Cognitive Deficits Associated With a Recently Reported Familial Neurodegenerative Disease
Open Access
- 1 September 2001
- journal article
- research article
- Published by American Medical Association (AMA) in Archives of Neurology
- Vol. 58 (9) , 1429-1434
- https://doi.org/10.1001/archneur.58.9.1429
Abstract
DEMENTIA is associated with a variety of degenerative brain processes that lead to distinct patterns of cognitive and behavioral decline.1 Occasionally, individuals demonstrate unusual patterns of cognitive decline or may lack the pathophysiology of known diseases, raising the question of an idiopathic or previously unknown dementing process. We have been observing a family in upstate New York in which a number of individuals have an unusual dementia associated with the presence of neuronal inclusion bodies (Collins bodies) that are composed primarily of neuroserpin, a brain-specific serine protease inhibitor (serpin).2Keywords
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