Lou Gehrig's disease

Lou Gehrig's disease

Introduction

Amyotrophic lateral sclerosis (abbreviated ALS, also called Lou Gehrig's disease, and in France, Charcot) is a degenerative disease of neuromuscular type. It occurs when some nerve cells called motor neurons gradually reduce their operation and die, causing paralysis, muscular progressive fatal prognosis: in its advanced stages patients experience total paralysis is accompanied by an exaltation of tendon reflexes (the result of loss of muscle control inhibitory).

Description

Despite being the most serious disease of motor neurons, ALS is just one of many diseases that exist in affected nerve cells. Among others, are included in this type of disease, spinal muscular atrophy and its variants youth and children, which only affect spinal motor neurons, the primary lateral sclerosis (PLS) in which only affect central motor neurons (brain ) and Kennedy disease or (spinobulbar progressive muscular atrophy ) is a genetic disorder that affects middle-aged men (Parker, 2004).

Some mechanisms arouse the suspicions of investigators as to the origin of the achievement of neurons that make up the nervous system. These include the deregulation of cell in the management of oxidative stress, the programmed cell death and the phenomenon of excitotoxicity. That said, no cause is officially foreshadowed be good. There are two types of Charcot's disease, the sporadic and familial. The first is about 90% of people with the disease. As for Lou Gehrig's disease family, it concerns only a minority of patients. This case of ALS is inherited and passed down from generation to generation while keeping the option of skipping a line. The incidence of this disease worsens from 40 years. However, this disease usually occurs in adults aged 50 to 70 years.

History

The name of the disease, first described in 1869 by French physician Jean Martin Charcot (1825-1893), specifying its main characteristics:

"Lateral sclerosis" indicates the loss of nerve fibers accompanied by a "sclerosis" or scarring glial in the side of the spinal cord, a region occupied by nerve fibers or axons that are ultimately responsible for control of voluntary movements.

"Sclerosis" (from the Greek, to: denial, my 'muscle'; trophic 'nutrition'), meanwhile, said the muscle atrophy that results from chronic muscle inactivity, having left the muscles receive nerve signals.

In ALS, brain functions not related to motor activity, that is, the sensitivity and intelligence remain unchanged. On the other hand, barely affected motoneurons need to control the extrinsic muscles of the eye, so to keep eye movements to the end. Similarly, the Commonwealth does not damage the nucleus of Onuf, so neither affected the sphincter muscles that control urination and defecation.

The disease affects especially people aged between 40 and 70 years, more frequently in men and between 60 and 69. Without knowing the specific cause, the Commonwealth has also affected, sometimes to groups of people: Italian football players, veterans of the Persian Gulf War and inhabitants of the island of Guam (Wade, 2001).

Symptoms

The Lou Gehrig's disease begins not always the same and does not break out even more with the same intensity. Symptoms and course of the disease vary from person to ...