학술논문
Sporadic Parkinson disease and Amyotrophic Lateral Sclerosis complex (Brait–Fahn–Schwartz Disease)
Document Type
Article
Author
Source
Subject
*PARKINSON'S disease
*AMYOTROPHIC lateral sclerosis
*DOPA
*NEURODEGENERATION
*NOSOLOGY
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*
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*
Language
ISSN
0022-510X
Abstract
Abstract: Clinical evidence for parkinsonism may accompany Amyotrophic Lateral Sclerosis with a frequency ranging from 5% to 17%. The concurrence of Amyotrophic Lateral Sclerosis and Parkinson''s disease, outside the known Guam and Kii Peninsula foci, is instead rare, but this raises the possibility of a common pathogenesis. Clinically this complex presents with a levodopa-responsive parkinsonism and Amyotrophic Lateral Sclerosis and has been termed Brait–Fahn–Schwartz disease. Here we describe two patients with this uncommon neurodegenerative complex. Both presented with Parkinson disease and progressed to a full blown Amyotrophic Lateral Sclerosis. We further suggest that the association of Parkinson disease and Amyotrophic Lateral Sclerosis represents a distinct nosological entity, which should be kept separated from extrapyramidal signs and symptoms that may occur in Amyotrophic Lateral Sclerosis. [Copyright &y& Elsevier]