Clinical Features of Rapidly Progressive Alzheimer’s DiseaseSchmidt C. · Redyk K. · Meissner B. · Krack L. · von Ahsen N. · Roeber S. · Kretzschmar H. · Zerr I.
Departments of aNeurology and bClinical Chemistry, Hospital of the Georg August University Goettingen, Goettingen, and cDepartment of Neuropathology, Hospital of the Ludwig Maximilian University Munich, Munich, Germany
Objective: To characterize clinical features, CSF biomarkers and genetic polymorphisms of patients suffering from a rapidly progressing subtype of Alzheimer’s dementia (rpAD). Methods: Retrospective analyses of 32 neuropathologically confirmed cases differentially diagnosed as AD out of a group with rapidly progressive dementia. CSF biomarkers (14-3-3, tau, β-amyloid 1–42) and genetic markers (PRNP codon 129, apolipoprotein E, ApoE, polymorphism) were determined. Results: Median survival was 26 months, age at onset 73 years. Biomarkers: mean β-amyloid 1–42: 266 pg/ml, median tau: 491 pg/ml, 14-3-3 positive: 31%. Genetic polymorphisms showed a predominance of methionine homozygosity at PRNP codon 129 and a low frequency of ApoE4 (38%, no homozygous patients). Thirty-five symptoms were studied. Frequent symptoms were myoclonus (75%), disturbed gait (66%) and rigidity (50%). Discussion: rpAD is associated with a diversity of neurological signs even able to mimic Creutzfeldt-Jakob disease. Biomarkers and genetic profile differ from those seen in classical AD. The findings on biomarkers, symptomatology and genetics may aid the differential diagnostic process.
Dr. Christian Schmidt
Department of Neurology, Georg August University of Goettingen
Robert-Koch-Strasse 40, DE–37075 Goettingen (Germany)
Tel. +49 55 139 6636, Fax +49 55 139 7020
Accepted: January 19, 2010
Published online: May 5, 2010
Number of Print Pages : 8
Number of Figures : 3, Number of Tables : 1, Number of References : 48
Dementia and Geriatric Cognitive Disorders
Vol. 29, No. 4, Year 2010 (Cover Date: May 2010)
Journal Editor: Chan-Palay V. (New York, N.Y.)
ISSN: 1420-8008 (Print), eISSN: 1421-9824 (Online)
For additional information: http://www.karger.com/DEM