*Department of Neurosurgery, ‡Department of Epileptology, ¶Department of Pathology/Neuropathology, §Department of Radiology, University of Bonn Medical Centre, Bonn, Germany; ‖Department of Neurosurgery, Unfallkrankenhaus Berlin, Berlin, Germany; **Department of Neuroradiology, University of Freiburg Medical Center, Freiburg, Germany.
Neurosurgery. 2014 Feb;74(2):147-53; discussion 153. doi: 10.1227/NEU.0000000000000210.
Multiple cerebral cavernous malformations (mCCMs) are known as potentially epileptogenic lesions. Treatment might be multimodal. Management of patients with mCCMs and epilepsy is challenging.
To evaluate (1) algorhythmic therapeutic sequences in patients with epilepsy associated to mCCMs, (2) whether there are predictive parameters to anticipate the development of drug-resistant epilepsy, and (3) seizure after epilepsy surgery compared to conservatively-treated drug-resistant patients.
All inpatients and outpatients with epilepsy associated to mCCMs from 1990 to 2010 and follow-up >12 months available were retrospectively analyzed.
Twenty-three patients matched inclusion criteria. Epilepsy became drug-resistant in 18/23 (78%) patients. No predictors were found for development of drug-resistant epilepsy. Median follow-up for both groups was 7.8 years. Nine patients did not qualify for surgical therapy and were treated conservatively. One patient of this cohort (11%) was seizure-free (International League Against Epilepsy [ILAE] class 1). Surgical treatment was performed in 9 patients; 7/9 (78%) of these patients were seizure-free (ILAE class 1) after epilepsy surgery for at least 12 months compared with 1/9 patients in the non-operated group. In 7/9 cases (78%) the largest CCM was resected. In 8/9 (89%) not all CCMs were resected.
After initial diagnosis of epilepsy associated to mCCMs, a primary conservative approach is reasonable. Surgical treatment can be successful in a large fraction of cases with drug-resistant epilepsy where an epileptogenic lesion is identified. Cases where surgery is not undertaken are likely to remain intractable.
多发性脑海绵状血管畸形（mCCMs）被认为是潜在的致痫病变。治疗可能是多模式的。mCCMs 伴癫痫患者的管理具有挑战性。
评估（1）mCCMs 伴癫痫患者的算法治疗顺序，（2）是否存在预测参数来预测耐药性癫痫的发生，以及（3）与保守治疗耐药性患者相比癫痫手术后的发作情况。
回顾性分析了 1990 年至 2010 年间所有符合 mCCMs 伴癫痫且随访时间>12 个月的住院和门诊患者。
23 例患者符合纳入标准。23 例患者中有 18 例（78%）癫痫耐药。未发现耐药性癫痫发生的预测因素。两组的中位随访时间均为 7.8 年。9 例患者不符合手术治疗条件，采用保守治疗。该队列中有 1 例患者（11%）无发作（国际抗癫痫联盟[ILAE]1 级）。9 例患者接受了手术治疗；9 例患者中有 7 例（78%）在癫痫手术后至少 12 个月内无发作（ILAE 1 级），而未手术组中只有 1 例患者无发作。在 7/9 例（78%）中，最大的 CCM 被切除。在 8/9 例（89%）中，并非所有 CCM 都被切除。
在最初诊断为 mCCMs 伴癫痫后，首先采用保守方法是合理的。对于发现致痫病变的耐药性癫痫患者，手术治疗可以取得很大的成功。未进行手术的病例可能仍然难以治疗。
