Sclerosis, primary lateral (PLS)

• Motor neuron disease

• 335.24 Primary lateral sclerosis

• G12.2 Motor neuron disease

• 5E: Impaired Motor Function and Sensory Integrity Associated With Progressive Disorders of the Central Nervous System

Description

• Motor neuron disease
• Degenerative neurologic disorder of the upper motor neurons in the brain and spinal cord
• First described by Dr. Jean-Martin Charcot
• Progressive weakness in the voluntary muscles
• Nerve cells degenerate causing weakness

Essentials of Diagnosis1

• Progression of symptoms over 3 years without evidence of lower motor neuron dysfunction
• Disease is progressive, but may persist for decades
• Evidence of loss of function in the lower extremities (LEs) first, then progressing to the muscles of speech and swallowing, and then loss in the upper extremities (UEs)

General Considerations

• Spontaneous onset for adults
• Breathing may be affected in the later stages of the disease
• Juvenile primary lateral sclerosis (JPLS) is linked to a mutation in the ALS2 gene

Demographics

• Onset 40 to 60 years-old; mean of 50 years-old
• Juvenile primary lateral sclerosis (JPLS)

Signs and Symptoms

• Antalgic gait
• Dysarthria
• Drooling, weakness of bulbar muscles
• LE onset with spastic paraparesis
• Later develop symptoms in the UEs and oropharyngeal muscles
• Entirely upper motor neuron signs and symptoms
• Sensation is spared
• Hyperactive reflexes
• Babinski sign
• Painful muscle spasms

Functional Implications

• Declining independence with bed mobility, transfers, ambulation
• Declining balance in sitting and standing
• Declining respiratory support for all daily and recreational activities
• Increasing need for advanced adaptive equipment

Possible Contributing Causes

• Sporadic, no know familial link for adults
• Juvenile form linked to gene mutation: ALS2

Differential Diagnoses

• Amyotrophic lateral sclerosis (ALS)
• Henoch-Schönlein purpura (HSP)
• Multiple sclerosis (MS)
• Spondylosis
• Meningioma
• Familial spastic paraplegia
• Tropical spastic paraparesis (TSP)
• Cerebral palsy (CP)

Laboratory Tests

• Blood tests to help rule out other diagnoses

Imaging

• MRI

Diagnostic Procedures

• Electromyography (EMG)

• Electromyography (EMG) will show neuropathic changes
• MRI may show slight atrophy of the motor cortices and Wallerian degeneration of the motor tracts

Medication

• Benzodiazepines or dantrolene sodium for spasticity

Medical Procedures

• To neurologist for confirmation of diagnosis and disease management
• To respiratory therapist for breathing support and devices
• To occupational therapist for ADL, UE splinting, and adaptive equipment
• To ALS Association for support and services

• Gait
• Wheelchair (w/c) mobility
• Depression
• Balance
  • Static sit
  • Dynamic sit
  • Static stand
  • Dynamic stand
  • Moving base of support (BOS)
• Muscle strength
• Muscle recruitment
...