Amyotrophic Lateral Sclerosis
Lou Gehrig's Disease
By Audrea Emmons
Pathophysiology
• Amyotrophic lateral sclerosis (ALS), often referred to as "Lou
Gehrig's Disease," is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.
Prognosis
• To date, there is no one test or procedure to ultimately establish the diagnosis of ALS. It is through a clinical examination and series of diagnostic tests, often ruling out other diseases that mimic ALS.
• electrodiagnostic tests including electomyography (EMG) and nerve conduction velocity (NCV)
• blood and urine studies including high resolution serum protein electrophoresis, thyroid and parathyroid hormone levels and 24-hour urine collection for heavy metals
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spinal tap x-rays, including magnetic resonance imaging (MRI) myelogram of cervical spine muscle and/or nerve biopsy thorough neurological examination
Signs and Symptoms
• The onset of ALS may be so subtle that the symptoms are frequently
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overlooked. muscle weakness in one or more of the following: hands, arms, legs or the muscles of speech, swallowing or breathing twitching (fasciculation) and cramping of muscles, especially those in the hands and feet impairment of the use of the arms and legs
"thick speech" and difficulty in projecting the voice in more advanced stages, shortness of breath, difficulty in breathing and swallowing Treatment
There is no cure for ALS. Stem cells have emerged as a major tool for research into the causes of ALS, and in the search for new