Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis is a serious neurologic disease characterized by progressive deterioration of the motor cells in the brain and spinal cord, affecting the motor neuron cells and the motor tracts in the brain and spinal cord. Thickening of tissue in the motor tracts of the lateral columns and anterior horns of the spinal cord . A chronic, progressive disease marked by gradual degeneration of the nerve cells in the central nervous system that control voluntary muscle movement. Eventually the ability of the brain to start and control voluntary movement is lost. 

Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig's disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles. The disease belongs to a group of disorders known as motor neuron diseases, which are characterized by the gradual degeneration and death of motor neurons. Individuals with ALS lose their strength and the ability to move their arms, legs, and body. When muscles in the diaphragm and chest wall fail, individuals lose the ability to breathe without ventilatory support.

Amyotrophic Lateral Sclerosis disease is relatively rare: About 5,000 new cases are diagnosed in the US each year. It almost always strikes after the age of 40, and afflicts more men than women.. The voluntary muscles weaken and become immobile. Involvement of both upper and lower motor neurons is characteristic. Patients develop variable hyperreflexia, clonus, spasticity, extensor plantar responses, and limb or tongue fasciculations

The earliest symptoms include muscle twitching, cramping, stiffness, or weakness. Speech may become slurred, and later there is difficulty chewing or swallowing. . Most individuals with type 1 amyotrophic lateral sclerosis die of respiratory failure within 3 to 5 years of the onset of symptoms. Symptoms of type 8 amyotrophic lateral sclerosis begin earlier than type 1 (between 25 and 44 years of age) but progress slowly over several years to several decades.

The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others. Motor neurons are nerve cells located in the brain, brainstem, and spinal cord that serve as controlling units and vital communication links between the nervous system and the voluntary muscles of the body. Messages from motor neurons in the brain (called upper motor neurons) are transmitted to motor neurons in the spinal cord (called lower motor neurons) and from them to particular muscles.

In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away (atrophy) , and twitch (fasciculations) . Eventually, the ability of the brain to start and control voluntary movement is lost. The disorder causes muscle weakness and atrophy; symptoms commonly appear in middle to late adulthood, with death in two to five years. This neuron loss causes muscles to weaken and waste away, leading to paralysis.

Causes of Amyotrophic Lateral Sclerosis

Some causes are given bellow

• Nearly 10% of ALS cases are familial; the disease is transmitted in an autosomal dominant fashion
• Sporadic ALS shares clinical features with familial ALS. However, no SOD1 mutations or polymorphisms have been identified in these patients. Common pathways of disease pathogenesis may play a role, with different molecular abnormalities that lead to similar phenotypes.
• Several studies have shown an inflammatory component to the affected spinal cord regions, with the presence of activated microglia, reactive astrocytes, and IgG deposition. Whether this reaction precedes or accompanies the molecular events that promote neuronal cell death is unknown.
• Although genetic factors are thought to play a role; about 10 percent of cases are clearly genetic, with a family history

Symptoms of Amyotrophic Lateral Sclerosis

Some symptoms - Progressive loss of muscle strength and coordination eventually interfere with the ability to perform routine activities, such as going up steps, getting out of a chair, or swallowing. Occasionally, breathing or swallowing muscles may be the first to be affected

Some listed here :

• paralysis
• muscle cramps
• voice changes, hoarseness
• speech impairment, slow or abnormal speech pattern
• difficulty swallowing, gags or chokes easily
• difficulty breathing (increasing effort required to breathe)
• head drop due to weak spinal and neck muscles
• A breathing test may be given to see if respiratory muscles are affected.
• Blood tests can exclude other conditions that may cause similar symptoms.

Treatment of Amyotrophic Lateral Scleros

Some treatment & advise are given here :

• Physicians can prescribe medications to help reduce fatigue, ease muscle cramps, control spasticity , and reduce excess saliva and phlegm .
• Drugs also are available to help patients with pain, depression , sleep disturbances, and constipation .
• Pharmacists can give advice on the proper use of medications and monitor a patient's prescriptions to avoid risks of drug interactions.
• With the help of occupational therapists, speech-generating devices can be activated by switches or mouse emulation techniques controlled by small physical movements of, for example, the head, finger or eyes.
• Useing of braces or a wheelchair
• Medical care in ALS is primarily palliative
• Medications such as baclofen and tizanidine may be used to relieve severe spasticity.

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