难治性癫痫相关脑炎的临床病理学观察

Clinicopathologic study of intractable epilepsy-related encephalitis

目的探讨难治性癫痫相关脑炎的临床病理学特点。方法收集经首都医科大学宣武医院功能神经外科手术切除治疗并符合脑炎后病理学改变的15例难治性癫痫病例的临床病理学资料进行回顾性分析。结果15例患者中男4例，女11例；平均发病年龄5．3岁（1～15岁），平均病程为4．7年（0．5～15年）。11例有明确既往史，包括病毒性脑炎9例，宫内缺氧1例和头部外伤史1例。15例患者中单侧大脑半球广泛受累5例，多个脑叶受累7例，单个脑叶受累3例。各脑叶受累情况为：颞叶受累13例，额叶和顶叶受累分别为8例，枕叶受累7例，岛叶受累4例。镜下观察可见蛛网膜下腔血管周围多量炎性细胞浸润；局部大脑皮质萎缩变薄，神经元不同程度脱失伴胶质细胞增生，严重者胶质瘢痕形成；另见周边皮质内散在小胶质细胞结节、淋巴套袖及噬神经细胞现象等脑炎的病理学改变。6例可观察到核内包涵体，免疫组织化学染色巨细胞病毒晚期蛋白阳性，其中有3例伴多核巨细胞浸润及散在钙化灶。另外，有7例同时伴有皮质发育不良；2例伴海马硬化。结论脑炎是引起癫痫发作的常见病因之一，如果不及时治疗可以导致难治性癫痫的严重后遗症。

Objective To investigate the clinicopathologic features of intractable epilepsy related encephalitis. Methods The clinical and pathologic findings of 15 cases of intractable epilepsy after functional neurosurgical treatment were reviewed and analyzed retrospectively. Results All patients, including four male and 11 female, had medically intractable epilepsy. The mean age of onset for seizure was 5.3 years （ 1 - 15 years） and the disease duration was 4. 7 years （0. 5 - 15 years）. A definite past history was identified in 11 patients, including viral encephalitis in nine patients, anoxia in utero and head trauma in one patient respectively. The extent and sites of involvement were different, including single cerebral hemisphere diffusely in five cases, muhiple lobes in seven cases, and single lobe in three cases. Temporal lobe was involved in 13 cases, frontal lobe in eight, parietal lobe in eight, occipital lobe in seven, and insular lobe in four. Microscopically, all cases were characterized by perivascular inflammatory cells infiltration in the subarachnoid space. The focal cerebral cortex showed obvious atrophy with various degrees of the neuronal loss and glial proliferation, eventually leading to glial scar formation. In addition, microglia nodules, lymphatic cuff and neuronophagia were also observed. Seven cases of focal cortical dysplasia were identified among the 11 cases with adequate perilesional cerebral cortex. Hippoeampus sclerosis was found in two cases. Intranuelear inclusions were seen in six cases, and these were immunopositive of cytomegalovirus- late antigen, and three eases also showed muhinucleated giant cells and calcifications. Conclusion Encephalitis is one of the common causes of refractory epilepsy, and may result in refractory epilepsy as a sequel.