Document Detail


Mosaicism for trisomy 21 in a patient with young-onset dementia: a case report and brief literature review.
MedLine Citation:
PMID:  18332257     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To describe a case of young-onset Alzheimer disease (AD) due to mosaicism for trisomy 21. DESIGN: Case report of a single patient. SETTING: Tertiary referral dementia clinic. PATIENT: A 55-year-old man with a mild degree of developmental delay but no previous diagnosis of Down syndrome and only minimal physical manifestations of Down syndrome presented with gradually progressive cognitive impairment consistent with probable AD. RESULTS: Fluorescent in situ hybridization analysis of interphase chromosomes revealed trisomy 21 in 10% of peripheral lymphocytes. CONCLUSIONS: As mosaicism for trisomy 21 can present with no or minimal manifestations of Down syndrome, it may be underdiagnosed as a cause of early-onset AD. Occult mosaicism for trisomy 21 may explain in part the previously described association between family history of Down syndrome and risk of AD. Screening for mosaicism with fluorescent in situ hybridization is indicated in selected patients with mild developmental delay and those with AD of young onset.
Authors:
John M Ringman; P Nagesh Rao; Po H Lu; Stephen Cederbaum
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Archives of neurology     Volume:  65     ISSN:  0003-9942     ISO Abbreviation:  Arch. Neurol.     Publication Date:  2008 Mar 
Date Detail:
Created Date:  2008-03-11     Completed Date:  2008-05-08     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372436     Medline TA:  Arch Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  412-5     Citation Subset:  AIM; IM    
Affiliation:
Alzheimer's Disease Research Center, Department of Neurology, University of California, Los Angeles, 10911 Weyburn Ave, Ste 200, Los Angeles, CA 90095-7226, USA. jringman@mednet.ucla.edu
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MeSH Terms
Descriptor/Qualifier:
Alzheimer Disease / etiology*,  genetics
Down Syndrome / complications*,  genetics
Humans
Male
Middle Aged
Mosaicism*
Grant Support
ID/Acronym/Agency:
AG-16570/AG/NIA NIH HHS; K08 AG-22228/AG/NIA NIH HHS

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