Neurocutaneous melanosis

Abstract

Neurocutaneous melanosis is a rare fatal neurological ailment characterised by cutaneous nevi with leptomeningeal melanosis.Though there is no effective treatment but able to diagnose and take care of symptoms and complications will help in the palliative care of these patients and would further help in counselling the relatives as per the prognosis of this disease. 12yr old boy rt.handed presented with left partial seizures in clusters followed with left sided weakness. He had episode of right partial status epilepticus 6 months back and 3 months back. On examination he was drowsy with power 0 in left UL & LL with increased salivation, neck stiffness. Skin examination showed giant melanocytic naevi on limbs, face, torso and back. MRI Brain showed parafalcine hypointense meninges on T2, hyperintense meninges on T1 images with nodular leptomeningeal enhancement with hydrocephalus. CSF study showed 1 lymphocyte, proteins 14mg/dl, sugar 56mg/dl. CSF cytology showed abnormal cells which were stained with May Grunwald Giemsa showing prominent nucleoli. CT chest and abdomen was normal.EEG showed generalised slowing with no epileptiform activity. His seizures were controlled with LVT, LAC, OXZ, CLZ. He was also started on diamox, mannitol for hydrocephalus. He had intermittent fever which was controlled with antibiotics. He had residual left hemiplegia and had to be fed through rhyles tube. He did continue to have intermittent seizures for which antiepileptic drugs were adjusted. Our subject had all the risk factors for NCM reported as presence of a GCMN, male sex, satellite nevi or multiple CMN, and head, neck, or posterior midline location.