Diseases affecting motor neurons, such as amyotrophic lateral sclerosis (Lou Gerhig's disease), hereditary spastic paraplegia and spinal bulbar muscular atrophy (Kennedy's disease) are a heterogeneous group of chronic progressive diseases and are among the most puzzling yet untreatable illnesses. Over the last decade, identification of mutations in genes predisposing to these disorders has provided the means to better understand their pathogenesis. The discovery 13 years ago of superoxide dismutase SOD1 mutations linked to ALS, which account for less than 2% of total cases, had a major impact in the field. However, despite intensive research effort, the pathways leading to the specific motor neurons degeneration in the presence of SOD1 mutations have not been fully identified.
     Compared to years past, people who come down with amyotrophic lateral sclerosis nowadays seem to have slower disease progression and to live long. Nonetheless, amyotrophic lateral sclerosis is still always fatal, ultimately. A history of head injury is associated with an increased risk of developing amyotrophic lateral sclerosis.

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Amyotrophic lateral sclerosis diagnosis
Researchers have identified three proteins found in significantly lower concentrations in the cerebrospinal fluid (CSF) of amyotrophic lateral sclerosis ALS patients than in healthy controls or patients with other neurologic diseases. At present, the diagnosis of amyotrophic lateral sclerosis is made by ruling out all treatable illnesses that may mimic amyotrophic lateral sclerosis, and waiting over time to see if it progresses. It can take up to 3 years to diagnose, and even then it is easily misdiagnosed. As a result, patients are left with a lot of uncertainty and anxiety as they watch themselves waste away. Three proteins are significantly reduced in the CSF of amyotrophic lateral sclerosis patients: a 4.8-kDa protein that is a proteolytic fragment of the neuroendocrine specific protein VGF; a 13.4-kDa protein identified as cystatin c; and a 6.7-kDa unidentified protein.
   Other muscles diseases are considered in the evaluation. Blood testing and muscle electrical testing with electromyography (EMG) and nerve conduction velocities (NCV) can be helpful for a diagnosis.

Diet and Lou Gehrig disease
A diet high in polyunsaturated fat and vitamin E is associated with a decreased likelihood of developing Lou Gehrig disease. The findings are based on a comparison of dietary intake for 132 patients with amyotrophic lateral sclerosis (before the disease set in) and 220 healthy "controls." High levels of polyunsaturated fatty acids or vitamin E seemed to decrease the risk of amyotrophic lateral sclerosis, while a high intake of both nutrients cut the risk even further, suggesting a synergistic effect. Specifically, a diet high in polyunsaturated fatty acids and vitamin E decreased the risk of developing amyotrophic lateral sclerosis ALS by 50 percent to 60 percent. By contrast, the researchers found no apparent anti- ALS effect for lycopene, flavonols, vitamin C, vitamin B2, glutamate, calcium, or plant-derived estrogens. Journal of Neurology, Neurosurgery, and Psychiatry, May 2006.

Amyotrophic Lateral Sclerosis symptom
Symptoms of amyotrophic lateral sclerosis ALS include progressive muscle weakness and wasting. Spontaneous tiny local areas of muscle twitching, called fasciculations, are characteristic in the majority of those afflicted with the disease. These may be sensed by the patient as muscle cramping. Lower extremity muscle wasting (atrophy) and weakness generally follows wasting of the arms, hands, and shoulders. Spastic muscles can be present.

Amyotrophic Lateral Sclerosis and Gulf War
Military service, particularly in the Gulf War, may be linked to development of amyotrophic lateral sclerosis. The evidence, however, is limited and inconsistent.

Amyotrophic lateral sclerosis prevalence
Four out of every 100,000 American has amyotrophic lateral sclerosis, also called Lou Gehrig's disease.

Amyotrophic Lateral Sclerosis questions
Q. Do you think lipoic acid would be helpful in preventing amyotrophic lateral sclerosis?
   A. It's an interesting question, but I don't know.