Journal of Pediatric Psychology 6(3) pp. 265-274, 1981
© 1981 Society of Pediatric Psychology
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Adolescent Males with Noonan's Syndrome: Behavioral and Erotosexual Status1
Department of Psychiatry and Behavioral Sciences and Department of Pediatrics, Johns Hopkins University and Hospital
2All correspondence should be directed to John Money, Department of Psychiatry and Behavioral Sciences, The Johns Hopkins Hospital, Baltimore, Maryland 21205
Although Noonan's syndrome in 46, XY males is the clinical counterpart of Turner's syndrome in 45, X females, it has no cytogenetic anomaly by which to confirm its diagnosis. Consequently, confidence in establishing the diagnosis requires multiple pathognomonic signs, and it is rare. This report (the first of its kind) on seven cases, is thus purely phenomenological and descriptive. The seven patients, 13 to 26 years old, all bore manifest stigmata of the syndrome, including peculiarity of appearance and were from childhood onward subject to social stigmatization because of their appearance. Five were excessively short. The fullscale IQs ranged from 80 to 127. In three cases there was a specific praxic deficit. They were handicapped in establishing and maintaining an agecommensurate social status. School achievement and rate of progress were adversely affected in all but one case. There was no evidence of postpubertal erotosexual impairment, except as secondary to diminished social-bonding opportunity. Five of the patients manifested developmental behavioral disability; in four cases outside professional help was sought.