Atypical presentation in Rasmussen encephalitis: delayed late-onset periodic epileptic spasms.

A five-and-a-half-year-old girl started experiencing progressive left hemiparesis at age two and a half years. At age five years and four months she started presenting clusters of asymmetric periodic epileptic spasms with no hypsarrhythmia. The ictal EEG showed periodic, constant and stereotyped complexes. Serial brain imaging revealed progressive atrophy of the right hemisphere with increased T2 signal on MRI. She underwent a right hemispherotomy, and histological examination showed signs of inflammation and features of focal cortical dysplasia (FCD). She has been seizure-free for 16 months. This case is unique in the following aspects: the presence of typical Rasmussen encephalitis features of progressive unilateral brain involvement without seizures, a delay of almost three years prior to seizure onset; an atypical seizure type presentation with periodic epileptic spasms and the presence of FCD associated with inflammatory changes. [Published with video sequences].

Fibrohistiocitoma benigno de osso temporal: relato de caso / Benign fibrous histiocytoma of the temporal bone: case report

Os autores relatam o caso de uma paciente com quadro de dor e edema em regiao retroauricular persistente após quadro de mastoidite e encefalite. mostrando à tomografia computadorizada sinais de irregularidade no contorno ósseo da mastóide. Submetida a timpanomastoidectomia, foi evidenciado tumor que acometia a cortical mastóidea e os tecidos próximos, cujo diagnóstico anatomopatológico foi de fibrohistiocitoma benigno. Este tumor raramente acomete o tecido ósseo quando ocorre, se localiza em ossos longos. O tratamento consiste em curetagem ou exérese cirúrgica de todo o tumor, havendo recorrência em torno de 11 por cento dos casos