Objective: To review the clinical features, neuropathologic features, clinical course, differential diagnosis, evaluation, and management strategies of the primary non-Alzheimer degenerative and prion disorders that cause dementia.
Data sources: The PubMed MEDLINE search engine was used to query for all published articles written in English from January 1990 to August 2005 using the keywords non-Alzheimer, tau, tauopathy, synuclein, synucleinopathy, prion, cognitive impairment, and dementia syndrome. These and related terms were queried on the following additional search engines: On-Line Mendelian Inheritance in Man and GeneTests. Reputable organizations whose aims include promoting education and research in specific syndromes and disorders were queried using the search engine Google.
Study selection: The original articles on the disorders and syndromes, and subsequent articles and consensus papers that discussed in detail the clinical features, pathologic features, differential diagnosis, evaluation, management strategies, or some combination thereof, were selected for this review.
Data extraction: Data were extracted from articles that include generally accepted concepts and guidelines on the non-Alzheimer degenerative and prion disorders as viewed by the author.
Data synthesis: The following data were synthesized and emphasized: the cardinal clinical features, differential diagnosis, findings on ancillary studies most helpful in establishing accurate diagnoses, diagnostic criteria, and key principles of management.
Conclusions: This article provides an up-to-date overview of the primary non-Alzheimer disorders that cause cognitive impairment/dementia to aid the clinician in establishing diagnoses and deciding on appropriate management.