Table 5-60, Motor Neuron Disease: ALS, Polio, Postpoliomyelitis - Physical Medicine and Rehabilitation Board Review

Characteristics	Amyotrophic Lateral Sclerosis (Als)	Poliomyelitis	Postpoliomyelitis Syndrome
Pathology	Degeneration of the anterior horn cell	Degeneration of the anterior horn cell	Loss of the anterior horn cell
Etiology	Unknown	Picornavirus orally enters the body and spreads via lymphoid system leading to orphaned muscle fibers	Death of the motor neuron due to aging Burnout of motor unit from increased metabolic demand. (Figure 5-127)
Clinical Presentation	Most commonly in men after the sixth decade First signs: asymmetric atrophy, weakness, fasciculations Dysphagia (oral, pharyngeal), dysarthria Pseudobulbar signs (crying and laughing) Bowel and bladder are spared Sensation is spared Extraocular muscles are spared Upper and lower motor neuron signs Prognosis: 50% die within 3 years, 30% live for 5 years, 10% live for 10 years Wheelchair by 12–18 months Predictors of survival: Age of onset (younger is better) Severity of onset Pulmonary function	Signs of infection: fever, malaise, sore throat, vomiting headache, back and neck pain and stiffness Weakness Absent MSR Bulbar palsies: dysphasia, nasal voice Sensation is spared Autonomic dysfunction can occur Prognosis Disease can progress or remit 25%: Severe disability 25%: Mild disability 50%: Complete recovery Mortality: 1%–4% chance in children. 10% chance in adults with bulbar and respiratory involvement	Halstead-Ross Criteria History of a previous diagnosis Recovery of function Stability for approximately 15 years Return of symptoms No other medical problems to explain new symptoms: Weakness Atrophy Fatigue Arthralgia Myalgia Cold intolerance
EDX Findings	NCS SNAP: Normal CMAP: Normal EMG AA, DR, LDLA MUAP LRRS Increased decrement SFEMG Increased jitter and fiber density Protocol: Abnormal activity in two muscles from two different nerve roots in three different body regions. Body Regions: Brain stem, Cervical, Thoracic, Lumbar	NCS SNAP: Normal CMAP: Normal EMG AA, DR, LDLA MUAP	NCS SNAP: Normal CMAP: Abnormal EMG AA, DR, GIANT MUAP LRRS Normal activity SFEMG Increased jitter, fiber density and Blocking Postpoliomeylitis syndrome electrophysiologically resembles old stable poliomyelitis. Its diagnosis is not based on EMG/NCS but on clinical presentation.
Treatment	Rehabilitation, prevent contractures, tracheostomy, respiratory therapy, riluzole (Rilutek^®) antiglutamate slows disease progression, prolongs ventilator time	Rehabilitation, pain management, prevent contractures	Rehabilitation, assistive devices, energy conservation, psychological counseling, avoid fatigue

Characteristics

Amyotrophic Lateral Sclerosis (Als)

Poliomyelitis

Postpoliomyelitis Syndrome

Pathology

Degeneration of the anterior horn cell

Loss of the anterior horn cell

Etiology

Unknown

Picornavirus orally enters the body and spreads via lymphoid system leading to orphaned muscle fibers

Death of the motor neuron due to aging Burnout of motor unit from increased metabolic demand. (Figure 5-127)

Clinical Presentation

Most commonly in men after the sixth decade
First signs: asymmetric atrophy, weakness, fasciculations
Dysphagia (oral, pharyngeal), dysarthria
Pseudobulbar signs (crying and laughing)
Bowel and bladder are spared
Sensation is spared
Extraocular muscles are spared
Upper and lower motor neuron signs
Prognosis: 50% die within 3 years, 30% live for 5 years, 10% live for 10 years
Wheelchair by 12–18 months
Predictors of survival:
- Age of onset (younger is better)
- Severity of onset
- Pulmonary function

Signs of infection: fever, malaise, sore throat, vomiting headache, back and neck pain and stiffness
Weakness
Absent MSR
Bulbar palsies: dysphasia, nasal voice
Sensation is spared
Autonomic dysfunction can occur
Prognosis Disease can progress or remit
25%: Severe disability
25%: Mild disability
50%: Complete recovery
Mortality: 1%–4% chance in children. 10% chance in adults with bulbar and respiratory involvement

Halstead-Ross Criteria

History of a previous diagnosis
Recovery of function
Stability for approximately 15 years
Return of symptoms
No other medical problems to explain new symptoms:
- Weakness
- Atrophy
- Fatigue
- Arthralgia
- Myalgia
- Cold intolerance

EDX Findings

NCS

SNAP: Normal

CMAP: Normal
EMG

AA, DR, LDLA MUAP
LRRS

Increased decrement
SFEMG
Increased jitter and fiber density
Protocol:
Abnormal activity in two muscles from two different nerve roots in three different body regions.
Body Regions:
Brain stem, Cervical, Thoracic, Lumbar

NCS

SNAP: Normal

CMAP: Normal
EMG
AA, DR, LDLA MUAP

NCS

SNAP: Normal

CMAP: Abnormal
EMG
AA, DR, GIANT MUAP
LRRS

Normal activity
SFEMG

Increased jitter, fiber density and Blocking

Postpoliomeylitis syndrome electrophysiologically resembles old stable poliomyelitis. Its diagnosis is not based on EMG/NCS but on clinical presentation.

Treatment

Rehabilitation, prevent contractures, tracheostomy, respiratory therapy, riluzole (Rilutek^®) antiglutamate slows disease progression, prolongs ventilator time

Rehabilitation, pain management, prevent contractures

Rehabilitation, assistive devices, energy conservation, psychological counseling, avoid fatigue

From: Motor Neuron Disease

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Table 5-60Motor Neuron Disease: ALS, Polio, Postpoliomyelitis

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