<prompt> The most likely diagnosis is a case of intractable convulsions mental defect and facial nevus is?Sturge Weber syndromeTuberous sclerosisVon-Hippen -Lindau diseaseVon- Reckling-Hausens disease  <response>: Ans. is a i.e., Sturge-weber syndrome o Sturge weber syndrome and Von-Reckling-Hausen disease NF-1 have been explained earlier about other options. Tuberous sclerosis Bournevilles disease o Tuberous sclerosis is characterized by : ? Cutaneous lesions --- Adenoma sebaceum, ash-leaf shaped hypopigmented macules, shagreen patches, depigmented nevi. Seizures Mental retardation o Other features --- Calcified subependymal nodules, Subependymal giant cell astrocytoma, ependymoma, Rhabdomyomas of the hea, and angiomyomas of the kidney, liver, adrenals and pancreas. Von Hippel - Lindau syndrome o This syndrome consists of Retinal, cerebellar and spinal hemangioblastoma. o Renal cell carcinoma, Pheochromocytoma, benign cyst of the kidney, liver, pancrease and epididymis may also occur. o Polycythemia may occur due to secretion of erythropoietin from hemangioblastoma. 