Emt. Yacubian et al., Rasmussen encephalitis associated with segmental vitiligo of the scalp: Clinicopathologic report, J CHILD NEU, 16(5), 2001, pp. 374-377
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.