ALS

ALS (Amyotrophic Lateral Sclerosis) is a peripheral nerve disorder also known colloqially as Lou Gehrig’s Disease. The peripheral nervous system includes parts outside of the central nervous system which is made up of the brain and spinal cord. These are nerves such as the cranial which connect the head, face, eyes, nose, ears and muscles of the face to the brain.  The peripheral system also includes nerves that connect the spinal cord to other parts of the body. This includes the 31+ pairs of spinal nerves and 100 billion nerve cells.

In ALS there is damage to the axon (messenger of the cell body) because the myelin sheath (membrane surrounding the axon) has been degraded. Exposure and consequential damage to motor nerves weakens and paralyzes muscles. Sensory nerve damage results in abnormal sensations like loss of feeling which can be progressive and fatal. The condition is characterized by stiff clumsy movements and a wasting or atrophy of the muscles. Diagnosis is usually determinedly electromyography or MRI along with blood tests. There is no cure and treatments focus on alleviating symptoms. The cause itself is unknown. About 10% of cases are considered hereditary.

Signs usually begin in the hands but my also show themselves in the feet and throat first.  If the throat muscles are affected people will have difficulty speaking and swallowing while the excess saliva may cause drooling. In the hands and feet cramps are common and may present before any other initial weakness. Tremors, muscle twitching and spasms are not unusual. Symptoms may progress more on one side than another. People may experience fatigue and weight loss. Controlling facial expressions may become difficult and range from the unintentional to inappropriate. As the disease progresses there may also be a loss of control over emotional responses such as laughing when intending to cry. Breathing problems may also arise forcing some to utilize a ventilator. The prognosis is often bleak with 50% of people dying within 3 years of diagnosis.

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