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  • Motor neuron disease

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  • 335.24 Primary lateral sclerosis

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  • G12.2 Motor neuron disease

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Description

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  • 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

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Essentials of Diagnosis1

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  • 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)

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General Considerations

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  • 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

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Demographics

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

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Signs and Symptoms

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  • 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

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Functional Implications

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  • 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

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Possible Contributing Causes

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

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Differential Diagnoses

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

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Laboratory Tests

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  • Blood tests to help rule out other diagnoses

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Imaging

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Diagnostic Procedures

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

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Medication

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  • Benzodiazepines or dantrolene sodium for spasticity

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Medical Procedures

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  • 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

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  • Gait
  • Wheelchair (w/c) mobility
  • Depression
  • Balance
    • Static sit
    • Dynamic sit
    • Static stand
    • Dynamic stand
    • Moving base of support (BOS)
  • Muscle strength
  • Muscle recruitment
  • Coordination
  • Postural control (as disease progresses)
  • Posture (as disease progresses)
  • ROM
  • Reflexes
    • Deep ...

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