Epilepsy surgery in adultonset Rasmussen’s enceph

Authors: Sara Casciato Carlo Di Bonaventura Anna Teresa Giallonardo Jinane Fattouch Pier Paolo Quarato Addolorata Mascia Alfredo D’Aniello Andrea Romigi Vincenzo Esposito Giancarlo Di Gennaro

Publish Date: 2015/04/16

Volume: 38, Issue: 3, Pages: 463-471

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Abstract

Rasmussen’s encephalitis RE is a rare immunemediated condition characterized by drugresistant focal epilepsy progressive neurological and cognitive deficits associated to unilateral hemispheric atrophy The onset is typically reported in childhood although adult cases ARE have been described While surgical strategies in childhood RE are well defined little is known about usefulness of epilepsy surgery in ARE patients We describe clinical features surgical approach and outcome of five ARE patients who underwent epilepsy surgery and we review the literature with regard to surgical ARE cases We retrospectively studied five ARE patients aged 21–38 years mean age 228 years who were followed after surgery for a period ranging from to 1 to 6 years Demographic electroclinical and neuroimaging data were systematically reviewed Four out of five subjects underwent invasive EEG monitoring to define epileptogenic zone Epilepsy outcome was defined according to Engel’s classification Surgery consisted of frontal corticectomy in three patients temporal lobectomy in one combined temporal lobectomy plus insular and frontobasal corticectomy in the remaining case No permanent neurological deficits were observed after surgery At the last followup observation one patient was seizurefree two subjects experienced rare disabling seizures another had moderate seizure reduction and one had no clinical improvement Our experience although limited to few cases suggests that resective surgery in ARE may play a role in the context of multidisciplinary therapeutical approach of this severe condition Since the lack of specific data about surgical options this topic seems to deserve further investigations and more targeted studiesIn this case series the authors have focused on partial resections and on adult patients only Thus this is a very special article and the reader should be aware that it is not wholly representative on the possible options for treating Rasmussen’s encephalitis The authors are correct in pointing out that not much has been published on treatment of Rasmussen’s encephalitis in adults However the authors have missed a recent paper on 27 adult hemispherotomy cases which included 4 cases with Rasmussen’s encephalitis J Schramm et al Seizure outcome functional outcome and quality of life after hemispherectomy in adults Acta Neurochir 2012 1541603–1612By focusing on nonhemispherical surgery types only there is a certain danger that the uninitiated reader gets the impression that partial resections are the typical form of treatment for Rasmussen’s encephalitis It is quite clear that concern about postoperative deficits was the main reason to not opt for hemispherotomy or hemispherectomy in their patients May be the title of the paper would better have been “Nonhemispherotomy surgery in adult onset Rasmussen’s encephalitis…”To not choose hemispherotomy was the more remarkable since all cases were righthemisheric so the risk of losing speech was most likely zero I missed a few sentences discussing the treatment options and the rationale for not doing hemispheric surgery Most Rasmussen patients already have some degree of hemisided weakness so the main risk for these patients would have been a certain increase in the spastic hemiparesis and of course new hemianopia So the big issue with adult Rasmussen’s is how much new deficit appears acceptable and weighing that disadvantage against the chances of complete seizure freedom after hemispherotomy The outcome in this small series shows low seizure freedom rate and is thus not satisfactory The question remains open in how far the pros and cons of hemispherotomy associated with hemianopia and increase in hemispasticity versus a good chance for complete seizure freedom was actually discussed with each patient and his family One can not clearly see that in the manuscriptIt should also be noted that in the 31 collected cases 12 only had a biopsy so there are only 19 cases in which some kind of therapeutic intervention was tried which means that the title of the table “General characteristics of surgically treated adult RE patients in the literature” is not quite correct

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