Department of Medicine, Epilepsy Research Centre, Austin Health, University of Melbourne, Melbourne, Vic., Australia.
Department of Neurology, Austin Health, Heidelberg, Vic., Australia.
Epilepsia. 2020 Dec;61(12):2675-2684. doi: 10.1111/epi.16729. Epub 2020 Oct 23.
To investigate the etiology and longitudinal clinical, neuropsychological, psychosocial, and surgical outcome profile of patients with medication refractory epilepsy and temporal encephaloceles with a view to highlight diagnostic clues and management strategies.
The comprehensive epilepsy program databases at two surgical epilepsy centers from January 2000 to October 2018 were reviewed for this observational study, to identify patients with encephaloceles causing temporal lobe epilepsy (TLE) and treated with surgical resection. Their clinical, radiological, neuropsychological, psychiatric, and surgical data were obtained. Body mass index (BMI) data were also reviewed due to possible correlation between idiopathic intracranial hypertension and encephaloceles.
Thirteen patients (eight female) were identified; only three were recognized on initial magnetic resonance imaging (MRI) report. Temporal encephaloceles were identified on the left in eight patients, on the right in three patients, and bilaterally in two patients. One patient had a strong family history of encephaloceles. The median BMI for patients with seizure onset ≤20 years of age was 22.4, whereas for patients with onset >20 years median BMI was 32.6 (P = .06). Five patients underwent a focal lesionectomy, three patients had limited temporal lobectomy, and five patients had standard anterior temporal lobectomy. Median postoperative follow-up was 5.5 years. All but one patient were free of disabling seizures. Nine of ten neuropsychologically tested patients had no discernable cognitive decline postoperatively. Postoperative psychosocial adjustment features were present in four patients.
Genetic factors and a possible association with idiopathic intracranial hypertension (given female predominance and elevated BMI) may contribute to the causation of temporal lobe encephaloceles. It is notable that a targeted surgical approach in the management of patients with TLE associated with encephaloceles has an excellent long-term clinical and neuropsychological outcome. Subtle encephaloceles should be actively searched for in patients with drug-resistant TLE because they significantly change surgical strategy and prognostication.
探讨药物难治性癫痫伴颞叶脑膨出患者的病因学及纵向临床、神经心理学、社会心理和手术结局特征，以突出诊断线索和管理策略。
本研究为观察性研究，对 2000 年 1 月至 2018 年 10 月期间两个外科癫痫中心的综合癫痫项目数据库进行了回顾，以确定因颞叶癫痫（TLE）伴脑膨出而接受手术切除的患者。获取了他们的临床、放射学、神经心理学、精神科和手术数据。由于特发性颅内高压和脑膨出之间可能存在相关性，还回顾了体重指数（BMI）数据。
共确定了 13 例患者（8 例女性）；仅有 3 例在初始磁共振成像（MRI）报告中被识别。8 例患者的颞叶脑膨出位于左侧，3 例位于右侧，2 例位于双侧。1 例患者有强烈的脑膨出家族史。发病年龄≤20 岁的患者的中位数 BMI 为 22.4，而发病年龄>20 岁的患者中位数 BMI 为 32.6（P=.06）。5 例患者行局灶性病变切除术，3 例患者行局限性颞叶切除术，5 例患者行标准前颞叶切除术。中位术后随访时间为 5.5 年。除 1 例患者外，所有患者均无致痫发作。10 例接受神经心理学测试的患者中，有 9 例术后认知功能无明显下降。4 例患者术后出现社会心理调整特征。
遗传因素和特发性颅内高压（鉴于女性患病率高和 BMI 升高）之间可能存在关联，这可能导致颞叶脑膨出的发生。值得注意的是，在伴有脑膨出的 TLE 患者的治疗中，采用靶向手术方法具有极好的长期临床和神经心理学结局。对于药物难治性 TLE 患者，应积极寻找隐匿性脑膨出，因为它们会显著改变手术策略和预后。
