PNS Demyelinating Disorders Flashcards

1
Q

GB - Definition

A

Demyelinating disease of the PNS - a number of variants exist
Classic form is Acute Inflammatory Demyelinating Polyneuropathy (AIDP)

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2
Q

GB - other names you may hear

A

Postinfectious polyneuritis
Acute infectious polyneuritis
Landry-GB

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3
Q

GB - Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

A

Chronically progressive or relapsing motor and sensory dysfunction
Symmetrical

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4
Q

GB - CIDP - associated with

A

Lymphocyte and macrophage infiltrations of perivascular region of a nerve

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5
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - epidemiology

A

1-3/100,000
Can occur at any age but usually 15-30, 50-75
M more than F
Physiologic stress may play role in onset

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6
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Etiology

A
Unknown
Immune system disorder
Misdirected allergic response
Environmental causes
Infection - usually respiratory or GI
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7
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - some known antecedent events

A
Viral infections 
Spirochetal infection
Bacterial infections
Vaccination
Surgery
Pregnancy
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8
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Known antecedent events - stronger associations with GB development

A

Viral - Cytomegalovirus, Epstein Barr, Zika

Bacterial - Camplyobacter jejuni, mycoplasma pneumoniae

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9
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Pathology

A

Multifocal neuropathy - ventral (anterior) root ganglia - motor
Inflammatory response with demyelination of axon of PNs

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10
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Demyelination occurs from

A

Distal to proximal

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11
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - 2 main pathologic processes

A
Primary demyelination
Axonal degeneration (this one will lead to more damage in the end)
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12
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis

A

CSF
Electrodiagnostics
Clinical findings

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13
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - CSF

A

Increased proteins

Normal cell counts

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14
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - Elecrodiagnosis

A

Not as useful in early stages
EMG, conduction blocks and demyelination effects
80% have dec nerve conduction velocity

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15
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - 3 out of 4

A

1 Reduced nerve conduction velocity
2 Conduction block or abnormal dispersion
3 Prolonged distal latencies
4 Prolonged F waves

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16
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - what is the most telling

A

CLinical findings

17
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - Clinical Findings

A
Paralysis
Reflexes
Sensation
Pain
Afebrile 
Hypotonic 
Autonomic
18
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - CLinical findings - Paralysis

A

Ascending from distal to proximal

Symmetrical

19
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - Clinical findings - Reflexes

A

Decreased or absent (LMN problem since anterior horn cell)

20
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - clinical findings - Sensation

A

Paresthesias

Mild sensory loss on testing

21
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - CLinical findings - autonomic

A

Bowel and bladder dysfunction
Sinus tachycardia
Arrhythmias
Postural hypotension

22
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Differential Diagnosis

A
Poliomyelitis
Diphtheritic polyneuropathy
HIV polyneuropathy
Myesthenia Gravis
Spinal cord tumors
Brainstem gliomas
Diabetic or alcohol neuropathies
23
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Clinical picture - typical presentation (8)

A

1 Non specific infection (2 to 6 wks prior to onset)
2 Paresthesia foot and hands
3 Progressive weakness of distal to proximal LE mm
4 Respiratory failure may develop at any time
5 Progressive weakness of distal to prox UE mm
6 Trunk and resp mm involvement
7 Bulbar/CN involvement may occur
8 Recovery

24
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Phases

A

Acute
Static/Plateau
Recovery

25
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Acute phase

A

Weakness and sensory issues progress

Max deficit within 2 to 3 wks

26
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Static/Plateau

A

Not worse, not better

4 days to 2 months

27
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Recovery

A

Usually begins within 2-4 weeks after progression ceases

28
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Prognosis

A

5% mortality rate
75% recover without significant neuro deficits
20% permanent, substantial neuro sequelae

29
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Long term impairments

A

Pain

Chronic fatigue 80% report some level

30
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Medical Intervention

A

Plasmaphoresis
Intravenous immune serum globulin (IVIg)
Supportive

31
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Supportive medical intervention consists of what

A

Respiratory support - ventilatory assist if needed

Prevention of complications - PE, Aspiration, Dysautonomia (arrhythmias, GI, urinary retention)

32
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Other medical interventions

A
Corticosteroids have been shown to have no real benefit for GB but helps with other s/s
Immunosuppressive therapy (interferon) not demonstrated significant effect either
Plasmaphoresis and IVIg are the primary two that have shown benefit
33
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - PT intervention - Acute phase

A

Protect their body!
Breathing
PROM
Positioning

34
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - PT intervention - Static phase

A

PROM, begin stretching

Pain control - positioning

35
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - PT intervention - Recovery phase

A
Think function
Biofeedback
Weigh shifts
Activities to develop speed of contraction
Developmental sequence activities
36
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - PT interventions - monitors

A

O2 saturation - monitor in all phases
Muscular aches and paresthesias - need to monitor after treatment sessions
Significant fatigue

37
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Indicators of progress

A

CSF
Subjective findings
Objective findings

38
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Indicators of progress - subjective findings

A

Sensory
Fatigue
Patient confidence

39
Q

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Indicators of progress - objective findings

A

EMG, NCS
Strength
Function, timed tests are particularly useful in latter stages of recovery