CEREBELLO-TRIGEMINAL-DERMAL DYSPLASIA (GOMEZ-LOPEZ-HERNANDEZ-SYNDROME) - DESCRIPTION OF 3 NEW CASES AND REVIEW

Cerebello-trigemino-dermal ''dysplasia'' is a rare neurocutaneous synd rome of craniosynostosis, ataxia, trigeminal anesthesia, scalp alopeci a, cerebellar anomaly, midface hypoplasia, corneal opacities, apparent ly low-set ears, mental retardation, and short stature. It seems to be a sporadic condition but little is known about its cause and pathogen esis in the few cases reported so far. We present three new unrelated patients and magnetic resonance images of the central nervous system, and review the four cases reported previously. We think that this is n ot such a rare condition, and that it is underdiagnosed. (C) 1997 Wile y-Liss, Inc.