Ito hypomelanosis is a rare neurocutaneous condition. We report on four observations in infants aged between 8 and 20 months. They all presented with epilepsy, psychomotor delay, and diffuse hypomelanosis. The electroencephalograms showed diffuse irritative abnormalities. Brain imaging was normal in two infants and showed hemispheric atrophy in another case. Despite antiepileptic treatment and physical therapy, no significant progression was noted and all children continued to have drug-resistant epilepsy and psychomotor delay.
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