Iwabuchi K, Yagishita S, Itoh Y, Amano N, Saitoh A
Department of Neuropathology, Psyhiatric Research Institute of Tokyo, Japan.
No To Shinkei. 1990 May;42(5):489-96.
A 45 year-old-female was admitted to Kanagawa Rehabilitation Center because of marked spastic paraplegia. There was no family history of neurological diseases. She had been drinking a great excess of alcohol since twenty years of age. Though she noticed the unsteadiness of gait several years prior to the admission, she had not been examined. On admission neurological examination revealed pyramidal weakness of both legs, and slight sensory impairment in distal part of the lower extremities. But she had neither difficulty in speech nor abnormal findings in the upper extremities. Her mentality was well preserved. Though computed tomography revealed cerebellar cortical atrophy, no signs of cerebellar impairment could not be found except spastic paraplegia. Blood chemistry failed to reveal liver dysfunction. There was no pernicious anemia. These symptoms were almost stationary until her death. At the age of 53, she died of gastric cancer. Postmortem examination disclosed marked degeneration of cerebellum and spinal cord. Cerebellar cortical degeneration, which was characterized by involvement of all layers of the cortex, showed unique distribution. The lingula, central lobule, culmen, superior portion of declive, anterior lobules and anteromedial half of simple lobule were severely degenerated, while other lobules were spared excluding moderate degeneration in inferomedian portion of the hemisphere. These cortical degeneration was prominent much more in vermis than in hemisphere. There was a mild myelin pallor in the lamellar white matter and slight fibrous gliosis. In the dentate nucleus, there were moderate neuronal loss with gliosis.(ABSTRACT TRUNCATED AT 250 WORDS)
一名45岁女性因明显的痉挛性截瘫入住神奈川康复中心。其家族中无神经疾病病史。她自20岁起就大量饮酒。尽管在入院前几年她就注意到步态不稳,但一直未接受检查。入院时神经检查发现双下肢锥体束征阳性,下肢远端有轻微感觉障碍。但她言语无障碍,上肢也无异常发现。其智力保存完好。尽管计算机断层扫描显示小脑皮质萎缩,但除痉挛性截瘫外未发现小脑损害的迹象。血液生化检查未发现肝功能障碍。无恶性贫血。这些症状在她去世前几乎没有变化。53岁时,她死于胃癌。尸检发现小脑和脊髓有明显退变。小脑皮质退变累及皮质各层,表现出独特的分布。舌叶、中央小叶、山顶、山坡上部、前叶和单小叶的前内侧半部分严重退变,而其他小叶未受累,半球下内侧部分有中度退变。这些皮质退变在蚓部比在半球更明显。板层白质有轻度髓鞘苍白和轻度纤维性胶质增生。齿状核有中度神经元丢失和胶质增生。(摘要截断于250字)