PNS Demyelinating Disorders Flashcards
GB - Definition
Demyelinating disease of the PNS - a number of variants exist
Classic form is Acute Inflammatory Demyelinating Polyneuropathy (AIDP)
GB - other names you may hear
Postinfectious polyneuritis
Acute infectious polyneuritis
Landry-GB
GB - Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)
Chronically progressive or relapsing motor and sensory dysfunction
Symmetrical
GB - CIDP - associated with
Lymphocyte and macrophage infiltrations of perivascular region of a nerve
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - epidemiology
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
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Etiology
Unknown Immune system disorder Misdirected allergic response Environmental causes Infection - usually respiratory or GI
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - some known antecedent events
Viral infections Spirochetal infection Bacterial infections Vaccination Surgery Pregnancy
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Known antecedent events - stronger associations with GB development
Viral - Cytomegalovirus, Epstein Barr, Zika
Bacterial - Camplyobacter jejuni, mycoplasma pneumoniae
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Pathology
Multifocal neuropathy - ventral (anterior) root ganglia - motor
Inflammatory response with demyelination of axon of PNs
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Demyelination occurs from
Distal to proximal
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - 2 main pathologic processes
Primary demyelination Axonal degeneration (this one will lead to more damage in the end)
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis
CSF
Electrodiagnostics
Clinical findings
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - CSF
Increased proteins
Normal cell counts
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - Elecrodiagnosis
Not as useful in early stages
EMG, conduction blocks and demyelination effects
80% have dec nerve conduction velocity
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - 3 out of 4
1 Reduced nerve conduction velocity
2 Conduction block or abnormal dispersion
3 Prolonged distal latencies
4 Prolonged F waves
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - what is the most telling
CLinical findings
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - Clinical Findings
Paralysis Reflexes Sensation Pain Afebrile Hypotonic Autonomic
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - CLinical findings - Paralysis
Ascending from distal to proximal
Symmetrical
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - Clinical findings - Reflexes
Decreased or absent (LMN problem since anterior horn cell)
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - clinical findings - Sensation
Paresthesias
Mild sensory loss on testing
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Diagnosis - CLinical findings - autonomic
Bowel and bladder dysfunction
Sinus tachycardia
Arrhythmias
Postural hypotension
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Differential Diagnosis
Poliomyelitis Diphtheritic polyneuropathy HIV polyneuropathy Myesthenia Gravis Spinal cord tumors Brainstem gliomas Diabetic or alcohol neuropathies
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Clinical picture - typical presentation (8)
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
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Phases
Acute
Static/Plateau
Recovery
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Acute phase
Weakness and sensory issues progress
Max deficit within 2 to 3 wks
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Static/Plateau
Not worse, not better
4 days to 2 months
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Recovery
Usually begins within 2-4 weeks after progression ceases
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Prognosis
5% mortality rate
75% recover without significant neuro deficits
20% permanent, substantial neuro sequelae
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Long term impairments
Pain
Chronic fatigue 80% report some level
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Medical Intervention
Plasmaphoresis
Intravenous immune serum globulin (IVIg)
Supportive
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Supportive medical intervention consists of what
Respiratory support - ventilatory assist if needed
Prevention of complications - PE, Aspiration, Dysautonomia (arrhythmias, GI, urinary retention)
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Other medical interventions
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
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - PT intervention - Acute phase
Protect their body!
Breathing
PROM
Positioning
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - PT intervention - Static phase
PROM, begin stretching
Pain control - positioning
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - PT intervention - Recovery phase
Think function Biofeedback Weigh shifts Activities to develop speed of contraction Developmental sequence activities
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - PT interventions - monitors
O2 saturation - monitor in all phases
Muscular aches and paresthesias - need to monitor after treatment sessions
Significant fatigue
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Indicators of progress
CSF
Subjective findings
Objective findings
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Indicators of progress - subjective findings
Sensory
Fatigue
Patient confidence
GB - Acute inflammatory demyelinating polyneuropathy (AIDP) - Indicators of progress - objective findings
EMG, NCS
Strength
Function, timed tests are particularly useful in latter stages of recovery