During polyneuropathy protocol: 1. Test ____ site 2. Start with _____ study 3. If abnormal --> ____ 4. If no response --> ____

1. most involved site when mild-to-moderate. Least involved if severe. 2. a. Peroneal motor (EDB) - stim at ankle and knee. record F response latency. If no response or abnormal response move to 3 or 4. b. Sural sensory c. Median sensory or ulnar (since high incidence of median pathology) 3. tibial motor (abd hall) stim at ankle and knee; record F response latency 4. peroneal motor (anterior tibial) stim at fibula and knee. ulnar motor (hypothenar) stim at elbow and wrist measure F response latency Median motor (thenar) stim at elbow and wrist measure F response latency If prominent CN involvement, stim at facial motor and blink reflex. ``` Needle exam - anterior tib - medial gastroc - first DI - lumbar paraspinals (if normal, intrinsic foot muscles should be tested. ```

Polyneuropathy Flashcards by Jamie Key

what are the three types of polyneuropathy?

Axonopathy
Myelinopathy
Neuronopathy

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What is the temporal profile that you can learn from EMG (3)

Acute 21 days 5 months
Subacute 6-11 months
Chronic 12-18 months

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What are the 4 predominant pathologies in peripheral neuropathy?

A. Axonal
B. Uniform demyelinating (usually hereditary)
C. Segmental demyelinating (usually acquired)
D. Axonal & Demyelinating (classic example is diabetes)

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What three things about polyneuropathy must you know to correctly diagnose?

generalized process
symmetric vs asymmetric
multifocal (spotty distribution)

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During polyneuropathy protocol:

Test ____ site
Start with _____ study
If abnormal –> ____
If no response –> ____

most involved site when mild-to-moderate. Least involved if severe.
a. Peroneal motor (EDB) - stim at ankle and knee. record F response latency. If no response or abnormal response move to 3 or 4.
b. Sural sensory
c. Median sensory or ulnar (since high incidence of median pathology)
tibial motor (abd hall) stim at ankle and knee; record F response latency
peroneal motor (anterior tibial) stim at fibula and knee.
ulnar motor (hypothenar) stim at elbow and wrist
measure F response latency
Median motor (thenar) stim at elbow and wrist
measure F response latency

If prominent CN involvement, stim at facial motor and blink reflex.

Needle exam
- anterior tib 
- medial gastroc
- first DI
- lumbar paraspinals
(if normal, intrinsic foot muscles should be tested.

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Nickerson protocol for polyneuropathy:

Lower ext sensory:
Upper ext sensory:
Lower ext motor:
Upper ext motor:

plus?

EMG:

Usually do longest sensory

Sural bilat - if can’t get, do superficial peroneal (peroneal commonly used as can get prox and distal stim)

ulnar - bc median pathology is common

peroneal and tibial

ulnar - F wave

do long loop (H waves and F reflexes)

EMG: sample lower extremity first. If abnormal do contralateral muscle

radic screen: atleast 5.
Tib ant, med gastroc, FDI
Upper, FDI and biceps (something distal and something proximal)

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In axonopathy, will have ____ phenomenon

_____ nerves and ____ fibers are affected first

___ is spared

___ is preserved if corrected

Overall, will see ____ and ___ on NCS

Prognosis:

dying back (not exclusive)

longest/largest

myelin sheath

endoneurium

decreased amplitude and area under the curve

slow re-growth and return of function over months to years.
incomplete recovery.

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Myelinopathy spares ____ but affects ______.

subsequent ____ loss occurs

______focal

distal or proximal?

Prognosis:

Recovery:

schwan cell, myeline sheath.

axonal loss (slight amount of axonal injury)

multifocal/segmental demyelination - doesn’t strip entire nerve.
- affects node of ranvier (multisites)

proximal before distal
uniform or segmental

prog: weeks to months

Functional recovery better than axonopathy
incomplete recovery

In acquired, will have excessive temperal dispersion (spread out, extra bumps), not in hereditary.

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Name the two most common uniform demyelinating mixed sensorimotor peripheral neruopathies

HMSN I, II, IV

2. Krabbe Leukodystrophy

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Name the three most common segmental demyelinating motor>sensory peripheral neuropathies

Other 6?

AIDP
CIDP
MGUS
Arsenic
Toxins
Diptheria
AIDS
Leprosy
Lyme disease

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Name the 4 most common axon loss motor > sensory peripheral neuropathies

other 3?

Porphyria
Vincristing
Axonal GBS
HMSN II, V
Dapsone
AIDP
Lead

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Name the 3 most common axonal loss sensory neuronopathy

Other 3

HSN I-V
Friedreich’s ataxia
cisplatinum
Sjogren’s syndrome
Pyridoxine
Crohn’s disease

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Name the three most common axon loss mixed sensorimotor peripheral neuropathies

other 9?

Critical illness polyneuropathy
ETOH
Toxic
Amyloidosis
Vit B12
Folate
Gold
Mercury
Paraneoplastic syndrome
Sarcoidosis
Lyme disease
HIV related

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Name the 2 most common mixed axonal and demyelinating sensorimotor peripheral neuropathies

uremia

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_____ is a condition that affects the cell body (DRG or anterior horn cell)

Neuronopathy

sensory neuronopathies fit in this category as well.

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If a myelin problem, will get what 3 things on NCS?

onset or peak latency delay
CV decreased
temporal dispersion

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Is this physiologic or pathologic temporal dispersion?

stimulate proximally you get amplitude x, stimulate distally and get slightly lower amplitude; this will happen with all nerves (this happens because longer length, some axons get there at the same time, there is phase cancellation, and the amplitude is decreased) The area under the curve will remain constant

Physiologic

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Is this physiologic or pathologic temporal dispersion?

stimulate proximally you get amplitude x, stimulate distally and you get an amplitude more than 20% less than x (less than 15% change in negative duration of wave). Overall curve is shortened (amplitude) and more spread out (duration). The area under the curve stays similar.

pathologic

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____ will be decrease in amplitude without increase in duration of the wave. Can be confused with_____

partial conduction block

pathologic temporal dispersion.

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What will be seen on axonal process for EMG

increased insertional activity
abnormal spontaneous activity - fibs/PSW
- reinnervation potentials (polyphasics, serrations, increased amplitudes, CRD if chronic)
- wont see abnormal spontaneous activity in demyelinating

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wont see _____ on EMG in demyelinating process

abnormal spontaneous activity

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Distal latency: 
Early demyelinating:
Chronic demyelinating: 
Acute axonal:
Chronic axonal:

increased
increased
normal or slightly increased
normal or slightly increased

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Nerve conduction velocity
Early demyelinating:
Chronic demyelinating: 
Acute axonal:
Chronic axonal:

decreased
decreased
normal or slightly decreased
normal or slightly decreased

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F-latency
Early demyelinating:
Chronic demyelinating: 
Acute axonal:
Chronic axonal:

increased or absent
increased or absent
normal or absent
normal or absent

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``` H-reflex Early demyelinating: Chronic demyelinating: Acute axonal: Chronic axonal: ```

increased latency or absent increased latency or absent absent absent

``` SNAP amplitude Early demyelinating: Chronic demyelinating: Acute axonal: Chronic axonal: ```

Decreased or absent decreased or absent decreased or absent decreased or absent

``` CMAP amplitude Early demyelinating: Chronic demyelinating: Acute axonal: Chronic axonal: ```

normal or decreased normal or decreased decreased decreased

``` MUAP duration Early demyelinating: Chronic demyelinating: Acute axonal: Chronic axonal: ```

normal normal or increased normal increased

``` MUAP amplitude Early demyelinating: Chronic demyelinating: Acute axonal: Chronic axonal: ```

normal normal or increased normal increased

``` polyphasics (subacute 3-6 months) Early demyelinating: Chronic demyelinating: Acute axonal: Chronic axonal: ```

normal increased normal increased

``` Recruitment Early demyelinating: Chronic demyelinating: Acute axonal: Chronic axonal: ```

normal or decreased decreased, rapid decreased, rapid decreased, rapid

``` abnormal spontaneous activity Early demyelinating: Chronic demyelinating: Acute axonal: Chronic axonal: ```

none none or fibs, PSW, CRD Fib/PSW, CRD none or fibs/PSW, CRDs

Uniform demyelinating mixed sensory-motor polyneuropathy is (inherited/acquired)? affects _____ fibers

inherited sensory and motor

What are the NCS findings for Uniform demyelinating mixed sensory motor polyneuropathy? (6)

1 prolonged latency 2. slow conduction velocity <60--80 (85?)% than normal 3. Morphology from proximal and distal stimulations are similar 4. prolonged F 5. minimal/no temporal dispersion, no conduction block 6. decreased amplitudes (low normal)

HSMN I is also known as ______. Nerve biopsy looks like _____. due to? Present in _____ yoa

Charcomarie tooth (different types IA, IB, IC) onion bulb formation, due to demyelination and remyelination process 20-30 yoa

Pathology? patient presents in 20-30s with clumsiness, multiple sprained ankles, pes cavus deformity, hammer toes, halux valgus, enlarged nerves, scoliosis, absent reflexes, decreased vibration/position sense what if essential tremor is involved?

HMSN I - CMT roussy levy syndrome can sometimes palpate sural nerve bc it is enlarged and has demyelination and remyelination.

CMT is inherited how? Decreased CV is averaged to be _____m/s or ____% below normal.

autosomal dominant 15-25m/s 60-80%

HMSN III is also known as _______. This is the ____ form. _____ inheritence. Presents in _____

Dejerene Sottas Syndrome severe autosomal recessive infancy

pathology? neurogenic kyphoscoliosis, pes cavus as babies, ataxia, areflexia, enlarged nerves (greater aricular, sural - can palpate)

HMSN III - Dejerene Sottas Syndrome

What is the difference between acquired and hereditary demyelinating sensorimotor peripheral neuropathies?

partial conduction block

HMSN IV is also known as _____. due to ______. Associated with ___ eye disorder Inheritence: Increased _____ protein

Refsum's disease lipid metabolism abnormality (phytanic acid oxidation deficiency) - failure of phytanic acid to undergo alpha oxidation retinitis pigmentosa (night blindness) --- also cerebellar dysfunction/ataxia. AR CSF protein

What is the uniform demyelinating mixed sensorimotor peripheral neuropathy that shows cognitive issues, seen in infancy secondary to a metabolic disorder?

Krabbe Leukodystrophy

EMG/NCS findings: 1. CV slow to 30s 2. varying degrees of conduction block (amplitude drop by 20% with no change in negative spike duration) 3. Pathologic temporal dispersion 4. increased phases when compare distal to proximal 5. increased peak and onset latency 6. prolonged or absent F wave 7. patchy sensory studies

acquired segmental demyelinization motor > sensory

What pathology? 1. ascending symmetrical weakness 2. distal > proximal 3. legs > arms 4. 70% after viral illness (gastroenteritis, URI) 5. spares occular (no diplopia) and sphincter muscles 6. Areflexic 7. positives seen on long loops (f waves)

AIDP - Guillain Barre

what pathology 1. symmetric onset 2. proximal > distal 3. three different types. 4. areflexic 5. varying courses.

CIDP

CIDP has varying courses _____% can have 1 episode then done ____% have slow decline ___% have slow stepwise decline _____% have rapid stepwise

40 15 15 30 types: slow monophasic (does not progress) chronic relapsing slow progressing stepwise

pathology - asymmetrical weakness, arms > legs - conduction block (loss of 20-25% amplitude from proximal to distal) in multiple nerves. - Fasciculatios seen - no sensory problems - antiglycolipid antibodies (anti GM1 antibodies) seen in this autoimmune disorder

multifocal motor neuronopathy with partial conduction block (MMNPCB) in differential with ALS

what is the pathology behind multifocal motor neuronopathy with partial conduction block? which category of peripheral neuropathy is it

anti-GM 1 antibodies - antiglycolipid antibodies segmental demyelinating motor>sensory peripheral neuropathy

Monoclonal gamopathy of undetermined significance (MGUS) is associated with ______ . Polyneuropathy starts ______ before they develop this. Check ____ (lab)

colon cancer 3-4 years before they develop cancer check CMP total protein will be high. Then get serum protein electrophoresis. They will have a spike of gamma portion of SPEP.

EMG/NCS findings for axonal loss motor>sensory

1. small amplitudes - the one that is proximal is just as small as the stimulation that is distal (50% of expected value) 2. No pathological temporal dispersion (small amount of physiologic temporal dispersion) 3. No conduction block 4. slightly increased latencies 5. slightly decreased CV (bc loose the fastest firing fibers due to axonal damage) 6. EMG: fibs/PSWs

Pathology? ``` Autosomal dominant inheritance incomplete penetrance (can have gene but not disease) classic triumvirate: psychosis, abd pain, polyneuropathy ```

porphyria

HSMN II also known as _____ presents in ____ decade inheritence? ____ amplitude, _____ CV

Neuronal CMT 3rd and 4th decade AD small, preserved CV (mid-upper 30s) will see pes cavus, hammer toes, leg muscle atrophy

name the three types of AiDP

1. demyelinating - segmental 2. axonal (fibs/PSW) 3. miller fischer varient

HSMN II belongs to which category of peripheral neuropathy

axonal loss motor > sensory

What is the classic triad of porphyria

psychosis, abd pain, polyneuropathy

Vincristine neuropathy shows what kind of peripheral neuropathy?

axonal loss motor > sensory months to years later.

Axonal loss sensory neuropathy and neuronopathy affects the _____

dorsal root ganglion (PURE SENSORY)

EMG/NCS findings for axonal loss sensory neuropathy: (5)

1. only sensory nerve affected 2. decreased amplitudes (proximal and distal waves) 3. normal motor NCS 4. slightly decreased CV (lose `fastest firing fibers) 5. EMG will be normal b/c EMG assesses motor

Friedreichs ataxia is what form of peripheral neuropathy? inheritance?

axonal loss pure sensory

pathology? cerebellar degeneration, areflexia, ataxia, scoliosis, pes cavus, nystagmus, upgoing toes (UMN); posterior column loss, vibration sense, position sense and 2 pt discrimination affected

freidreichs ataxia

pathology? areflexia, ataxia, diplopia, and ophthalmoplegia. increased CSF proeint. Associated with Gq1B antibody

miller fischer variant

cisplatinum belongs to which peripheral neuropathy

sensory axonal loss

alcohol, pharmaceuticals, neutritional deficits, toxic neuropathies (CO2, organophosphates), CIP are all belonging to which peripheral neuropathy

axonal loss sensorimotor

Pathology? 1. slowed CV in upper extremity limit of normal 32 2. slowed CV in lower extremity lower limit of normal 28 3. no temporal dispersion (differentiates from axonal demyelinating sensorymotor polyneuropathy) - prox and distal stim are the same. 4. prolonged latencies (onset and peak) 5. small amplitudes 6. EMG with fibs/PSWs

axonal loss mixed sensorimotor

pathology 1. pathological temporal dispersion 2. slowed CV 3. decreased amplitudes 4. prolonged latencies 5. axonal timeline on EMG

axonal demyelinating mixed sensorimotor polyneuropathy (acquired)

what are the 6 different ways someone can have diabetic polyneuropathy?

1. Sensory 2. Sensorimotor 3. Autonomic (gastroporesis) - presents as orthostasis, neurogenic B/B, abnormal sweating) 4. Diabetic amyotrophy - focal lumbar polyneuropathy radiculopathy (multiple roots) 5. Mononeuritis multiplex - polyneuropathy of blood supply to nerve (vaso nevorum) - one nerve in multiple spots along that nerve 6. Cranial nerve involvement

uremia belongs to which peripheral neuropathy

axonal demyelinating sensorimotor polyneuropathy (acquired) will continue to have neuropathy after kidney transplant

which diabetic peripheral neuropathy presents with gastroparesis, orthostasis, neurogenic b/b, and abnormal sweating?

DM autonomic polyneuropathy

pathology - inflamed nerve - injured blood supply to the nerve - axonal abnormalities - asymmetric - patchy - vasculitis and DM associated (poorly controlled)

mononeuritis multiplex