A Review of the Non-Alzheimer Dementias

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.