[Sturge-Weber-Krabbe syndrome. A severe form in a monozygote female twin]. / Syndrome de Sturge-Weber-Krabbe. Forme grave chez une jumelle monozygote.

We report a severe case of Sturge-Weber syndrome in one of two monozygotic twins. This syndrome included a facial portwine stain over the trigeminal ophthalmic V1 area, and ipsilateral vascular anomalies of the eye and of the pia mater. CT scans and MRI were very informative. The cerebral regional blood flow, studied with SPECT, showed a paradoxically high rate.