Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease in adults. It affects motor neurons in the central nervous system. The discovery of novel mutations in the gene encoding SOD1 that cause familial ALS has led to the development of genetically engineered animal models that have substantially enhanced our understanding of this disease. In nearly 98% of ALS cases, the cause for the disease is unknown. There are several proposed mechanisms which may produce the clinical phenotype of ALS. This article reviews the various aspects of ALS, including clinical features, pathology, epidemiology, pathogenic mechanisms, clinical management, and potential therapies.

[1]  R. Sufit,et al.  Promoting excellence in end‐of‐life care in ALS , 2005, Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases.

[2]  T. Munsat,et al.  Amyotrophic Lateral Sclerosis: A Guide for Patients and Families , 2001 .

[3]  P. Shaw,et al.  Molecular and cellular pathways of neurodegeneration in motor neurone disease , 2005, Journal of Neurology, Neurosurgery & Psychiatry.

[4]  J. Rabkin,et al.  Bilateral extraocular muscle metastasis from primary breast cancer , 2005, Neurology.

[5]  L. Bruijn,et al.  Unraveling the mechanisms involved in motor neuron degeneration in ALS. , 2004, Annual review of neuroscience.

[6]  P. Andersen Amyotrophic lateral sclerosis associated with mutations in the CuZn superoxide dismutase gene , 2006, Current neurology and neuroscience reports.

[7]  D. Thurman,et al.  Temporal and geographic variation in United States motor neuron disease mortality, 1969–1998 , 2005, Neurology.