| Psychiatric phenotype of the fragile X-associated tremor/ataxia syndrome (FXTAS) in males: newly described fronto-subcortical dementia. | |
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MedLine Citation:
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PMID: 16426093 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: The authors describe and quantify the neuropsychiatric symptoms present in a cohort of males with the fragile X mental retardation 1 (FMR1) premutation allele who have developed fragile X-associated tremor/ataxia syndrome (FXTAS). METHOD: Fourteen male carriers of the FMR1 premutation who had clinical manifestations of the FXTAS syndrome and 14 age- and education-matched controls were assessed with the Neuropsychiatric Inventory (NPI), formal cognitive testing, and genetic analysis. RESULTS: Males with FXTAS had significantly higher total NPI scores (p < .004) and significantly higher scores on the agitation/aggression (p < .004), depression (p < .004), apathy (p < .004), disinhibition (p < .004), and irritability (p < .004) scales, compared with controls. Cognitive performances on the Mini-Mental State Examination did not correlate with severity of symptoms on the NPI. CONCLUSIONS: The neuropsychiatric manifestations of FXTAS, based on this preliminary report, appear to cluster as a fronto-subcortical dementia. Clinicians encountering patients with clinical dementia with motor symptoms suggesting FXTAS should consider genetic testing to determine whether the patient's dementia syndrome is secondary to a fragile X premutation carrier status. |
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Authors:
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Susan Bacalman; Faraz Farzin; James A Bourgeois; Jennifer Cogswell; Beth L Goodlin-Jones; Louise W Gane; Jim Grigsby; Maureen A Leehey; Flora Tassone; Randi J Hagerman |
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Publication Detail:
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Type: Comparative Study; Journal Article; Research Support, N.I.H., Extramural |
Journal Detail:
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Title: The Journal of clinical psychiatry Volume: 67 ISSN: 0160-6689 ISO Abbreviation: J Clin Psychiatry Publication Date: 2006 Jan |
Date Detail:
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Created Date: 2006-01-23 Completed Date: 2006-03-28 Revised Date: 2009-11-19 |
Medline Journal Info:
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Nlm Unique ID: 7801243 Medline TA: J Clin Psychiatry Country: United States |
Other Details:
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Languages: eng Pagination: 87-94 Citation Subset: IM |
Affiliation:
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M.I.N.D. Institute, and Departments of Psychiatry and Behavioral Sciences, UCDavis Medical Center, University of California-Davis, 2230 Stockton Boulevard, Sacramento, CA 95817, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Aged Ataxia / diagnosis, genetics* Dementia / classification, diagnosis, genetics* Fragile X Mental Retardation Protein / genetics* Fragile X Syndrome / diagnosis*, genetics* Genetic Predisposition to Disease / genetics Genetic Testing Heterozygote Heterozygote Detection Humans Male Middle Aged Personality Inventory Phenotype* Psychiatric Status Rating Scales Severity of Illness Index Sex Factors Syndrome Tremor / diagnosis, genetics* Wechsler Scales |
| Grant Support | |
ID/Acronym/Agency:
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HD 02274/HD/NICHD NIH HHS; HD 36071/HD/NICHD NIH HHS; NS 044299/NS/NINDS NIH HHS; NS 45352/NS/NINDS NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/FMR1 protein, human; 139135-51-6/Fragile X Mental Retardation Protein |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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