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BR 3rd Ed > 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 are types of demyelinating sensimotor (uniform) PN?

A 
(Genetic Disorders)
• HMSN-I
• HMSN-III
• HMSN-IV
• Leukodystrophy
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24
Q

What are types of demyelinating motor > sensory (segmental) PN?

A 
(Acquired Disorders)
• AIDP
• CIDP
• Arsenic
• Toxins
• Monoclonal
 gammopathy
• Diphtheria
• AIDS
• Leprosy
• Lyme disease
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25
What are types of demyelinating/axonal loss sensimotor PN?
• Diabetes mellitus • Uremia
26
What are types of axonal loss motor >sensory PN?
* Porphyria * Vincristine * Lead * AIDP * Dapsone * HMSN-II
27
What are types of axonal loss sensory PN?
``` • Cis-platinum • Friedreich’s ataxia • HSN • Sjögren’s syndrome • Pyridoxine • Crohn’s disease ```
28
What are types of axonal loss sensorimotor PN?
``` • Amyloidosis • ETOH • Vitamin B12 • Folate • Toxins • Gold • Mercury • Paraneoplastic syndrome • Sarcoidosis • Lyme disease • HIV related ```
29
What is the DDX for foot drop?
* Diffuse polyneuropathy: Diabetes * Mononeuropathy: Common peroneal (fibular); peroneal (fibular) portion of the sciatic * Plexopathy * Radiculopathy: L4–L5 * Central: Tumor, CVA, AVM, SCI
30
What are causes of Diffuse Axonal Polyneuropathy?
``` Toxins: Heavy metals Drugs: Vincristine, alcohol Deficiency: Vit B6 Metabolic: Uremia, DM Paraneoplastic Hereditary—HMSN II Infectious: Lyme’s, HIV ```
31
What are causes of Multifocal Diffuse Axonal Polyneuropathy?
``` Microangiopathic: Vasculitis, DM Amyloidosis Paraneoplastic Infectious: CMV Metabolic: Porphyria Compression ```
32
What are causes of Diffuse Dymelinating Polyneuropathy?
Hereditary: HMSN-I, IV; Deficiency: Hypothyroidism Toxic: Amiodarone, Arsenic
33
What are causes of Multifocal Dymelinating Polyneuropathy?
Autoimmune: AIDP, CIDP Multiple compressions Leprosy
34
What is the pattern of inheritance for CMT?
Autosomal dominant
35
What is the age of onset for CMT?
Early childhood in first 2 years
36
Describe the motor loss in CMT.
Slow progressive distal motor>sensory ABN Distal>proximal weakness Intrinsic foot and lower anterior leg
37
Describe the sensory loss in CMT.
Lower>upper limbs ABN vibration and proprioception Stocking/glove
38
Describe clinical characteristics of CMT
Pes cavus Hammer toes Champagne bottle leg Hypertrophy of periopheral nerves (greater auricular nerve)
39
What is Roussy-Levy syndrome?
CMT associated with an essential tremor
40
What is seen on nerve biopsy in CMT?
Onion bulb formation from focal demyelination, then remyelination
41
What is seen on NCS in CMT?
``` SNAP: ABN CMAP: ABN, CV decreased 70% No temporal dispersion or conduction block ```
42
What is the etiology of AIDP/GBS?
Possible viral attack on the myelin and Schwann cells
43
What is the onset of AIDP/GBS?
1–4 weeks postillness, vaccination, or surgery
44
What is the 1st sign of AIDP/GBS?
Ascending sensory abnormalities
45
Describe CN involvement in AIDP/GBS.
Most common: CN VII | CN I and II unaffected
46
What are variants of AIDP/GBS?
Miller-Fisher syndrome | Pure sensory
47
What are signs of AIDP/GBS?
Ascending sensory ABN and weakness ABN reflexes Resp/autonomic failure
48
What is the first EDX sign of AIDP/GBS?
ABN F wave
49
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) ```
50
What is the etiology of CIDP?
Possible immune mediated response
51
What is the onset of CIDP?
Any age, peaks at 50 to 60 years of age
52
What is the clinical presentation of CIDP?
``` Relapsing/remitting Sensory ABN Symmetric proximal>distal weakness ABN reflexes Less CN involvement ```
53
What is the etiology of Hansen's disease?
Mycobacterium Leprae
54
What is the MC neuropathy world-wide?
Hansen's disease (Leprosy)
55
What is the clinical presentation of Hansen's disease (Leprosy)?
Sensory ABN Wrist drop Foot drop Facial palsy
56
What is seen on nerve biopsy in Hansen's disease (Leprosy)?
Foamy histiocyte invasion
57
What is the etiology of porphyria?
Defect heme | synthesis
58
What is the clinical presentation of porphyria?
``` Female>male Lower limb pain/weakness Back/ABD pain Seizures Mental status changes Reaction to barbiturates, sulfa ```
59
What are lab features of porphyria?
Deep red urine
60
What is the clinical presentation of lead toxicity?
``` Progressive UE weakness Radial neuropathy ABD discomfort Blue lines in gums Blindness Epilepsy Encephalopathy (child) ```
61
What are lab features of lead toxicity?
Blood/urine: Lead Basophilic stippling in RBCs x-ray lead lines
62
What is the clinical presentation of lead toxicity?
Lower limb paresthesias and weakness ABN MSR
63
What is the clinical presentation of dapsone toxicity?
Ascending foot and hand neuropathy Methemoglobinemia
64
What is axonal AIDP associated with?
CMV and C. jejuni | infection
65
Which form of AIDP has a poor prognosis?
Axonal worse than demyelinating
66
What is the inheritance pattern of HMSN II and CMT II?
Autosomal dominant
67
What is the clinical presentation of HMSN II and CMT II?
``` Onset 2nd decade Weakness ABN MSR Less foot involvement Tremor Ataxia ```
68
What is seen on nerve biopsy in HMSN II and CMT II?
No onion bulb formation
69
What is the clinical presentation of Cis-platinum?
``` Painful parasthesia in hands and feet Nephrotoxicity Ototoxicity Myelosuppression GI complaints ```
70
What is seen on nerve biopsy in Cis-plantinum, Friedreich's ataxia, Sjogren's syndrome and pyirdoxine toxicity?
Abnormal large axons
71
What is the inheritance pattern of Friedreich's ataxia?
Autosomal recessive
72
What is the onset of Friedreich's ataxia?
2 to 16 yo
73
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 ```
74
What is the clinical presentation of Sjogren's syndrome?
``` Dry eyes Dry mouth Keratoconjunctivitis Associated w/ RA ```
75
What is the clinical presentation of Pyridoxine (B6)?
ABN sensation Gait disturbances + Lhermitte’s sign Sx improve w/ stopping drug
76
What dose of Pyridoxine (B6) causes toxicity?
>600 mg/day
77
What is the clinical presentation of ETOH toxicity?
``` Sensory ABN Wrist/foot drop Korsakoff’s psychosis Wernicke’s encephalopathy +/- myopathy ```
78
What is seen in nerve biopsy of ETOH toxicity?
Wallerian degeneration
79
What is the etiology of neuropathy with ETOH toxicity?
Malnutrition or direct | nerve injury
80
What is the etiology of neuropathy with amyloidosis?
Amyloid deposition in | DRG
81
What is the clinical presentation of amyloidosis?
``` Sensory ABN Weight loss Hepatomegaly Purpura Nephrotic syndrome CHF ```
82
What seen on tissue biopsy in amyloidosis?
(+) birefringence with | Congo red staining
83
What is the clinical presentation of sarcoidosis?
``` Low birth weight Fatigue Bilateral hilar adenopathy Uveitis CN involvment ```
84
What are lab findings in Sarcoidosis?
Inc ESR | Nerve biopsy: sarcoid tubercles
85
What are the types of neuropathy associated with DM?
Polyneuropathy Mononeuropathy Autonomic disorders Amyotrophy
86
What is seen on nerve biopsy in DM?
Small and large fiber ABN
87
What is the neuropathy presentation in uremia?
Sensory ABN Hypersensitive to touch Restless leg syndrome
88
What is seen on nerve biopsy in uremia?
Paranodal demyelination Axon loss
89
What is multifocal motor neuropathy (MNN)?
Immune-mediated disorder causing inflammatory demyelination and remyelination
90
What is the clinical presentation of multifocal motor neuropathy (MNN)?
``` Slowly progressing focal weakness Fasciculations and cramps Atrophy and myokymia Asymmetric red MSR Sensation is normal Resembles motor neuron disease (MND) ```
91
What is seen on nerve biopsy in multifocal motor neuropathy (MNN)?
Endoneurial edema Lymphocytic inflammation Red myelin density Onion bulb formation
92
What are lab findings in multifocal motor neuropathy (MNN)?
Inc anti-GM1 antibody titers
93
What is multifocal motor neuropathy (MNN) defined by on EDX?
Multifocal motor conduction block. More than one site of CB can occur in a single motor nerve
94
What is seen on EMG in multifocal motor neuropathy (MNN)?
ABN spontaneous activity, including fasciculations and myokymic discharges
95
What are differences in weakness between MMN and MND?
In MNN activity is confined to the muscles of clinical weakness and MND it is diffusely distributed.
96
What are differences in nerve patterns between MMN and MND?
In MNN activity can be traced back to peripheral nerve territories and MND it can be traced to a spinal segmental pattern.
97
What are the categories of HIV-related neuropathy?
``` -Distal Symmetric Polyneuropathy Inflammatory -Demyelinating Polyneuropathy -Mononeuropathy Multiplex -Progressive Polyradiculopathy -Autonomic Neuropathy ```
98
What is affected in Distal Symmetric Polyneuropathy?
Sensory and automonic fibers | Motor in advanced cases
99
What is the etiology of Mononeuropathy Multiplex?
Thrombosis of the vasa nervorum leads to multiple lesions in various nerves
100
What is affected in Mononeuropathy Multiplex?
Primarily axonal loss with relative myelin sparing
101
What is the etiology of progressive polyradiculopathy in HIV?
Cytomegalovirus
102
What is the clinical presentation of progressive polyradiculopathy in HIV?
Severe asymmetrical | pain, numbness, and motor deficits in the legs. Bowel/bladder dysfunctions

BR 3rd Ed (92 decks)

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