Spread of vimentin-immunoreactive cells within the plaque-like lesion in the spinal anterior horn of a patient with post-poliomyelitis syndrome
Spread of vimentin-immunoreactive cells within the plaque-like lesion in the spinal anterior horn of a patient with post-poliomyelitis syndrome
复制标题
脊髓灰质炎后综合征患者脊髓前角斑块样病变内波形蛋白免疫反应细胞的扩散
DOI：
10.1111/neup.12768
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发表时间：
2021
期刊：
影响因子：
2.3
通讯作者：
Sekijima Y
中科院分区：
文献类型：
--
作者：
Hineno A;Oyanagi K;Yoshida T;Sakai Y;Kannno H;Sekijima Y
本研究中的一名日本男子在两岁时右腿严重无力。腿部力量逐渐恢复和发展，一直到50岁左右都能打高尔夫球、爬山。从55岁左右开始，他就无法从弯腰的姿势站起来了。肌肉无力和萎缩蔓延到他的右臂，肌电图显示他的下肢和上肢存在神经源性模式。该患者被诊断为患有脊髓灰质炎后综合症（PPS）。 60岁以后出现双腿麻木、臀部疼痛。当时的计算机断层扫描和磁共振成像显示，下胸椎有脊椎病和骨赘突出，压迫脊髓的第二腰段。 80岁时，他因恶性淋巴瘤和急性间质性肺炎去世。病理检查显示脊髓第二腰段出现横贯性脊髓病并完全坏死。右侧脊髓前角及第三、四腰段中间区萎缩，弥漫性胶质增生。脊髓第三、四腰段右前角可见椭圆形斑块样病变。斑块样病变中的神经元和突触素免疫反应性完全消失。与病变相对应的波形蛋白免疫反应细胞显着扩散，而神经胶质纤维酸性蛋白免疫反应星形胶质细胞均匀存在于脊髓第三和第四腰段两侧的前角和中间区。使用尸检材料进行病毒学检查，脊髓灰质炎病毒呈阴性。在脊髓中未发现 43 kDa 免疫反应包涵体的反式激活反应 DNA 结合蛋白和布尼纳小体。本文展示了 PPS 中特殊“斑块样病变”内波形蛋白免疫反应细胞显着反应的新发现。
A Japanese man in the present study experienced acute weakness in his right leg as a two year old. The strength in his leg gradually recovered and developed, and he could play golf and climb mountains up to around the age of 50. From approximately 55 years of age, he became unable to stand up from a stooped position. Muscle weakness and atrophy spread to his right arm, and an electromyography revealed a neurogenic pattern in his lower and upper extremities. The patient was diagnosed as having post‐poliomyelitis syndrome (PPS). Numbness in both the legs and pain in the buttocks occurred after 60 years of age. Computed tomography and magnetic resonance imaging at that time revealed spondylosis and protrusion of an osteophye in lower thoracic vertebrae compressing the second lumbar segment of the spinal cord. He died of malignant lymphoma and acute interstitial pneumonia at 80 years of age. Pathological examination revealed transverse myelopathy at the second lumbar segment of the spinal cord and total necrosis. The anterior horn and the intermediate zone of the third and fourth lumbar segments of the spinal cord on the right side were atrophic and diffusely gliotic. An oval‐shaped plaque‐like lesion was observed in the right anterior horn at the third and fourth lumbar segments of the spinal cord. Neurons and synaptophysin immunoreactivity had completely disappeared in the plaque‐like lesion. A striking spread of vimentin‐immunoreactive cells was found corresponding to the lesion, while glial fibrillary acidic protein‐immunoreactive astrocytes existed evenly in the anterior horn and intermediate zone on both sides of the third and fourth lumber segments of the spinal cord. Virological examination using the autopsied materials was negative for poliovirus. Neither transactivation response DNA‐binding protein of 43 kDa‐immunoreactive inclusion nor Bunina body was seen in the spinal cord. The present paper demonstrates new findings of a noteworthy response of the vimentin‐immunoreactive cells within the peculiar “plaque‐like lesion” in the PPS.