An Unique Case of Ito Syndrome Presenting with Juvenile Myoclonic Epilepsy and Normal Intelligence

Hypomelanosis of Ito (HI) appears to be the third most common neurocutaneous disease, second only to neurofibromatosis and tuberous sclerosis. Approximately three fourths of the patients with typical skin lesions have systemic manifestations. Hypomelanosis of Ito has been found to be associated with few cases of intellectual disability. HI is not very commonly associated with Myoclonic Epilepsy. In this article, we describe a case of Hypomelanosis of Ito with normal intelligence. The case presented to the treating institution with Myoclonic epilepsy which made the case even more a rarer entity. The very fact that the case was having a fairly normal intelligence made the case all the more interesting.