| Neurocognitive testing in late-onset Tay-Sachs disease: a pilot study. | |
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MedLine Citation:
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PMID: 18618288 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVES: To test neurocognitive function in patients with late-onset Tay-Sachs disease (LOTS) using a computerized system to assess whether cognition is a clinically relevant outcome measure of possible therapeutic intervention in LOTS. METHODS: Ten adults with Tay-Sachs disease were administered at least one battery of the Mindstreams Neurotrax system for evaluation of cognitive function. Six sub-scores and a Global Cognitive Score (GCS) were tabulated. A disease specific severity score was also devised with six domains. RESULTS: Despite identical genotypes, all patients but the two oldest had > or = 3/6 sub-scores one standard deviation below normal mean (100); verbal and executive functions were most affected. The severity score measured other functions. CONCLUSIONS: Because of provocative findings on re-testing in patients exposed to miglustat, and despite the very small cohort, cognitive function may be an appropriate and clinically relevant outcome measure for future therapeutic interventions in LOTS. |
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Authors:
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D Elstein; G M Doniger; E Simon; I Korn-Lubetzki; R Navon; A Zimran |
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Publication Detail:
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Type: Evaluation Studies; Journal Article Date: 2008-05-30 |
Journal Detail:
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Title: Journal of inherited metabolic disease Volume: 31 ISSN: 1573-2665 ISO Abbreviation: J. Inherit. Metab. Dis. Publication Date: 2008 Aug |
Date Detail:
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Created Date: 2008-08-15 Completed Date: 2008-11-12 Revised Date: 2009-04-16 |
Medline Journal Info:
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Nlm Unique ID: 7910918 Medline TA: J Inherit Metab Dis Country: Netherlands |
Other Details:
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Languages: eng Pagination: 518-23 Citation Subset: IM |
Affiliation:
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Gaucher Clinic, Shaare Zedek Medical Center, P.O. Box 3235, Jerusalem, 91031, Israel. elstein@szmc.org.il |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Age of Onset Aged Algorithms Cognition Disorders / diagnosis*, etiology* Diagnosis, Computer-Assisted / methods Diagnostic Techniques, Neurological* Female Humans Male Middle Aged Pilot Projects Research Design Severity of Illness Index Tay-Sachs Disease / complications* |
| Comments/Corrections | |
Comment In:
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J Inherit Metab Dis. 2009 Apr;32(2):310-1
[PMID:
19240988
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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