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Cuccurullo Book > 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
Q

Describe the motor loss in CMT.

A
  • Slow progressive distal motor>sensory ABN
  • Distal>proximal weakness
  • Intrinsic foot and lower anterior leg
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26
Q

Describe the sensory loss in CMT.

A

Lower>upper limbs
ABN vibration and proprioception
Stocking/glove

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27
Q

Describe clinical characteristics of CMT

A

Pes cavus
Hammer toes
Champagne bottle leg
Hypertrophy of peripheral nerves (greater auricular nerve)

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28
Q

What is Roussy-Levy syndrome?

A

CMT associated with an essential tremor

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29
Q

What is seen on nerve biopsy in CMT?

A

Onion bulb formation from focal demyelination, then remyelination

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30
Q

What is seen on NCS in CMT?

A 
SNAP: ABN
CMAP: ABN,
CV decreased 70% No
temporal dispersion or
conduction block
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31
Q

What is the etiology of AIDP/GBS?

A

Possible viral attack on the myelin and Schwann cells

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32
Q

What is the onset of AIDP/GBS?

A

1–4 weeks post illness, vaccination, or surgery

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33
Q

What is the 1st sign of AIDP/GBS?

A

Ascending sensory abnormalities

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34
Q

Describe CN involvement in AIDP/GBS.

A

Most common: CN VII

CN I and II unaffected

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35
Q

What are variants of AIDP/GBS?

A

Miller-Fisher syndrome

Pure sensory

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36
Q

What are signs of AIDP/GBS?

A

Ascending sensory ABN and weakness
ABN reflexes
Resp/autonomic failure

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37
Q

What is the first EDX sign of AIDP/GBS?

A

ABN F wave

38
Q

What EDX findings are associated with poor prognosis of AIDP/GBS?

A 
CMAP: Amp <20% of normal
NCV <40% of
normal
F wave: Absent
EMG: ABN activity (axonal involvement)
39
Q

What is the etiology of CIDP?

A

Possible immune mediated response

40
Q

What is the onset of CIDP?

A

Any age, peaks at 50 to 60 years of age

41
Q

What is the clinical presentation of CIDP?

A 
Relapsing/remitting
Sensory ABN
Symmetric proximal>distal weakness
ABN reflexes
Less CN involvement
42
Q

What is the etiology of Hansen’s disease?

A

Mycobacterium Leprae

43
Q

What is the MC neuropathy world-wide?

A

Hansen’s disease (Leprosy)

44
Q

What is the clinical presentation of Hansen’s disease (Leprosy)?

A

Sensory ABN
Wrist drop
Foot drop
Facial palsy

45
Q

What is seen on nerve biopsy in Hansen’s disease (Leprosy)?

A

Foamy histiocyte invasion

46
Q

What is the etiology of porphyria?

A

Defect heme

synthesis

47
Q

What is the clinical presentation of porphyria?

A 
Female>male
Lower limb pain/weakness
Back/ABD pain
Seizures
Mental status changes
Reaction to barbiturates, sulfa
48
Q

What are lab features of porphyria?

A

Deep red urine

49
Q

What is the clinical presentation of lead toxicity?

A 
Progressive UE weakness
Radial neuropathy
ABD discomfort
Blue lines in gums
Blindness
Epilepsy
Encephalopathy (child)
50
Q

What are lab features of lead toxicity?

A

Blood/urine: Lead
Basophilic stippling
in RBCs
x-ray lead lines

51
Q

What is the clinical presentation of lead toxicity?

A

Lower limb
paresthesias and
weakness
ABN MSR

52
Q

What is the clinical presentation of dapsone toxicity?

A

Ascending foot and
hand neuropathy
Methemoglobinemia

53
Q

What is axonal AIDP associated with?

A

CMV and C. jejuni

infection

54
Q

Which form of AIDP has a poor prognosis?

A

Axonal worse than demyelinating

55
Q

What is the inheritance pattern of HMSN II and CMT II?

A

Autosomal dominant

56
Q

What is the clinical presentation of HMSN II and CMT II?

A 
Onset 2nd decade
Weakness
ABN MSR
Less foot involvement
Tremor
Ataxia
57
Q

What is seen on nerve biopsy in HMSN II and CMT II?

A

No onion bulb formation

58
Q

What is the clinical presentation of Cis-platinum?

A 
Painful parasthesia in hands and feet
Nephrotoxicity
Ototoxicity
Myelosuppression
GI complaints
59
Q

What is seen on nerve biopsy in Cis-plantinum, Friedreich’s ataxia, Sjogren’s syndrome and pyirdoxine toxicity?

A

Abnormal large axons

60
Q

What is the inheritance pattern of Friedreich’s ataxia?

A

Autosomal recessive

61
Q

What is the onset of Friedreich’s ataxia?

A

2 to 16 yo

62
Q

What is the clinical presentation of Friedreich’s ataxia?

A 
ABN sensation
Weakness
ABN MSR
Limb and trunk ataxia
Optic atrophy
Dysarthria
Cardiomyopathy
Kyphoscoliosis
Pes cavus
63
Q

What is the clinical presentation of Sjogren’s syndrome?

A

Dry eyes
Dry mouth
Keratoconjunctivitis
Associated w/ RA

64
Q

What is the clinical presentation of Pyridoxine (B6)?

A

ABN sensation
Gait disturbances
+ Lhermitte’s sign
Sx improve w/ stopping drug

65
Q

What dose of Pyridoxine (B6) causes toxicity?

A

> 600 mg/day

66
Q

What is the clinical presentation of ETOH toxicity?

A 
Sensory ABN
Wrist/foot drop
Korsakoff’s psychosis
Wernicke’s
encephalopathy
\+/- myopathy
67
Q

What is seen in nerve biopsy of ETOH toxicity?

A

Wallerian degeneration

68
Q

What is the etiology of neuropathy with ETOH toxicity?

A

Malnutrition or direct

nerve injury

69
Q

What is the etiology of neuropathy with amyloidosis?

A

Amyloid deposition in DRG

70
Q

What is the clinical presentation of amyloidosis?

A 
Sensory ABN
Weight loss
Hepatomegaly
Purpura
Nephrotic syndrome
CHF
71
Q

What seen on tissue biopsy in amyloidosis?

A

(+) birefringence with Congo red staining

72
Q

What is the clinical presentation of sarcoidosis?

A 
Low birth weight
Fatigue
Bilateral hilar adenopathy
Uveitis
CN involvment
73
Q

What are lab findings in Sarcoidosis?

A

Inc ESR

Nerve biopsy: sarcoid tubercles

74
Q

What are the types of neuropathy associated with DM?

A

Polyneuropathy
Mononeuropathy
Autonomic disorders
Amyotrophy

75
Q

What is seen on nerve biopsy in DM?

A

Small and large fiber ABN

76
Q

What is the neuropathy presentation in uremia?

A
  • Sensory ABN
  • Hypersensitive to touch
  • Restless leg syndrome
77
Q

What is seen on nerve biopsy in uremia?

A

Paranodal
demyelination
Axon loss

78
Q

What is multifocal motor neuropathy (MMN)?

A

Immune-mediated disorder causing inflammatory demyelination and remyelination

79
Q

What is the clinical presentation of multifocal motor neuropathy (MMN)?

A 
Slowly progressing focal weakness
Fasciculations and cramps
Atrophy and myokymia
Asymmetric red MSR
Sensation is normal
Resembles motor neuron disease (MND)
80
Q

What is seen on nerve biopsy in multifocal motor neuropathy (MMN)?

A

Endoneurial edema Lymphocytic inflammation
Red myelin density
Onion bulb formation

81
Q

What are lab findings in multifocal motor neuropathy (MMN)?

A

Inc anti-GM1 antibody titers

82
Q

What is multifocal motor neuropathy (MMN) defined by on EDX?

A

Multifocal motor conduction block. More than one site of CB can occur in a single motor nerve

83
Q

What is seen on EMG in multifocal motor neuropathy (MMN)?

A

ABN spontaneous activity, including fasciculations and myokymic discharges

84
Q

What are differences in weakness between MMN and MND?

A

In MMN activity is confined to the muscles of clinical weakness and MND it is diffusely distributed.

85
Q

What are differences in nerve patterns between MMN and MND?

A

In MMN activity can be traced back to peripheral nerve territories and MND it can be traced to a spinal segmental pattern.

86
Q

What are the categories of HIV-related neuropathy?

A 
-Distal Symmetric Polyneuropathy
Inflammatory
-Demyelinating Polyneuropathy
-Mononeuropathy Multiplex
-Progressive Polyradiculopathy
-Autonomic Neuropathy
87
Q

What is affected in Distal Symmetric Polyneuropathy?

A

Sensory and automonic fibers

Motor in advanced cases

88
Q

What is the etiology of Mononeuropathy Multiplex?

A

Thrombosis of the vasa nervorum leads to multiple lesions in various nerves

89
Q

What is affected in Mononeuropathy Multiplex?

A

Primarily axonal loss with relative myelin sparing

90
Q

What is the etiology of progressive polyradiculopathy in HIV?

A

Cytomegalovirus

91
Q

What is the clinical presentation of progressive polyradiculopathy in HIV?

A

Severe asymmetrical

pain, numbness, and motor deficits in the legs. Bowel/bladder dysfunctions

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