黑皮质素受体基因多态性与婴儿痉挛症及促肾上腺皮质激素反应性的相关性研究

目的探讨黑皮质素受体（MC2R～MC4R）基因变异及单核苷酸多态性与婴儿痉挛症发病及促肾上腺皮质激素治疗反应性的关系,并对研究中发现的最具意义的单核苷酸多态性进行深人的功能学研究。方法采用DNA直接测序法检测MC2R～MC4R基因编码区及启动子区基因突变及多态性;SHEsis和HaplOView3．32程序构建单体型,分析不同多态性基因型、等位基因及所构建单体型在婴儿痉挛症患儿和正常对照受试儿童中的分布频率,并观察其与促肾上腺皮质激素治疗反应的相关性。结果测序显示,MC2R基因启动子区共存在4个单核苷酸多态性位点,其中-2T〉C为新发现位点,3个单核苷酸多态性位点（rsl893220、rs2186944和．2T〉C）组间分布频率差异具有统计学意义（P=0．04,0．02,0．01）。常见单体型TCCT对婴儿痉挛症的发生具有保护作用（P=O．00）,且TCCT携带者比非携带者对促肾上腺皮质激素治疗更为敏感（P=0．00）。体外功能学研究证实,MC2R基因TCCT型启动子转录效率是TCCC的4倍（P=0．00）,经促肾上腺皮质激素刺激后含TCCT单体型启动子MC2RmRNA表达水平上调5倍（P：0．00）,而含TCCC单体型启动子仅上调1．50倍（P〉0．05）。MC4R基因启动子区单核苷酸多态性位点rsll872992与婴儿痉挛症的发生有关,婴儿痉挛症组患儿TC型频率低于正常对照组（P=0．00）：r型等位基因携带者对促肾上腺皮质激素治疗效果优于非丁型等位基因携带者（P=0．01）。但MC3R单核苷酸多态性组间分布频率差异无统计学意义（P〉0．05）。结论黑皮质素受体单核苷酸多态性与婴儿痉挛症的发生有一定关系,并可影响促肾上腺皮质激素治疗的反应性。这一发现有助于理解婴儿痉挛症的发病机制,并为临床早期预测促肾上腺皮质激素治疗反应提供一定线索。     

婴儿痉挛症临床下发作时血清NSE水平研究

目的通过对婴儿痉挛症(infantile spasms,IS)患儿24h视频脑电图监测,并定量测量血清特异性烯醇化酶(neuron-specific enolase,NSE)浓度,来判断临床下发作时IS是否存在脑损伤。方法对28例IS患儿进行治疗,其中痉挛症状消失1个月的患者,完善第2次24h视频脑电图并采集血清测NSE浓度,按照其脑电图异常与否分成2组,分别与正常对照组比较。结果24h视频脑电图正常的IS患儿的血清NSE(9.88±1.84)μg/L与正常儿童血清NSE(9.13±1.23)μg/L比较无显著差异,P=0.28;24h视频脑电图异常的患儿的血清NSE(13.73±1.93)μg/L与正常儿童血清NSE有显著差异,P0.01。结论婴儿痉挛症临床下发作时,血清中NSE升高,表示婴儿痉挛症临床下发作时可能存在脑损伤。     

婴儿痉挛症影像学特征及对治疗效果影响的回顾性研究

目的回顾性分析209例婴儿痉挛症患者颅脑影像学特征,并观察脑结构性异常是否对治疗效果产生影响。方法选择2014-01—2019-10于郑州大学第三附属医院首诊的209例婴儿痉挛症患者为研究对象,分析其影像学检查特征,将影像学检查提示存在结构性异常且认为与婴儿痉挛症的发生、发展相关的患者纳入观察组(102例),无影像学异常的患者为对照组(107例),对比2组患者治疗后短期控制率及脑电图(EEG)改善率,短期内控制后复发率及复发时间。采用Logistic回归模型分析影像学异常对短期控制率及脑电图改善率的影响。统计长期控制发作的婴儿痉挛症患者抗癫痫药物应用情况。结果观察组影像学特征主要包括先天性脑发育畸形(9.8%)及脑软化(90.2%),顶枕叶脑软化较多见(49.0%),其中可追溯到有明确围生期低血糖占24.5%。观察组与对照组药物治疗的短期控制率(χ²=0.562,P=0.453)及复发率差异无统计学意义(χ²=1.834,P=0.176),脑电图改善率差异有统计学意义(χ²=3.957,P=0.047)。Logistic回归分析提示观察组结构性异常为脑电图未改善的高危因素[P=0.048,OR(95%CI)=0.566(0.322~0.994)]。2组控制痉挛发作时间>24周的患者维持性应用的抗癫痫药物主要有丙戊酸钠、托吡酯、左乙拉西坦。结论脑软化及先天性脑发育畸形为IS的主要影像学特征,顶枕叶脑软化是导致IS的高危因素;存在脑结构性异常可影响IS患者脑电图的改善率。长期控制发作的患者口服抗癫痫药物以丙戊酸钠、托吡酯、左乙拉西坦为主。     

狭颅症合并婴儿痉挛症一例治疗及预后

目的探讨狭颅症合并婴儿痉挛症的临床表现、诊断、治疗及预后情况。方法对山东大学齐鲁儿童医院神经内科2017年12月收治的1例狭颅症合并婴儿痉挛症患儿的临床表现、诊断、治疗(给予促肾上腺皮质激素,疗程14天,颅盖重建术式,随后给予托吡酯治疗)以及预后(随访2年)情况进行回顾,并结合文献进行分析。结果先证者通过临床、影像学检查、视频脑电图确诊为婴儿痉挛症和狭颅症,通过激素冲击治疗和外科治疗阻止了癫痫发作并纠正了狭颅症,取得了较好的临床预后。结论此病例为国内第一例报道狭颅症合并婴儿痉挛症,且与国外的治疗方法进行比较,总结出较好的治疗手段和手术时机,对指导该类疾病的临床诊断和治疗具有重要意义。     

强的松治疗婴儿痉挛症的对照性研究

婴儿痉挛症是一种婴儿及幼儿期特定年龄的抽搐性疾病.患儿常有不同程度的智力障碍和高峰失律脑电图.1958年以来ACTH及肾上腺皮质激素的临床应     

雷帕霉素治疗结节性硬化症合并婴儿痉挛症一例报告并文献复习

目的 探讨雷帕霉素治疗结节性硬化症合并婴儿痉挛症的临床疗效. 方法 对解放军总医院儿童医学中心2011年6月收治的1例结节性硬化症合并婴儿痉挛症患儿应用雷帕霉素治疗前后的临床表现、辅助检查方法、治疗结果等进行回顾性分析. 结果 患儿有明显的皮肤损害,主要表现为色素脱失斑,同时合并婴儿痉挛症,头颅MRI及CT均有异常改变.患儿应用雷帕霉素治疗3个月后可见色素脱失减轻,痉挛发作较前明显减少,头颅MRI显示室管膜下结节较前减小,脑电图较前改善,智能发育进步,且治疗过程中未见明显不良反应. 结论 应用雷帕霉素治疗结节性硬化症合并婴儿痉挛症有显著疗效,安全性好.     

癫癇发作控制后的脑电图研究报告

目的：探讨癫癇临床发作症状控制后，常规脑电图（REEG）和动态脑电图（AEEG）随访意义及脑电图（EEG）的异常情况。方法：采用SOLAR ROVER-8型AEEG仪，对2000至2005年随访的癫癇大发作临床发作控制后的86例患者，进行REEG和AEEG的监测。结果：REEG正常69例（80．％），异常17例（19．8％）。癇样波1例（5．9％），非特异性异常16例（94．1％）。AEEG正常36例（41．9％），异常50例（58．1％）。癇样波11例（22．0％），非特异性异常39例（78．0％）。两组阳性率差异有显著统计学意义，x^2=26．63，P〈0．001。结论：AEEG用于研究癫癇控制后的脑电监测均优于REEG。控制后的脑电图异常以非特异：性高幅阵发性0、6波为主。控制的年限越长脑电图异常率和癇样波发放率愈低。有和于抗癫癇药物疗效的观察和指导癫癇临床用药。     

婴儿痉挛症的外科治疗

婴儿痉挛症又名West综合征,1841年West首先描述其独特的痉挛性发作,1952年Gibbs发现高幅失律（hypsarrhythmia）脑电图为其特点,并命名为West综合征。婴儿痉挛症临床药物治疗效果差,是儿童期最常见的灾难性癫痫,约有1／4的患者转化为另一灾难性癫痫．Lennox—Gastaut综合征。     

合成促皮质素及维生素B6联合治疗婴幼儿癫痫的疗效观察

目的：探讨合成促皮质素（Ｃｏｒｔｒｏｓｙｎ，长效确杜先）及维生素Ｂ６（吡哆醇）联合治疗婴幼儿癫痫的疗效。方法：１４例年龄为３个月至４岁的患儿（婴儿痉挛症６例，其它顽固性癫痫８例）给予维生素Ｂ６ ５０～１００ｍｇ／ｄ肌肉注射１０天后，再以逐渐延长用药间隔的方式肌肉注射Ｃｏｒｔｒｏｓｙｎ０．０１５～０．０２５ｍｇ／ｋｇ．ｄ，总疗程约２个月，观察用药后的癫痫发作情况、脑电图（ＥＥＧ）改变、血像、肝肾功能及血电解质变化。结果：治疗后１１例患儿癫痫发作停止，２例顽固性癫痫患儿发作减少，分别由周１５次及２０次减少至每周３次及８次，１例顽固性癫痫患儿因不良反应明显终止治疗。ＥＥＧ及一般临床情况有改善。治疗过程中部分患儿有低血钾、低血钙、浮肿等，但血液肝肾功能检查未见异常变化。结论：Ｃｏｒｔｒｏｓｙｎ及维生素Ｂ６联合治疗婴儿痉     

合成促皮质素及维生素B6联合治疗婴幼儿癫痫

目的　观察合成促皮质素 (Cortrosyn,长效确杜先 )及维生素 B6 (吡哆醇 )联合治疗婴幼儿癫痫的疗效。方法　对 2 0例年龄为 3个月至 4岁的患儿 (婴儿痉挛症 9例 ,顽固性癫痫 11例 )给予维生素 B6 5 0～10 0 m g/ d肌肉注射 10天后 ,再以逐渐延长用药间隔的方式肌肉注射 Cortrosyn 0 .0 15～ 0 .0 2 5 mg/ kg· d,总疗程约 2个月 ,观察用药前后的癫痫发作情况、脑电图 (EEG)改变、血象、肝肾功能及血电解质变化。结果　治疗后 14例患儿癫痫发作停止 ,5例顽固性癫痫患儿发作减少 ,分别由每周 15～ 40次减少至每周 3～ 14次 ,1例顽固性癫痫患儿因不良反应明显而终止治疗。 EEG及一般临床情况有改善。治疗过程中部分患儿有低血钾、低血钙、浮肿等 ,但血液、肝肾功能检查未见异常变化。结论　 Cortrosyn及维生素 B6 联合治疗婴儿痉挛症及顽固性癫痫的近期疗效肯定     

Hypothalamic hamartoma and infantile spasms

PURPOSE: A small number of patients with hypothalamic hamartoma (HH) present with infantile spasms (IS) as an initial or early seizure type. We describe the clinical features of IS associated with HH. METHODS: Our series of patients with HH and epilepsy was reviewed to identify cases with a history of IS. The clinical features and neuroradiological findings in this study group were compared to a control group of patients with HH and refractory epilepsy, but without a history of IS. RESULTS: We identified six patients with HH and a history of IS in this series (n = 122, 4.9%). Five of the six are male. Four of the six patients (67%) developed IS as their first seizure type. The mean age for onset of IS was 6.2 months (range 4-9 months). Results of electroencephalographic (EEG) study at the time of IS diagnosis showed hypsarrhythmia in two (33%). Five patients were treated with adrenocorticotropic hormone (ACTH), and four of the five (80%) responded with control of IS. However, these patients developed other seizure types, and were ultimately refractory to medical management. Aside from the IS, no significant differences in clinical and imaging features were determined between the study group and the control group. CONCLUSIONS: HH should be included in the differential diagnosis for infants presenting with IS. These patients may have hypsarrhythmia on initial EEG, and may respond to ACTH treatment with improvement of IS. However, all became refractory with other seizure types, more commonly seen in HH patients. Focal pathologies associated with IS may be subcortical, as well as cortical, in nature. We have not identified any predictive features for the occurrence of IS in the HH population.     

A longitudinal study of clinical and electroencephalographic findings in a female infant with early myoclonic encephalopathy]

We reported a female infant with early myoclonic encephalopathy (EME). She was diagnosed on the basis of clinical and laboratory features including electroencephalographic and magnetic resonance image (MRI) findings. Frequent erratic myoclonic seizures appeared since 28 days after birth and EEG showed a typical suppression-burst pattern. We administered a high-dose pyridoxal phosphate, thyrotropin-releasing hormone analogue (TRH), and then ACTH, but could not control the seizures at all. With seizure types, we observed the change from erratic myoclonus to tonic spasms in series, with concomitant EEG change to hypsarhythmia at the age of 6 months. Cranial MRI revealed delayed myelination in the white matter but no brain malformation. We administered ACTH to her again and succeeded partially in the decrease of the seizure frequency, and significantly in the improvement of EEG findings. It is supposed that the responsiveness to ACTH treatment changed with age as the seizure patterns developed from erratic myoclonus to tonic spasm.     

抗精神病药所致代谢综合征的干预

目的：探讨综合干预措施对抗精神病药所致代谢综合征的疗效。方法：将122例使用抗精神病药导致代谢综合征的精神分裂症患者随机分为干预组60例和对照组62例,干预组在抗精神病药治疗的同时,进行标准护理、饮食、运动、睡眠、戒烟、限酒和每周1h认知行为治疗的综合干预,对照组仅给予传统的健康教育,饮食、运动无特殊要求;6个月后测定两组体质量指数、血压、血糖和血脂改善情况。结果：两组干预前血压、血脂、血糖及体质量差异无显著性（P均〉0.05）;综合干预后以干预组血压、血糖、血脂及体质量指数各项指标改善明显,两组间比较,差异有统计学意义（P〈0.05或P〈0.01）。两组干预效果比较,以干预组血压（χ2=7.85,P〈0.05）、血糖（χ2=29.56,P〈0.01）、血脂（χ2=43.27,P〈0.01）及体质量指数（χ2=46.34,P〈0.01）改善效果显著。结论：对抗精神病药所致代谢综合征的患者进行综合干预,能明显促进各项代谢异常指标的改善。     

经鼻蝶神经内镜垂体瘤手术与显微镜手术的对比研究

目的对比经鼻蝶神经内镜垂体瘤手术与显微镜手术的有效性及安全性,探讨前者的应用价值。方法分析2012年1月至2014年1月我院接受经鼻蝶神经内镜手术与显微镜手术的90名垂体瘤患者的临床资料,其中经鼻蝶神经内镜手术者52例作为观察组,显微镜手术者48例为对照组,比较两组患者肿瘤全切除率、视力视野恢复情况、内分泌功能治愈率、手术并发症（鼻出血、蝶窦炎、脑脊液鼻漏、神经血管损伤、尿崩、垂体前叶功能减退、颅内感染等）的发生率以及手术时间及住院时间。结果两组患者一般资料比较,差异均无统计学意义（P〉0.05）,具有可比性。观察组与对照组相比,肿瘤全切除率无统计学差异（49/52vs 44/48,χ^20.252,P=0.615）;术后视力视野改善有效率的比较,两组无统计学差异（25/27vs 21/24,χ^20.373,P=0.542）;观察组生长激素型治愈8例（72.7%,8/11）催乳素型治愈7例（77.8%,7/9）,促肾上腺皮质激素型治愈4例（80%,4/5）,促甲状腺激素型治愈3例（100%,3/3）,总治愈率为78.6%（22/28）;对照组生长激素型治愈11例（78.6%,11/14）催乳素型治愈6例（75.0%,6/8）,促肾上腺皮质激素型治愈4例（66.7%,4/6）,促甲状腺激素型治愈2例（66.7%,2/3）,总治愈率为74.2%（23/31）,两组内分泌功能治愈率的比较,差异无统计学意义（χ^20.156,P=0.693）。两组患者手术并发症的比较,差异无统计学意义（8/52vs 10/48,χ^20.502,P〉0.05）。观察组与对照组手术时间及住院天数相比,差异均有统计学意义（79±21vs 93±26,t=2.972,P=0.004;5.6±2.4vs 6.9±3.2,t=2.309,P=0.023）。结论经鼻蝶神经内镜垂体瘤切除术与显微镜手术相比,在肿瘤切除、视力视野及内分泌功能改善、手术并发症方面类似,但可以缩短手术及住院时间,患者恢复更快。     

颞叶癫痫手术效果与术前综合定位手段的相关分析

目的 探讨颞叶癫痫手术治疗效果与多种术前定位方法的相关关系.方法 选择2001年9月～2008年9月在我院行颞叶切除的癫痫患者99例,随访1～6年,按Engle预后分级,Ⅰ级:65例,Ⅱ级10例,Ⅲ级9例,Ⅳ级15例,Ⅰ～Ⅱ级为预后良好,分析这组病例的术前定位方法与手术效果的关系,确定其预测价值.结果 (1)发作间期脑电图颞区有局限慢波的48例患者中41例(85.4%)手术疗效好,无局限性慢波的27例中17例(63%)手术效果好,两者之间有显著性差异(χ2=4.97,P=0.027);(2)脑电图发作间期蝶骨电极单侧尖波57例中,效果好的47例(83%),双侧尖波18例,效果好11例(61%),两组之间无显著性差异(χ2=3.566,P=0.063);(3)发作期起始脑电图表现形式一致的56例中,效果好47例(84%),表现不一致的12例中4例(33%)手术效果好,两者之间有显著性差异(χ2=13.5,P=0.001);(4)发作极期脑电图:发作过程中脑电图无扩展的23例中14例(60%)手术效果好;脑电图向手术切除侧同侧扩展的39例中34例(87%)疗效好;向对侧扩展的6例中3例(50%)效果好,三者之间显著性差异(χ2=7.535,P=0.023);(5)头颅MRI与手术效果:82例患者的头颅MRI:未见结构异常7例中6例(85.7%)效果好,单侧颞区萎缩的10例中4例(40%)效果好,海马硬化的27例中24例(88.9%)效果好,颞区有明显结构异常的18例中13例(72.2%)效果较好,其他部位异常信号的20例中17例(85%)效果好,显示不同MRI改变之间的手术效果有显著性差异(χ2=11.463,P<0.02);(6)埋置颅内电极的20例中9例(45%)效果较好,未埋置颅内电极79例中66例(80%)效果好,二者之间有显著性差异(χ2=16.36,P=0.001);(7)其他:手术切除的侧别、发作症状和有无继发性的全面发作均与手术效果无明显的相关性.结论 颞叶癫痫手术切除效果与头颅MRI的结构改变有关;放置颅内电极与未放置颅内电极的手术效果有明显的差异,并与放置颅内电极的病例定位难度较大有关;颞叶切除的手术效果与患者不同次发作期脑电图改变是否一致有明显的相关性,发作起始期脑电图改变方式一致的手术效果较不一致的效果好;发作间期背景无局限性慢波、有局限性慢波的手术疗效之间有显著性差异,有慢波的手术疗效好,而发作间期的颞区尖波不能预测手术效果.     

Infantile spasms treated with the ketogenic diet: Prospective single‐center experience in 104 consecutive infants

Purpose: In 2002, we reported our preliminary experience using the ketogenic diet (KD) for predominantly intractable infantile spasms (IS) in 23 infants. Since that time, we have increased our use of the KD for this condition including those with new‐onset IS. Methods: Infants were referred and prospectively started on the traditional KD from 1996 to 2009 at our institution. Included subjects had documented clinical IS, hypsarrhythmia on electroencephalography (EEG), and parental consent to start the KD. Efficacy was assessed through phone communication, clinic visits, and EEG every 3 months. Results: One hundred four infants, mean age 1.2 years, were started on the KD for IS, of which 74 (71%) had a symptomatic etiology. Previous therapy for this patients included a mean of 3.6 anticonvulsants; 71% including corticosteroids or vigabatrin. Using an intent‐to‐treat analysis, >50% spasm improvement occurred in 64% at 6 months and 77% after 1–2 years. Thirty‐eight (37%) became spasm‐free for at least a 6‐month period within a median 2.4 months of starting the KD. In addition, 62% reported improvement in development, 35% had EEG improvement, and 29% were able to reduce concurrent anticonvulsants. Adverse effects were noted in 33%, of which 6% had diminished linear growth. Older age at onset of IS and fewer prior anticonvulsants were more likely to be associated with >90% spasm improvement at 6 months. Discussion: The KD is an efficacious therapy for IS in approximately two‐thirds of patients treated, and it should be considered strongly after failure of corticosteroids and vigabatrin.     

West syndrome and other infantile epileptic encephalopathies--Indian hospital experience.

Children with infantile epileptic encephalopathies comprising 3.5% of the Pediatric Neurology Clinic registrations in a tertiary care hospital were retrospectively analyzed. Data were retrieved from case records and analyzed for seizure semiology, prenatal and perinatal insults, developmental status and relevant investigations. The various therapeutic modalities and their influence on spasm frequency, long-term development and final seizure status were compared. The two primary outcome variables analyzed included final seizure status and developmental outcome. Of the 94 infantile epileptic encephalopathies, West syndrome was the commonest (55.3%), of which two thirds were symptomatic. Etiological factors were prenatal in 66.6% and perinatal in 33.3%. The initial response to ACTH was good in 54.5% with subsequent relapse in 27.8% and for prednisolone was 52.9 and 44.4%, respectively, compared to 25.3% spasms control with conventional antiepileptic drugs. Disease category of infantile epileptic encephalopathies evolved in 4, i.e. early myoclonic encephalopathy to West syndrome 1, early infantile epileptic encephalopathy to West syndrome 1, West syndrome to Lennox-Gastaut syndrome 2. Psychomotor retardation was seen in 88.2%, with 16.1% having normal development at onset of spasms. Microcephaly was associated with delayed development but did not influence final seizure outcome. Final seizure outcome was poor in children with delayed development at onset (adjusted odds ratio [OR]=4), delay in diagnosis >12 months (OR=2.27) and in children with Lennox-Gastaut syndrome (OR=4.75). ACTH/prednisolone and antiepileptic treatment versus antiepileptics alone showed a good final seizure response in 36.6% versus 20%. Development on follow up was delayed in children with initial psychomotor retardation (OR=23.4) and abnormal electroencephalogram (OR=7.46). Perinatal factors constituted one third of symptomatic West syndrome. The use of ACTH/corticosteroids resulted in good initial spasm control though final seizure outcome and development were unaffected. Prednisolone had similar response to ACTH in spasm control but higher subsequent relapse rate. Vigabatrin was useful though often unaffordable. The identification of a neurometabolic etiology, though uncommon, has significant therapy implications. Delay in diagnosis was common and negatively influenced final seizure outcome.     

West syndrome and other infantile epileptic encephalopathies--Indian hospital experience

Children with infantile epileptic encephalopathies comprising 3.5% of the Pediatric Neurology Clinic registrations in a tertiary care hospital were retrospectively analyzed. Data were retrieved from case records and analyzed for seizure semiology, prenatal and perinatal insults, developmental status and relevant investigations. The various therapeutic modalities and their influence on spasm frequency, long-term development and final seizure status were compared. The two primary outcome variables analyzed included final seizure status and developmental outcome. Of the 94 infantile epileptic encephalopathies, West syndrome was the commonest (55.3%), of which two thirds were symptomatic. Etiological factors were prenatal in 66.6% and perinatal in 33.3%. The initial response to ACTH was good in 54.5% with subsequent relapse in 27.8% and for prednisolone was 52.9 and 44.4%, respectively, compared to 25.3% spasms control with conventional antiepileptic drugs. Disease category of infantile epileptic encephalopathies evolved in 4, i.e. early myoclonic encephalopathy to West syndrome 1, early infantile epileptic encephalopathy to West syndrome 1, West syndrome to Lennox-Gastaut syndrome 2. Psychomotor retardation was seen in 88.2%, with 16.1% having normal development at onset of spasms. Microcephaly was associated with delayed development but did not influence final seizure outcome. Final seizure outcome was poor in children with delayed development at onset (adjusted odds ratio [OR] = 4), delay in diagnosis >12 months (OR = 2.27) and in children with Lennox-Gastaut syndrome (OR = 4.75). ACTH/prednisolone and antiepileptic treatment versus antiepileptics alone showed a good final seizure response in 36.6% versus 20%. Development on follow up was delayed in children with initial psychomotor retardation (OR = 23.4) and abnormal electroencephalogram (OR = 7.46). Perinatal factors constituted one third of symptomatic West syndrome. The use of ACTH/corticosteroids resulted in good initial spasm control though final seizure outcome and development were unaffected. Prednisolone had similar response to ACTH in spasm control but higher subsequent relapse rate. Vigabatrin was useful though often unaffordable. The identification of a neurometabolic etiology, though uncommon, has significant therapy implications. Delay in diagnosis was common and negatively influenced final seizure outcome.     

颞叶内侧癫痫的两种术式对发作控制的对比分析

目的观察前颞叶切除术和选择性海马、杏仁核切除术对颞叶内侧癫痫的发作控制效果是否有差别。方法 2009年1月至2010年12月在我科行前颞叶切除术的67例颞叶内侧患者为A组;2011年6月至2013年5月在我科行选择性海马、杏仁核切除术的46例颞叶内侧患者为B组;统计分析两组术后1年发作控制为Engel I-II级和Engel III-IV级的人数。结果 A组Engel I-II级56例(83.58%),Engel III-IV级11例(16.42%);B组Engel I-II级40例(86.95%),Engel III-IV级6例(13.05%)。经χ2检验两组术后对MTLE发作的控制率无统计学差异,χ2=0.243,P0.05。结论前颞叶切除术和选择性海马、杏仁核切除术对颞叶内侧癫痫发作都能获得良好的控制,两者疗效无明显差异。