Abstracts

RATIONALE: Rasmussen's syndrome (RS) is considered a progressive unilateral encephalopathy in which pathological findings include lymphocytic cuffing and microglial nodules scattered mainly or, in most cases, exclusively distributed in one cerebral hemisphere. We report the cases of two children in which these findings and clinical characteristics of RS coexisted with Vogt-Koyanagi syndrome and a parasitic infection of the nervous system. METHODS: We report unusual presentations of RS in two patients. RESULTS: Two 3 and 5 year-old boys presented the classical clinical presentation of RS consisting of uncontrolled seizures configuring epilepsia partialis continua and progressive hemiparesis. These neurological signs were preceded in the first case by right ear dysacusia and focal areas of depigmentation on the right frontoparietal area of the scalp, right ocular involvement with perilimbal vitiligo, iris depigmentation and hypopigmented areas on retinal pigment epithelium. The second patient presented marked eosinophilia in the spinal fluid and blood, IgG antibodies to toxocara in plasma and spinal fluid as well as IgG antibodies to cysticercosis. Neuroimaging studies demonstrated hyperintense signal areas throughout the right hemisphere in both patients.Therapy with corticosteroids in both, associated to thiabendazole in the second, were ineffective. Both patients had hemispherectomies and the pathological studies, even with intensive search of the possible agent, were negative. The pathological findings were undistinguishable from the classical findings in RS. CONCLUSIONS: Pathological and clinical findings of RS may represent a mere immunoreactive response of the nervous system to different kinds of pathological processes. Why it remains restricted to one hemisphere even in systemic diseases continues an unsolved question.