Summary
All identified Israeli patients with amyotrophic lateral sclerosis (ALS) with onset of the disease from 1959 through 1975 (n=318) were evaluated clinically. Most of our patients (63%) presented with weakness; only 10% presented with atrophy and 3% with fasciculations. In 31% of the cases, the onset of the disease was focal and 22% of the patients presented with bulbar signs, but only 6 patients presented with emotional lability (pseudo-bulbar). Twelve per cent of the patients presented with muscle cramps, pain or paraesthesia. Atypical signs such as motor cranial nerve lesion, dementia, sphincter disturbance and deep sensation loss are discussed. A relatively high proportion of our patients suffered from malignant tumour, but with no association with any specific tumour. The median survival time was 3 years. Patients with onset of their disease with bulbar signs had a shorter life expectancy (2.2 years): Twenty nine per cent of our patients survived for more than 5 years and 16% for more than 10 years.
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Supported in part by grants from the Amyotrophic Lateral Sclerosis Society of America, and Yad Avi-Hayishuv, Israel
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Gubbay, S.S., Kahana, E., Zilber, N. et al. Amyotrophic lateral sclerosis. A study of its presentation and prognosis. J Neurol 232, 295–300 (1985). https://doi.org/10.1007/BF00313868
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DOI: https://doi.org/10.1007/BF00313868