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March 1967

Familial Amyotrophic Lateral Sclerosis: A Subgroup Characterized by Posterior and Spinocerebellar Tract Involvement and Hyaline Inclusions in the Anterior Horn Cells

Author Affiliations

New York; Rochester, Minn
From the Laboratory Division, Montefiore Hospital and Medical Center, New York (Dr. Hiranoand Section of Medical Statistics, Epidemiology, and Population Genetics (Dr. Kurland), and Section of Experimental and Anatomic Pathology (Dr. Sayre), Mayo Clinic, Rochester, Minn.

Arch Neurol. 1967;16(3):232-243. doi:10.1001/archneur.1967.00470210008002

IT HAS BEEN suggested that there may be at least three major types of amyotrophic lateral sclerosis (ALS)1,2: (1) the sporadic or classic type, (2) the familial and presumably hereditary type which accounts for about 5% to 10% of recent series of ALS cases,3 and (3) the Marianas Islands type which is so prevalent on Guam and other islands in the western Pacific.4 The relationships of these various forms of ALS to one another are still unclarified. Individual cases of the three types cannot be distinguished on clinical grounds alone. However, in the Marianas Islands type upper motor neuron features may be more prominent initially, the mean age of onset is a few years less than that of the classic type, and, pathologically, in addition to the usual findings in ALS, there are characteristic and often widespread neurofibrillary changes, even in relatively young patients.4

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