TY - JOUR
T1 - Judgment of duration in individuals with ataxia-telangiectasia
AU - Mostofsky, Stewart H.
AU - Kunze, Jane C.
AU - Cutting, Laurie E.
AU - Lederman, Howard M.
AU - Denckla, Martha B.
N1 - Funding Information:
We acknowledge the support of the Pediatric Clinical Research Unit at the Johns Hopkins Medical Institution and the Ataxia Telangiectasia Children’s Project. This research was partially supported by the National Institutes of Health (National Institute of Neurologic Disease and Stroke) Grant K08 NS02039–02.
Copyright:
Copyright 2017 Elsevier B.V., All rights reserved.
PY - 2000
Y1 - 2000
N2 - Several clinical investigations with adults suggest that the cerebellum may be critical for judgment of explicit time intervals; however, little work has been done in populations with lesions of the cerebellum acquired during development. We evaluated 17 individuals with ataxia-telangiectasia (AT), an autosomal recessive disorder with on set in early childhood characterized by diffuse, almost selective, degeneration of the cerebellar cortex, and 21 normal controls, matched for age. Because patients with AT have motor impairment, verbal IQ (VIQ) was used to estimate intelligence; VIQ was significantly lower in the group with AT (p < .0001). Participants were tested using a test of judgment of duration that has been found to be impaired in adults with cerebellar lesions and a contrasting auditory control task (not impaired in adults with cerebellar lesions) involving judgment of pitch. After statistically controlling for VIQ, the 2 groups did not differ significantly on judgment of pitch, but those with AT performed significantly worse than controls on judgment of duration (p = .01). Children and adolescents with AT show deficits in judgment of duration but not of pitch, suggesting that the cerebellum may be critical for judgment of explicit time intervals at all ages.
AB - Several clinical investigations with adults suggest that the cerebellum may be critical for judgment of explicit time intervals; however, little work has been done in populations with lesions of the cerebellum acquired during development. We evaluated 17 individuals with ataxia-telangiectasia (AT), an autosomal recessive disorder with on set in early childhood characterized by diffuse, almost selective, degeneration of the cerebellar cortex, and 21 normal controls, matched for age. Because patients with AT have motor impairment, verbal IQ (VIQ) was used to estimate intelligence; VIQ was significantly lower in the group with AT (p < .0001). Participants were tested using a test of judgment of duration that has been found to be impaired in adults with cerebellar lesions and a contrasting auditory control task (not impaired in adults with cerebellar lesions) involving judgment of pitch. After statistically controlling for VIQ, the 2 groups did not differ significantly on judgment of pitch, but those with AT performed significantly worse than controls on judgment of duration (p = .01). Children and adolescents with AT show deficits in judgment of duration but not of pitch, suggesting that the cerebellum may be critical for judgment of explicit time intervals at all ages.
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U2 - 10.1207/S15326942DN1701_04
DO - 10.1207/S15326942DN1701_04
M3 - Article
C2 - 10916575
AN - SCOPUS:0033926402
SN - 8756-5641
VL - 17
SP - 63
EP - 74
JO - Developmental Neuropsychology
JF - Developmental Neuropsychology
IS - 1
ER -