Amyotrophic Lateral Sclerosis
- 1 October 1997
- journal article
- review article
- Published by American Medical Association (AMA) in Archives of Neurology
- Vol. 54 (10), 1246-1250
- https://doi.org/10.1001/archneur.1997.00550220050013
Abstract
Amyotrophic lateral sclerosis (ALS) is among the most dire neurological diseases. The essential clinical feature of the disorder is relentless, lethal paralysis, usually beginning in midadult years. The disease is caused by a slow, progressive loss of motor neurons in the brain and spinal cord. It usually begins focally and then spreads. In most cases, there is concurrent involvement of corticospinal (upper) and spinal (lower) motor neurons, although in some instances the spinal motor neuron features predominate. Involvement of the spinal motor neurons produces muscle denervation of the affected muscles and fasciculations, followed by muscle atrophy. When corticospinal motor neurons degenerate, the weakness is accompanied by spasticity. The mean age at onset of ALS is 55 years; the mean duration is about 4 years. The incidence of new cases is approximately 1 per 100 000 population. The total number of cases is about 5 per 100 000 population. In the United States, it is estimated that there are 20 000 to 30 000 cases. About 10% of cases are inherited as an autosomal dominant trait1; familial and sporadic ALS are clinically indistinguishable.Keywords
This publication has 20 references indexed in Scilit:
- Selective loss of glial glutamate transporter GLT‐1 in amyotrophic lateral sclerosisAnnals of Neurology, 1995
- Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis.Proceedings of the National Academy of Sciences, 1995
- A Controlled Trial of Riluzole in Amyotrophic Lateral SclerosisNew England Journal of Medicine, 1994
- Age‐Dependent Impairment of Mitochondrial Function in Primate BrainJournal of Neurochemistry, 1993
- Decreased Glutamate Transport by the Brain and Spinal Cord in Amyotrophic Lateral SclerosisNew England Journal of Medicine, 1992
- HEREDITARY MOTOR SYSTEM DISEASES (CHRONIC JUVENILE AMYOTROPHIC LATERAL SCLEROSIS)Brain, 1990
- Ubiquitin deposits in anterior horn cells in motor neurone diseaseNeuroscience Letters, 1988
- Biological effects of the superoxide radicalArchives of Biochemistry and Biophysics, 1986
- Familial adult motor neuron disease: amyotrophic lateralsclerosisNeurology, 1986
- The central nervous system in motor neurone diseaseJournal of Neurology, Neurosurgery & Psychiatry, 1970