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Rapidly progressive Alzheimer's disease: a multicenter update.
Christian Schmidt1, Stephane Haïk, Katsuya Satoh
1National Reference Center for TSE Surveillance, Department of Neurology, Georg-August-University, Goettingen, Germany. cschmid2@gwdg.de
Journal of Alzheimer'S Disease : JAD
|March 31, 2012
Summary
Rapidly progressive Alzheimer's disease (rpAD) cases were characterized, revealing distinct features. This subtype, often misdiagnosed as prion disease, shows specific cerebrospinal fluid biomarker profiles and unique genetic characteristics.
Area of Science:
- Neurology
- Neuroscience
- Pathology
Background:
- Alzheimer's disease (AD) typically presents with gradual cognitive decline.
- A rapidly progressive subtype (rpAD) exists, often initially misdiagnosed as prion disease due to its swift clinical course.
- Characterization of rpAD is crucial for understanding disease heterogeneity and improving diagnosis.
Purpose of the Study:
- To define the clinical, genetic, biomarker, and neuropathological features of rapidly progressive Alzheimer's disease (rpAD).
- To investigate whether rpAD represents a distinct subtype of Alzheimer's disease.
Main Methods:
- Retrospective analysis of neuropathologically confirmed rpAD cases from multiple centers.
- Examination of genetic properties (APOE, PRNP codon 129), cerebrospinal fluid (CSF) biomarkers, and clinical data.
- Comparison of rpAD features with classic AD and prion disease phenotypes.
Main Results:
- Eighty-nine rpAD patients were analyzed, with a median survival of 10 months.
- APOE and PRNP codon 129 genotype distributions were similar to healthy controls; APOE ε4 homozygosity was absent.
- CSF biomarkers were abnormal, consistent with AD, but 14-3-3 proteins were detected in 42% of cases.
Conclusions:
- Accumulating evidence supports the existence of a distinct rapidly progressive Alzheimer's disease (rpAD) subtype.
- The unique APOE profile in rpAD suggests it may represent a separate form of Alzheimer's disease.
- Further research is needed to fully elucidate the mechanisms and diagnostic criteria for rpAD.
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