Combined congenital abnormalities of the skin and central nervous system are well recognized-such conditions as Sturge-Weber syndrome, tuberous sclerosis, and neurofibromatosis. A very much rarer and less well-known member of this group is neurocutaneous melanosis. posterior fossa decompression was performed, and it was noted at operation that the meninges overlying the cerebellum were thickened and pigmented. The underlying cerebellar cortex also appeared to be pigmented. After operation the child renained well for eight weeks, but it was then noted that he was ataxic and though he was talking well he was emitting a shrill high-pitched cry at frequent intervals. The posterior fossa decompression was tense and bulging, and a Pudenz valve was therefore inserted into the right lateral ventricle draining into the right internal jugular vein. The child then remained fairly well, apart from some degree of ataxia, for a further period of four months, when he was readmitted with increasing restlessness, ataxia, and rigidity. The posterior fossa decompression was again tense and bulging, and at operation the Pudenz valve was found to be blocked by a protein coagulum, and it was replaced. Three days after the operation the child's conscious level began to decline:he developed left-sided Jacksonian epileptiform attacks and, despite repeated ventricular taps, died at 25 months.NECROPSY. There were no abnormal pathological findings outside of the skin and the central nervous system. In particular, no evidence of tumour was found in any other organ. The skin showed numerous large pigmented hairy naevi on the skin of the limbs, chest, trunk, back, and scalp. These varied in size, but ranged up to a maximum diameter of 6 cm.The dura appeared normal and showed no evidence of pigmentation, but the pia-arachnoid showed a diffusely. grey coloration most marked over the right cerebral hemisphere. In addition there were numerous scattered FiG.

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