Konno H, Takase S, Fukui T
Department of Clinical Pathology, Kohnan Hospital, Sendai.
No To Shinkei. 2001 Sep;53(9):875-80.
The present case, 78-years-old female, had been troubled with SMON during 28 years, and was suddenly died from cerebral hemorrhage. The neuropathology characteristic for SMON could not be revealed in the spinal cord on routine histological preparations. There were decreasing number of immunopositive granules in the nucleus gracilis of medulla oblongata for synaptophysin, and in the marginal zone of the lumbar dorsal horn for substance P. These findings showed the presence of tractus gracilis degeneration and the disappearance of nociceptive fibers from the dorsal roots, respectively. To elucidate the histopathological changes of the spinal cord in long standing cases with SMON, the 11 cases reported were reviewed. The mean duration from the onset to death was 22.8 years(16 to 33 years). The mean age was 76.3 years old(54 to 92), and the 10 cases were female. All cases showed sensory impairment of the lower extremities, while deep tendon reflex exaggerated was seen in 7 cases. There were nine cases involving dorsal funiculi degeneration of the spinal cord, in contrast the lateral funiculi alternation were noticed in only two cases. It was considered that the corticospinal tract alteration becomes undiscernible during the long course, although the dorsal funiculi degeneration remained.
本病例为一名78岁女性,患亚急性脊髓视神经病28年,突然死于脑出血。在常规组织学切片上,脊髓未显示出亚急性脊髓视神经病的神经病理学特征。延髓薄束核中突触素免疫阳性颗粒数量减少,腰髓后角边缘区P物质免疫阳性颗粒数量减少。这些发现分别表明存在薄束变性和背根伤害性纤维消失。为阐明亚急性脊髓视神经病长期病例脊髓的组织病理学变化,对报告的11例病例进行了回顾。从发病到死亡的平均病程为22.8年(16至33年)。平均年龄为76.3岁(54至92岁),10例为女性。所有病例均有下肢感觉障碍,7例出现腱反射亢进。9例出现脊髓后索变性,仅2例出现侧索改变。尽管后索变性仍然存在,但认为在病程较长时皮质脊髓束改变变得难以辨认。