnormal large axons
60
What is the inheritance pattern of Friedreich's ataxia?
Autosomal recessive
61
What is the onset of Friedreich's ataxia?
2 to 16 yo
62
What is the clinical presentation of Friedreich's ataxia?
``` ABN sensation Weakness ABN MSR Limb and trunk ataxia Optic atrophy Dysarthria Cardiomyopathy Kyphoscoliosis Pes cavus ```
63
What is the clinical presentation of Sjogren's syndrome?
Dry eyes Dry mouth Keratoconjunctivitis Associated w/ RA
64
What is the clinical presentation of Pyridoxine (B6)?
ABN sensation Gait disturbances + Lhermitte’s sign Sx improve w/ stopping drug
65
What dose of Pyridoxine (B6) causes toxicity?
>600 mg/day
66
What is the clinical presentation of ETOH toxicity?
``` Sensory ABN Wrist/foot drop Korsakoff’s psychosis Wernicke’s encephalopathy +/- myopathy ```
67
What is seen in nerve biopsy of ETOH toxicity?
Wallerian degeneration
68
What is the etiology of neuropathy with ETOH toxicity?
Malnutrition or direct | nerve injury
69
What is the etiology of neuropathy with amyloidosis?
Amyloid deposition in DRG
70
What is the clinical presentation of amyloidosis?
``` Sensory ABN Weight loss Hepatomegaly Purpura Nephrotic syndrome CHF ```
71
What seen on tissue biopsy in amyloidosis?
(+) birefringence with Congo red staining
72
What is the clinical presentation of sarcoidosis?
``` Low birth weight Fatigue Bilateral hilar adenopathy Uveitis CN involvment ```
73
What are lab findings in Sarcoidosis?
Inc ESR | Nerve biopsy: sarcoid tubercles
74
What are the types of neuropathy associated with DM?
Polyneuropathy Mononeuropathy Autonomic disorders Amyotrophy
75
What is seen on nerve biopsy in DM?
Small and large fiber ABN
76
What is the neuropathy presentation in uremia?
- Sensory ABN - Hypersensitive to touch - Restless leg syndrome
77
What is seen on nerve biopsy in uremia?
Paranodal demyelination Axon loss
78
What is multifocal motor neuropathy (MMN)?
Immune-mediated disorder causing inflammatory demyelination and remyelination
79
What is the clinical presentation of multifocal motor neuropathy (MMN)?
``` Slowly progressing focal weakness Fasciculations and cramps Atrophy and myokymia Asymmetric red MSR Sensation is normal Resembles motor neuron disease (MND) ```
80
What is seen on nerve biopsy in multifocal motor neuropathy (MMN)?
Endoneurial edema Lymphocytic inflammation Red myelin density Onion bulb formation
81
What are lab findings in multifocal motor neuropathy (MMN)?
Inc anti-GM1 antibody titers
82
What is multifocal motor neuropathy (MMN) defined by on EDX?
Multifocal motor conduction block. More than one site of CB can occur in a single motor nerve
83
What is seen on EMG in multifocal motor neuropathy (MMN)?
ABN spontaneous activity, including fasciculations and myokymic discharges
84
What are differences in weakness between MMN and MND?
In MMN activity is confined to the muscles of clinical weakness and MND it is diffusely distributed.
85
What are differences in nerve patterns between MMN and MND?
In MMN activity can be traced back to peripheral nerve territories and MND it can be traced to a spinal segmental pattern.
86
What are the categories of HIV-related neuropathy?
``` -Distal Symmetric Polyneuropathy Inflammatory -Demyelinating Polyneuropathy -Mononeuropathy Multiplex -Progressive Polyradiculopathy -Autonomic Neuropathy ```
87
What is affected in Distal Symmetric Polyneuropathy?
Sensory and automonic fibers | Motor in advanced cases
88
What is the etiology of Mononeuropathy Multiplex?
Thrombosis of the vasa nervorum leads to multiple lesions in various nerves
89
What is affected in Mononeuropathy Multiplex?
Primarily axonal loss with relative myelin sparing
90
What is the etiology of progressive polyradiculopathy in HIV?
Cytomegalovirus
91
What is the clinical presentation of progressive polyradiculopathy in HIV?
Severe asymmetrical | pain, numbness, and motor deficits in the legs. Bowel/bladder dysfunctions

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