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. Cases of amyotrophic lateral sclerosis or ALS-like conditions have arisen in apparent association with HMG-CoA reductase inhibitors (statin drugs) and/or other lipid-lowering drugs.
     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.

Fruit and vegetable intake reduces risk for amyotrophic lateral sclerosis
Fruit and Vegetable Intake and Risk of Amyotrophic Lateral Sclerosis in Japan.
Neuroepidemiology. 2009 February. Okamoto K, Kihira T, Kobashi G, Washio M, Sasaki S, Yokoyama T, Miyake Y, Sakamoto N, Inaba Y, Nagai M. Department of Public Health, Aichi Prefectural College of Nursing and Health, Nagoya, Japan.
There has been little interest in the role of nutrition in the prevention of amyotrophic lateral sclerosis. We investigated the relationship between dietary intake of vegetables, fruit, and antioxidants and the risk of amyotrophic lateral sclerosis in Japan. Between 2000 and 2004, we recruited 153 amyotrophic lateral sclerosis patients aged 18-81 years with disease duration of 3 years within the study period in accordance with El Escorial World Federation of Neurology criteria. Three hundred and six gender- and age-matched controls were randomly selected from the general population. A higher consumption of all fruits and vegetables and fruit alone in the highest quartiles was associated with a statistically significantly reduced risk of amyotrophic lateral sclerosis. Although not statistically significant, a beneficial association between intake of all vegetables, green and yellow vegetables and other vegetables and amyotrophic lateral sclerosis was found. No statistically significant dose-response relationship was observed between intake of beta-carotene, vitamin C and vitamin E and the risk of amyotrophic lateral sclerosis. Our findings suggest that higher intake of food rich in antioxidants such as fruit and vegetables confer protection against the development of ALS.

Role of nutrition and exercise
Nutritional and exercise-based interventions in the treatment of amyotrophic lateral sclerosis.
Clin Nutr. 2009 Sep 24. Patel BP, Hamadeh MJ. School of Kinesiology and Health Science, York University, Toronto, Ontario, Canada M3J 1P3; Muscle Health Research Centre, York University, Toronto, Ontario, Canada M3J 1P3.
Disease pathogenesis in amyotrophic lateral sclerosis involves a number of interconnected mechanisms all resulting in the rapid deterioration of motor neurons. The main mechanisms include enhanced free radical production, protein misfolding, aberrant protein aggregation, excitotoxicity, mitochondrial dysfunction, neuroinflammation and apoptosis. The aim of this review is to assess the efficacy of using nutrition and exercise-related interventions to improve disease outcomes in ALS. Studies involving nutrition or exercise in human and animal models of ALS were reviewed. Treatments conducted in animal models of ALS have not consistently translated into beneficial results in clinical trials due to poor design, lack of power and short study duration, as well as differences in the genetic backgrounds, treatment dosages and disease pathology between animals and humans. However, vitamin E, folic acid, alpha lipoic acid, lyophilized red wine, coenzyme Q10, epigallocatechin gallate EGCG, Ginkgo biloba, melatonin, Copper chelators, and regular low and moderate intensity exercise, as well as treatments with catalase and l-carnitine, hold promise to mitigating the effects of ALS, whereas caloric restriction, malnutrition and high-intensity exercise are contraindicated in this disease model.

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.