PNS Demyelinating Disorders

Question 1

GB - Definition

Answer 1

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

Question 2

GB - other names you may hear

Answer 2

Postinfectious polyneuritis
Acute infectious polyneuritis
Landry-GB

Question 3

GB - Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

Answer 3

Chronically progressive or relapsing motor and sensory dysfunction
Symmetrical

Question 4

GB - CIDP - associated with

Answer 4

Lymphocyte and macrophage infiltrations of perivascular region of a nerve

Question 5

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - epidemiology

Answer 5

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

Question 6

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Etiology

Answer 6

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

Question 7

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

Answer 7

Viral infections 
Spirochetal infection
Bacterial infections
Vaccination
Surgery
Pregnancy

Question 8

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

Answer 8

Viral - Cytomegalovirus, Epstein Barr, Zika

Bacterial - Camplyobacter jejuni, mycoplasma pneumoniae

Question 9

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Pathology

Answer 9

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

Question 10

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

Answer 10

Distal to proximal

Question 11

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

Answer 11

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

Question 12

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis

Answer 12

CSF
Electrodiagnostics
Clinical findings

Question 13

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

Answer 13

Increased proteins

Normal cell counts

Question 14

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

Answer 14

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

Question 15

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

Answer 15

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

Question 16

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

Answer 16

CLinical findings

Question 17

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

Answer 17

Paralysis
Reflexes
Sensation
Pain
Afebrile 
Hypotonic 
Autonomic

Question 18

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

Answer 18

Ascending from distal to proximal

Symmetrical

Question 19

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

Answer 19

Decreased or absent (LMN problem since anterior horn cell)

Question 20

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

Answer 20

Paresthesias

Mild sensory loss on testing

Question 21

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

Answer 21

Bowel and bladder dysfunction
Sinus tachycardia
Arrhythmias
Postural hypotension

Question 22

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

Answer 22

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

Question 23

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

Answer 23

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

Question 24

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Phases

Answer 24

Acute
Static/Plateau
Recovery

Question 25

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

Answer 25

Weakness and sensory issues progress | Max deficit within 2 to 3 wks

Question 26

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

Answer 26

Not worse, not better | 4 days to 2 months

Question 27

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Recovery

Answer 27

Usually begins within 2-4 weeks after progression ceases

Question 28

GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Prognosis

Answer 28

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

Question 29

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

Answer 29

Pain | Chronic fatigue 80% report some level

Question 30

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

Answer 30

Plasmaphoresis Intravenous immune serum globulin (IVIg) Supportive

Question 31

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

Answer 31

Respiratory support - ventilatory assist if needed | Prevention of complications - PE, Aspiration, Dysautonomia (arrhythmias, GI, urinary retention)

Question 32

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

Answer 32

``` 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 ```

Question 33

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

Answer 33

Protect their body! Breathing PROM Positioning

Question 34

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

Answer 34

PROM, begin stretching | Pain control - positioning

Question 35

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

Answer 35

``` Think function Biofeedback Weigh shifts Activities to develop speed of contraction Developmental sequence activities ```

Question 36

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

Answer 36

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

Question 37

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

Answer 37

CSF Subjective findings Objective findings

Question 38

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

Answer 38

Sensory Fatigue Patient confidence

Question 39

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

Answer 39

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