Selective Spatial Processing Deficits in an At-Risk Subgroup of the Fragile X Premutation

Autor/inn/en	Hocking, Darren R.; Kogan, Cary S.; Cornish, Kim M.
Titel	Selective Spatial Processing Deficits in an At-Risk Subgroup of the Fragile X Premutation
Quelle	In: Brain and Cognition, 79 (2012) 1, S.39-44 (6 Seiten) PDF als Volltext Verfügbarkeit
Sprache	englisch
Dokumenttyp	gedruckt; online; Zeitschriftenaufsatz
ISSN	0278-2626
DOI	10.1016/j.bandc.2012.02.005
Schlagwörter	Short Term Memory; Genealogy; Males; Spatial Ability; Cognitive Processes; Neurological Organization; Tests; Age; Intelligence Quotient; Adults; Identification + Suchen Sie Ihr Suchwort? Kurzzeitgedächtnis; Ahnenforschung; Genealogie; Male; Männliches Geschlecht; Räumliches Vorstellungsvermögen; Cognitive process; Kognitiver Prozess; Examination; Prüfung; Examen; Alter; Lebensalter; Intelligenzquotient; Identifikation; Identifizierung
Abstract	Until a decade ago, it was assumed that males with the fragile X premutation were unaffected by any cognitive phenotype. Here we examined the extent to which CGG repeat toxicity extends to visuospatial functioning in male fragile X premutation carriers who are asymptomatic for a late-onset neurodegenerative disorder, fragile X-associated tremor/ataxia syndrome (FXTAS). Thirty-three premutation males aged 20-68 years [divided into two groups: 16 low-repeat carriers (CGG greater than 55-less than 100) and 17 high-repeat carriers (CGG greater than 100)] with a family history of fragile X syndrome and 62 non-affected adult males with normal "FMR1" alleles were recruited. Subjects underwent neuropsychological tests of visuospatial and visual working memory functioning and visuoperceptual processing. On measures of visuospatial processing, the high-repeat carriers performed significantly worse than the normal allele group when age and IQ were covaried out. With increasing age and only in carriers of a larger (greater than 100 repeats) premutation allele was there a greater decrement in visuospatial working memory functioning. Performance on spatial and perceptual judgement tasks failed to show similar specificity in males within the upper premutation range. We conclude that identification of selective visuospatial impairments in carriers of a larger premutation allele indicates greater CGG repeat toxicity in specific neural regions. Longitudinal follow-up studies will be needed to determine whether subtle decline in visuospatial functioning is associated with the later onset of motor symptoms of FXTAS. (Contains 3 tables and 1 figure.) (As Provided).
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Erfasst von	ERIC (Education Resources Information Center), Washington, DC
Update	2017/4/10