下丘脑错构瘤(HH)相关癫痫的术后预后在癫痫发作自由(SF)方面已得到广泛研究,而认知和精神结局的报道和定义较少。这是对英语论文的系统回顾,分析一系列HH相关癫痫患者(≥5例,至少6个月的随访),于2002年1月至2022年12月发布。使用Engel量表/等效量表测量SF。我们研究了与不同手术技术相关的结果,和根据Delalande分类的HH类型。我们评估了手术后的神经心理和神经精神状况,以及术后并发症的发生。纳入了46篇报告1318名患者的文章,其中十个儿科系列。686/1222例患者报告SF(56,1%)。在24项研究的663名患者中报告了Delalande分类,其中70例为I型HH(10%),320为II型HH(48%),189例为III型HH(29%),84例为IV型HH(13%)。在663例患者中,有243例报告了SF的结局。在24种I型HH中的12种(50%)中报告了SF,132个II型HH中的80个(60,6%),59个III型HH中的32个(54,2%)和28个IV型HH中的12个(42,9%)。在显微手术后129/262(49,2%)达到SF,内窥镜手术后102/199(51,3%),伽玛刀手术后46/114(40,6%),245/353(69.4%)射频热凝后,MRI引导激光间质热治疗后107/152(70,4%)。饮食过多/体重增加是最多报道的手术并发症。其他是电解质改变,尿崩症,甲状腺功能减退,短暂性热疗/热疗。据报道,显微外科手术后记忆缺陷的百分比最高,而在显微手术或内窥镜手术后报告偏瘫和颅神经麻痹。30项研究报告了424/819(51,7%)患者的发育迟缓/智力障碍。248/346名患者获得了整体改善(72%),70/346是稳定的(20%),28/346恶化(8%)。22项研究报告了257/465名患者的精神疾病(55.3%)。78/98患者改善(80%),13/98保持稳定(13%),7/98变得更糟(7%)。大多数患者具有非结构化的认知/精神病学评估。根据现有数据,HH相关癫痫患者的手术治疗应个体化,目标不仅是达到最好的癫痫结果,还有最佳的认知和精神结果。
The post-surgical outcome for Hypothalamic Hamartoma (HH) related epilepsy in terms of seizure freedom (SF) has been extensively studied, while cognitive and psychiatric outcome has been less frequently reported and defined. This is a systematic review of English language papers, analyzing the post-surgical outcome in series of patients with HH-related epilepsy (≥5 patients, at least 6 months follow-up), published within January 2002-December 2022. SF was measured using Engel scale/equivalent scales. We looked at the outcome related to different surgical techniques, and HH types according to Delalande classification. We evaluated the neuropsychological and neuropsychiatric status after surgery, and the occurrence of post-surgical complications. Forty-six articles reporting 1318 patients were included, of which ten pediatric series. SF was reported in 686/1222 patients (56,1%). Delalande classification was reported in 663 patients from 24 studies, of which 70 were type I HH (10%), 320 were type II HH (48%), 189 were type III HH (29%) and 84 were type IV HH (13%). The outcome in term of SF was reported in 243 out of 663 patients. SF was reported in 12 of 24 type I HH (50%), 80 of 132 type II HH (60,6%), 32 of 59 type III HH (54,2%) and 12 of 28 type IV HH (42,9%). SF was reached in 129/262 (49,2%) after microsurgery, 102/199 (51,3%) after endoscopic surgery, 46/114 (40,6%) after gamma knife surgery, 245/353 (69,4%) after radiofrequency thermocoagulation, and 107/152 (70,4%) after MRI-guided laser interstitial thermal therapy. Hyperphagia/weight gain were the most reported surgical complications. Others were electrolyte alterations, diabetes insipidus, hypotiroidism, transient hyperthermia/poikilothermia. The highest percentage of memory deficits was reported after microsurgery, while hemiparesis and cranial nerves palsy were reported after microsurgery or endoscopic surgery. Thirty studies reported developmental delay/intellectual disability in 424/819 (51,7%) patients. 248/346 patients obtained a global improvement (72%), 70/346 were stable (20%), 28/346 got worse (8%). 22 studies reported psychiatric disorders in 257/465 patients (55,3%). 78/98 patients improved (80%), 13/98 remained stable (13%), 7/98 got worse (7%). Most of the patients had non-structured cognitive/psychiatric assessments. Based on the available data, the surgical management in patients with HH related epilepsy should be individualized, aiming to reach not only the best epilepsy result, but also the optimal cognitive and psychiatric outcome.