Jacobs Syndrome (Triple X Syndrome; XXX Syndrome; Super Female Syndrome) 649

General: Caused by sex chromosomal anomaly with 44 autosomal and 3X sex chromosomes; due to nondisjunction; often associated with autosomal trisomies; majority of cases are asymptomatic.

Ocular: Hypertelorism; epicanthus; mongoloid, slanted lid fissure; strabismus.

Clinical: Microcephaly; oligophrenia, occasionally with secondary amenorrhea; abnormal dentition; high-arched palate; hypogenitalism; occasionally mental retardation; early menopause.

Jacobs PA, et al. Evidence for the existence of the human "superfemale." Lancet 1959; 1:423.

Kohn G, et al. Trisomy X in three children. J Pediatr 1968; 72:248.