Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis or ALS:  The most common motor neuron disease of muscular atrophy, ALS results in degeneration of upper motor neurons in the medulla oblongata and lower motor neurons in the spinal cord.  This neurologic disease causes progressive physical degeneration but doses not impair the patient's mental status.  Onset usually occurs between ages 40 and 70.  Most patients diagnosis with ALS die within 3 to 10 years after onset, usually due to aspiration pneumonia or respiratory failure.

Precipitating factors for acute deterioration may include:  trauma, viral ;infections, and physical exhaustion.  Disorders that must be differentiated from ALS include CNS syphilis, multiple sclerosis, spinal cord tumors, and syringomyelia.

Cause:  May be autosomal dominant inheritance, nutritional deficiency of motor neurons related to a disturbance in enzyme metabolism, or autoimmune disorders that affect immune complexes in the renal glomerulus and basement membrane, or metabolic interference in nucleic acid production by the nerve fibers.

Symptoms:

Atropy and weakness (especially in the muscles of the forearms and the hands

Impaired speech

Difficulty chewing and swallowing

Difficulty breathing

Excessive drooling and possible choking

Does not impair mental status

Treatment:

No effective treatment exists for ALS

Management aims to control symptoms and provide emotional, psychological, and physical support

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