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Clinical Lewy body syndromes

Lookup NU author(s): Professor Ian McKeith

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Abstract

Lewy bodies are spherical, intracytoplasmic, eosinophilic, neuronal inclusions comprising abnormally truncated and phosphorylated intermediate neurofilament proteins, alpha -synuclein, ubiquitin, and associated enzymes. The clinical presentation of LB disease varies according to the site of LB formation and associated neuronal loss. Three main clinicopathological syndromes have been described-movement disorder, autonomic failure, and dementia. Parkinsonism. is the most common presentation of LB disease developing in middle life. In older patients, a mixture of cognitive, autonomic, and motor dysfunction is more common. Dementia with LB (DLB) is a relatively recently described clinicopathological syndrome that accounts for up to 20% of all cases of dementia in old age. Patients, typically in their seventh and eighth decades, have LB pathology in cortical neurons as well as in the brain stem. LB disease should be considered in the differential diagnosis of a wide range of clinical presentations including episodic disturbances of consciousness, syncope, sleep disorders, and unexplained delirium.

Publication metadata

Author(s): McKeith IG

Publication type: Article

Publication status: Published

Journal: Annals of the New York Academy of Science: Molecular Basis of Dementia

Year: 2000

Volume: 920

Pages: 1-8

ISSN (print): 0077-8923

ISSN (electronic): 1749-6632

Publisher: Wiley-Blackwell Publishing Ltd.

URL: http://dx.doi.org/10.1111/j.1749-6632.2000.tb06898.x

DOI: 10.1111/j.1749-6632.2000.tb06898.x

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