COGNITIVE-FUNCTIONING, NEUROLOGIC STATUS AND BRAIN IMAGING IN CLASSICAL GALACTOSEMIA

被引:63
作者
KAUFMAN, FR
MCBRIDECHANG, C
MANIS, FR
WOLFF, JA
NELSON, MD
机构
[1] CHILDRENS HOSP LOS ANGELES,DEPT RADIOL,LOS ANGELES,CA 90027
[2] UNIV SO CALIF,DEPT PSYCHOL,LOS ANGELES,CA 90089
[3] UNIV WISCONSIN,WAISMAN CTR,MADISON,WI 53705
关键词
NEUROPSYCHOLOGIC; NEUROLOGIC; BRAIN MAGNETIC RESONANCE IMAGING;
D O I
10.1007/BF02143794
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
A historical group of 45 children (4-18 years) and adults (18-39 years) with classical galactosemia had deficits of cognitive function that were variable and not related to the age at diagnosis or to severity of illness at presentation. There was a trend for patients to score highest on visual processing tasks. The standardized tests of speech and memory skills fell within the same range as the Broad Cognitive Ability score, indicating that the speech and language deficits may be part of a more global set of cognitive impairments. Scores on the Beery Visual Motor Integration and Block Design Tests fell in approximately the same range as other cognitive abilities. In addition, there was a high incidence of abnormality detected on MRI and 12 patients had neurologic symptoms that included ataxia, tremor and dysmetria. These abnormalities did not correlate with the age at diagnosis, severity of illness at presentation or scores on cognitive testing. The pathophysiology of neurologic and neuropsychologic impairments remains unknown. Since these appear to be unrelated to the duration of galactose exposure, other factors impacting on outcome need to be understood so that strategies can be developed to improve what appears to be a global impairment of cognitive function.
引用
收藏
页码:S2 / S5
页数:4
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