The Syndromic Nature of Amyotrophic Lateral Sclerosis
Let me start with a confession, I do not believe in amyotrophic lateral sclerosis. This used to bother me, but now I recognize this disbelief as an advance and an advantage. It is an advance because it explains things previously unexplainable like the clinical variations in presentation, duration, and severity of the disorder, the occasional remissions and improvements and the unusual cases that are due to a definite cause. It is an advantage because it enables me to initiate a detailed evaluation of each patient, with a clear conscience that I am not wasting time or money and with reasonable expectation of success. I conduct the investigation as if the patient’s life depended on the outcome, as it often does. My motto is to spend as much time and money evaluating each patient with ALS as I would have spent if that patient had cancer or leukemia or some other usually serious and often fatal condition.
KeywordsAmyotrophic Lateral Sclerosis Motor Neuron Muscle Biopsy Spinal Muscular Atrophy Motor Neuron Disease
Unable to display preview. Download preview PDF.
- 1.A. Schanen, J. Mikol, C. Guizoiu, C. Vital, M. Coquet, A. Lagueny, J. Julien, and M. Haguenau, Forme familiale liee au sexe d1 amyotrophic spinale progressive de lfadulte, Rev.Neurol (Paris), 140: 720–727 (1984).Google Scholar
- 2.M. Kaback, J. Miles, M. Yaffe, H. Itabashi, H. Mclntyre, M. Goldberg, and T. Mohandas, Hexaminidase-A (Hex A) deificiency in early adulthood: a new type of GM2 gangliosidosis, Am.J.Hum.Genet., 30: 31A (1978).Google Scholar
- 5.B. Patten, Phosphate and parathyroid disorders associated with the syndrome of amyotrophic lateral sclerosis, in: “Human Motor Neuron Diseases”, L. P. Rowland, ed., pp. 181–200, Raven Press, New York (1982).Google Scholar
- 6.P. Stortebecker, Motor neuron disorder. Deficiency of arterial blood supply to spinal cord and brain stem, Stortebecker Foundation for Research, pp.53-60, Stockholm (1983).Google Scholar
- 7.B. Patten, Neuropathy and motor neuron syndromes associated with plasma cell disease, Acta Neurol.Scand., 70:47–61 (1984). This reference is often miscited as being in volume 69 of Acta Neurol. Scand. The correct volume is 70.Google Scholar
- 9.B. Patten, ALS of autoimmune origin, Neurology, Suppl. 1, 35: 251 (1985).Google Scholar
- 10.D. W. Mulder, ed., “The Diagnosis and Treatment of Amyotrophic Lateral Sclerosis”, Houghton Mifflin Professional Publishers, Medical Division, Boston, MA (1980).Google Scholar
- 11.L. P. Rowland, ed., “Human Motor Neuron Diseases. Advances in Neurology”, Vol. 36, Raven Press, New York (1982).Google Scholar
- 12.F. Clifford-Rose, ed., “Research Progress in Motor Neuron Disease”, Progress in Neurology Series, Pitman, Bath, UK (1984).Google Scholar