Bulteau Christine, Grosmaitre Catherine, Save-Pédebos Jessica, Leunen Dorothée, Delalande Olivier, Dorfmüller Georg, Dulac Olivier, Jambaqué Isabelle
INSERM U1129 "Infantile Epilepsies and Brain Plasticity", Paris, France; Université Paris Descartes, Sorbonne Paris Cité, France; CEA, Gif sur Yvette, France; Rothschild Foundation Hospital, Pediatric Neurosurgery Department, Paris, France.
INSERM U1129 "Infantile Epilepsies and Brain Plasticity", Paris, France; Université Paris Descartes, Sorbonne Paris Cité, France; CEA, Gif sur Yvette, France; Rothschild Foundation Hospital, Pediatric Neurosurgery Department, Paris, France.
Epilepsy Behav. 2015 Dec;53:51-7. doi: 10.1016/j.yebeh.2015.07.044. Epub 2015 Oct 28.
Hemispherotomy (H) is the gold standard treatment to cure epilepsy in Rasmussen encephalitis (RE). Linguistic prognosis after surgery remains the main issue when the dominant hemisphere is involved. The topic of the present research is to specify the long-term linguistic profile of the right hemisphere after left dominant H for RE.
We followed 6 children 8.4 to 14.6 years of age who underwent left H for RE. Preoperatively, four children experienced aphasia, but for two, worsening occurred after surgery. Age at H ranged from 4.1 to 8.4 years. The mean duration of epilepsy was 1.2 years and 5.6 years for follow-up. Neuropsychological evaluation included longitudinal follow-up of intellectual efficiency measurement and a long-term outcome of language using various components of receptive and expressive oral speech with computerized tasks.
Preoperatively, verbal comprehension index (VCI) was dramatically decreased in 4/6 patients, and performance reasoning index (PRI) was low in 5/6 participants, demonstrating a global impact of RE itself. Postoperatively, all children recovered sufficiently to attend a regular VCI (above 70) in a mean of 5 years after H, and 5/6 recovered normal or adapted school. There was a dissociation in favor of VCI, while PRI decreased in 5/6 patients. We found a specific linguistic profile for these children recovering language in the right hemisphere: normal verbal comprehension, and weakness of grammatical judgment, word repetition, statement production, semantic verbal fluency and metaphonological abilities. Language recovery scores were statistically correlated with those of Working Memory Index.
This study emphasizes for the first time the ability of the right hemisphere to functionally reorganize language over a long period of time following left H for RE. Syntactic abilities and phonology remain low and support the hypothesis of an early left hemispheric specialization. Nevertheless, lexico-semantic processes recover in the right hemisphere that could reflect a pre-existing potential of both hemispheres. Our results support a decision to proceed to H in classical left RE disease until the late childhood even if there is no complete aphasia before surgery. These data should be taken in account in the overall postoperative follow-up and rehabilitation strategy.
大脑半球切除术（H）是治疗拉斯穆森脑炎（RE）所致癫痫的金标准。当优势半球受累时，术后的语言预后仍是主要问题。本研究的主题是明确左侧优势半球大脑半球切除术治疗RE后右侧半球的长期语言特征。
我们对6名年龄在8.4至14.6岁之间接受左侧大脑半球切除术治疗RE的儿童进行了随访。术前，4名儿童出现失语，但有2名儿童术后病情恶化。大脑半球切除术时的年龄在4.1至8.4岁之间。癫痫的平均病程为1.2年，随访时间为5.6年。神经心理学评估包括对智力效率测量的纵向随访以及使用接受性和表达性口语的各种组成部分并借助计算机任务对语言的长期结果评估。
术前，4/6的患者言语理解指数（VCI）显著下降，5/6的参与者表现推理指数（PRI）较低，这表明RE本身具有全面影响。术后，所有儿童在大脑半球切除术后平均5年恢复到足以达到正常的VCI（高于70），5/6的儿童恢复到正常或适应学校学习。存在有利于VCI的分离现象，而5/6的患者PRI下降。我们发现这些在右侧半球恢复语言功能的儿童具有特定的语言特征：言语理解正常，但语法判断、单词重复、语句生成、语义言语流畅性和元音韵能力较弱。语言恢复分数与工作记忆指数的分数在统计学上相关。
本研究首次强调了右侧半球在左侧大脑半球切除术治疗RE后长时间内进行语言功能重组的能力。句法能力和音韵能力仍然较低，支持早期左侧半球特化的假说。然而，右侧半球的词汇语义过程恢复，这可能反映了两个半球预先存在的潜力。我们的结果支持在经典的左侧RE疾病中，即使术前没有完全失语，直到儿童晚期也可进行大脑半球切除术的决定。这些数据应在术后的整体随访和康复策略中予以考虑。
