Rasmussen encephalitis associated with segmental vitiligo of the scalp: Clinicopathologic report

A 3-year-old boy with segmental vitiligo, poliosis, and alopecia over the r ight side of the scalp developed epilepsia partialis continua involving the left side of the body and progressive atrophy of the right cerebral hemisp here. There was a right ear dysacusia and a perilimbal vitiligo associated with an area of iris depigmentation in the right eye. Pleocytosis and hyper gammaglobulin were detected in cerebrospinal fluid. Because medical treatme nt that included phenobarbital, phenytoin, carbamazepine, oxcarbazepine, be nzodiazepines, corticosteroids, gamma -globulin, and a ketogenic diet was i neffective, he underwent a right hemispherectomy. Neuropathologic examinati on. showed a widespread scattered inflammatory process with numerous microg lial nodules and perivascular lymphocytic cuffing associated with degenerat ive changes with severe neuronal loss, loosening of the neuropil, and micro cystic changes with tissue collapse. The coexistence of vitiligo and possib ly Vogt-Koyanagi-Harada syndrome in this child reinforces the autoimmune th eory as the pathogenesis of Rasmussen syndrome.