海马硬化14例临床病理分型及意义

Clinico-pathological classification and significance of 14 cases hippocampal sclerosis

目的探讨海马硬化(hippocampal sclerosis,HS)的组织学特点、病理分型及临床意义。方法回顾性分析14例因HS所致的慢性颞叶癫痫患者的临床资料,结合免疫组化检测结果观察其海马组织形态学,并对患者术后癫痫发作情况进行随访。结果患者年龄5～42岁(平均20岁),男性9例,女性5例,临床均以慢性颞叶癫痫为主要表现。MRI示12例病变累及单侧海马、2例双侧受累。组织学主要表现为海马CA1-CA4区选择性锥体神经元丢失及胶质细胞增生,同时伴齿状回颗粒细胞数量和分布的异常。根据神经元丢失区域及程度的不同,14例分为经典1a型4例(CA1区神经元严重丢失,除CA2区外其他各区中度丢失)和经典1b型8例(各区神经元均严重丢失),非典变异型2型(仅CA1区神经元严重丢失)和3型(神经元丢失以CA4区为主)各1例,1b型最为多见。免疫组化染色神经元核抗原NeuN可清晰显示海马各区神经元的分布及丢失情况。13例获随访,结果表明经典型患者预后好。结论 HS病理形态学改变简单,规范的取材及熟悉海马正常的组织学形态是HS正确诊断及分型的前提和基础,正确的诊断及分型可为临床判断预后提供依据。

Purpose To investigate the morphological features, pathological classification and clinical significance of hippocampal sclerosis （HS）. Methods Clinical data of 14 HS patients suffering from mesial temporal lobe epilepsies was analyzed, and hippocam- pal neuoral cell loss was observed by light microscopy and immunohistochemistry. The follow-up data after surgery were analyzed. Re- suits The patients＇ age ranged from 5 to 42 years （mean, 20 years）, among them 9 male and 5 female. All cases displayed symp- toms of chronic temporal lobe epilepsies. Unilateral hippocampal were involved in 12 patients, and two cases had bilateral lesions. Pathological changes presented with selective neuronal loss in hippocampal subfields, accompanying with glial proliferation and granule cell lesions in dentate gyms. A classical pattern was observed in 12 cases, and in which 8 cases showed extensive neuronal ceils in all hippocampal subfields （Typelb） , 4 cases with severe cell loss in CA1 and moderate neuronal loss in all other subfields excluding CA2 （Typela）. Atypical variants were characterized either by severe neuronal loss restricted to sector CA1 （Type 2） or to the hilar region （Type 3 ） were respectively observed in one patient. Immunohistochemistry staining for NeuN clearly displayed loss and distribution of neuronal cells in CA1-CA4 subfields. Follow-up data （6 to 37 months in 13 cases） displayed the classical type had a good prognosis. Conclusions The pathological changes of hippocampal sclerosis are simple in nature. A well-preserved surgical specimens and famil- iar with the normal hippocampal are the basis of correct diagnosis and histopathological classification, and the latter can allow a reliable prediction for postsurgical outcome.