A NEW NEUROCUTANEOUS SYNDROME POSSIBLY RELATED TO OTAS NEVUS

The case of a 42-year-old Sudanese man is reported who had developed h yperpigmentation of the left face and rapidly progressive right hemipa resis followed by myoclonus at the age of 30. Blue-grey hyperpigmentat ion was noted in the territory of the left ophthalmic, maxillary and m andibular trigeminal nerve branches, with alopecia of the right face a nd axilla. CT of the brain revealed left-sided paraventricular calcifi cations and MRI showed atrophy of the left cerebral hemisphere and mes encephalon, a calcified haemangioma in the left basal ganglia, an old infarct in the right central pons, and cerebellar atrophy. This case r esembles Ota's syndrome; however, atypical distribution of the facial nevus, the epidermal location of hyperpigmentation, and the combinatio n of vascular and degenerative cerebral malformations indicate that th is is a new hitherto unreported neurocutaneous vascular syndrome.