TY - JOUR
T1 - Long-term outcome after limited cortical resections in two cases of adult-onset Rasmussen encephalitis
AU - Villani, Flavio
AU - Didato, Giuseppe
AU - Deleo, Francesco
AU - Tringali, Giovanni
AU - Garbelli, Rita
AU - Granata, Tiziana
AU - Marras, Carlo Efisio
AU - Cordella, Roberto
AU - Spreafico, Roberto
PY - 2014
Y1 - 2014
N2 - Rasmussen encephalitis (RE) is a progressive inflammatory disorder characterized by brain hemiatrophy, unilateral focal deficits, and drug-refractory focal epilepsy. Epilepsia partialis continua (EPC) is a hallmark of the disease. Several immunomodulatory treatments may slow but not halt the disease progression. The treatment of choice still relies on surgical hemispheric disconnection, which is burdened by heavy neurologic morbidity. More limited cortical resections, although more tolerable, are usually considered to be, at best, only transiently effective in RE. Hemispheric disconnections may be not feasible when neurologic functions are preserved and the dominant hemisphere is affected. Adult patients with a milder RE course that preserves neurologic function for a long period are particularly at risk of developing severe deficits after surgery. In this study we present the histories of two patients with adult-onset RE who have undergone selective cortical resections to control EPC, avoiding, at the same time, the severe postsurgical deficits that may be induced by hemispheric disconnective surgery. The good result obtained on EPC has been stable over a prolonged period; however, this result was not paralleled by the stop of neurologic progression in one of the two cases. A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.
AB - Rasmussen encephalitis (RE) is a progressive inflammatory disorder characterized by brain hemiatrophy, unilateral focal deficits, and drug-refractory focal epilepsy. Epilepsia partialis continua (EPC) is a hallmark of the disease. Several immunomodulatory treatments may slow but not halt the disease progression. The treatment of choice still relies on surgical hemispheric disconnection, which is burdened by heavy neurologic morbidity. More limited cortical resections, although more tolerable, are usually considered to be, at best, only transiently effective in RE. Hemispheric disconnections may be not feasible when neurologic functions are preserved and the dominant hemisphere is affected. Adult patients with a milder RE course that preserves neurologic function for a long period are particularly at risk of developing severe deficits after surgery. In this study we present the histories of two patients with adult-onset RE who have undergone selective cortical resections to control EPC, avoiding, at the same time, the severe postsurgical deficits that may be induced by hemispheric disconnective surgery. The good result obtained on EPC has been stable over a prolonged period; however, this result was not paralleled by the stop of neurologic progression in one of the two cases. A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.
KW - Cortectomy
KW - Drug-refractory epilepsy
KW - Epilepsy surgery
KW - Inflammatory encephalopathy
KW - Rasmussen encephalitis
KW - Resective surgery
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U2 - 10.1111/epi.12596
DO - 10.1111/epi.12596
M3 - Article
C2 - 24702622
AN - SCOPUS:84901348495
VL - 55
JO - Epilepsia
JF - Epilepsia
SN - 0013-9580
IS - 5
ER -