The Japanese journal of neuropsychology

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ArticleTitle	Neuropsychology of 22q11.2 deletion syndrome and Prader-Willi Syndrome
Language	J
AuthorList	Motoichiro Kato, Takaki Maeda
Affiliation	Department of Neuropsychiatry, Keio University School of Medicine
Publication	Japanese Journal of Neuropsychology: 20 (3), 150-156, 2004
Received
Accepted
Abstract	22q11.2 deletion syndrome is associated with mild intellectual disability, social withdrawal, and a high prevalence of neurospychiatric disorders including schizophrenia and mood disorder. AS for the neuropsychological characteristics of this contiguous gene syndrome, the most consistent finding is the discrepancy between Verbal and Performance IQ, indicating poor performances on no-verbal skills. Moreover, some researchers provided the evidences for a unique cognitive profile of 22q11.2 deletion syndrome, which was similar to that identified in children with non-verbal learning disabilities. On the other hand, the recent neurodevelopmental studies demonstrated that patients with Prader-Willi Syndrome showed a superior visuomotor processing function including surprising ability on jigsaw puzzle, and a better result on Performance IQ, although this syndrome is also associated with intellectual disability and psychiatric disorder such as obsessive compulsive disorder and bulimia. The neuropsychological comparative study is necessary to elucidate the neurodevelopmental mechanism of the uneven cognitive phenotypes in 22q11.2 deletion syndrome and Prader-Willi Syndrome.
Keywords	22q11.2 deletion syndrome, CATCH22, Prader-Willi Syndrome, contiguous gene syndrome, cognitive disorder