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Classic Amyotrophic Lateral Sclerosis With Dementia
Juhani Wikström, MD;
Anders Paetau, MD;
Jorma Palo, MD;
Raimo Sulkava, MD;
Matti Haltia, MD
Arch Neurol. 1982;39(11):681-683.
Abstract
• Documented cases of classic amyotrophic lateral sclerosis (ALS) combined with severe dementia are few and come from the isolated populations of the Western Pacific. In this report, three women had a clinically and neuropathologically documented combination of ALS and dementia. In all cases the symptoms and signs of ALS were mainly bulbar. In two of them dementia appeared first, followed by ALS. At autopsy, there was a marked discrepancy between the severe degree of dementia of Alzheimer's type and the slight nonspecific neuropathologic findings without Alzheimer's changes. Our cases bear a close resemblance to recently published Japanese cases. It may be concluded that the combination of sporadic ALS and progressive dementia seems to be a clinical entity without consistent neuropathologic changes and that it also occurs in the western countries.
Author Affiliations
From the Departments of Neurology (Drs Wikström, Palo, and Sulkava) and Pathology (Drs Paetau and Haltia), University of Helsinki.
Footnotes
Accepted for publication Dec 31, 1981.
Reprint requests to Department of Neurology, University of Helsinki, Haartmaninkatu 4, SF-00290 Helsinki 29, Finland (Dr Wikström).
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