Neuropathological Features of MS in Japan
Neuropathological Features of MS in Japan
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日本多发性硬化症的神经病理学特征
DOI：
10.1159/000408050
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发表时间：
1982
期刊：
影响因子：
--
通讯作者：
J. Tateishi
中科院分区：
文献类型：
--
作者：
T. Tabira;J. Tateishi
对6例多发性硬化患者进行了神经病理学检查，其中典型多发性硬化症1例，器质性多发性硬化症5例，后5例表现为反复发作的横断性脊髓病、严重的视力损害和部分脑干体征。病理上主要病变位于脊髓和视神经。脊髓灰质也经常受累。这些病变的特点是严重的脱髓鞘和组织坏死，纤维性星形胶质细胞修复能力差，即使在最近严重的病变中也缺乏炎细胞，其中经常出现新鲜出血。脑干被盖和脑白质也有少量病变，几乎局限于脑室周围区域。回顾1955~1980年日本报道的典型多发性硬化症尸检病例，共收集典型多发性硬化症17例，器质性多发性硬化性多发性硬化症25例，病变多局限于视神经和脊髓，混合型43例。经典型病例病程较长，以男性为主，病程进展缓慢。从这些发现中，我们总结出日本MS的特点：1)典型MS发生率低，Devic型MS发生率高，2)视神经和脊髓病变好发，3)坏死性病变，不仅脊髓和视神经，而且在大脑也有偶见空洞形成，4)胶质细胞增生差，5)坏死型MS血管周围粘连差，与西方MS的不同可能是遗传基础。
SummaryNeuropathological studies were done on our 6 cases of multiple sclerosis including a classical MS and 5 Devic type MS. The latter 5 cases showed recurrent transverse myelopathy, severe visual impairment and some brainstem signs. Pathologically main lesions were present in the spinal cord and optic nerve. The gray matter was also frequently affected in the spinal cord. The lesions were characterized by severe demyelination with tissue necrosis, poor repair by fibrous astrocytes, and lack of inflammatory cells even in the recent severe lesions where fresh hemorrhages were often present. These lesions were also present in the brainstem tegmentum and cerebral white matter to a lesser extent, which were almost limited to the periventricular regions. In some cases lesions characteristic to the classical MS were combined in the cerebrum and brainstem.From the review of autopsy cases reported in Japan between 1955 and 1980 we collected 17 cases of classical MS, 25 cases of Devic type MS in which severe necrotic lesions were almost restricted to optic nerve and spinal cord, and 43 cases of combined form. The classical cases showed longer duration, male predominace and chronic progressive course. The Devic form showed female predominace, shorter duration and remitting course.From these findings we abstracted the following characteristic features of Japanese MS; 1)lower incidence of classical MS and higher incidence of Devic type MS,2)preferential occurrence of lesions in the optic nerve and spinal cord,3)necrotizing lesions with occasional cavity formation not only in the spinal cord and optic nerve but also in the cerebrum,4)poor gliosis, and5)poor perivascular cuffing in the necrotic form.The difference from western MS is probably on the genetic back ground.