Amyotrophic Lateral Sclerosis (ALS) is a group of neurodegenerative diseases that motor nerve cells in the brain, the brain stem and spinal affected. As a result loss of function and muscle atrophy occurs.
There are several types: sporadic ( the most common), familial and territorial (typical of the island of Guam). In turn they are classified as classical training of ALS, spinal muscular atrophy, progressive bulbar palsy, pseudobulbar palsy and primary lateral sclerosis.
ALS affects people between 40 and 70 years with higher incidence in males. The cause is unknown but seems to be linked to the accumulation of protein (SOD1, TDP43 and FUS) in nerve cells or neurons, leading to destroy. It is also related with accumulation of glutamate, a neurotransmitter. When different forms, it is suspected that the cause may be different. There are genetic factors (such as mutation of a gene) but there are also studies that point to autoimmune origin.Although the disease varies in each individual, these symptoms may occur:
- Fasciculations (involuntary muscle contractions in the hands and feet)
- Progressive motor function loss: loss of strength and awkwardness, especially in the hands and feet
- Muscle fatigability (loss of strength with exercise)
- Frequent falls, for involvement in legs
- Crying or laughing uncontrollably
- Slurred speech or swallowing
The diagnosis must make a Neurologist. It is based on clinical examination, imaging tests, laboratory tests-including analysis of cerebrospinal fluid obtained with Lumbar puncture and neuropsychological tests.
Unfortunately , there is no curative treatment. It is a drug that slows, in part, the progression of the disease. Although not all are equal ELA, the earlier therapy starts, the greater the effect.