Hineno Akiyo, Oyanagi Kiyomitsu, Yoshida Takuhiro, Sakai Yasuhiro, Kanno Hiroyuki, Sekijima Yoshiki
Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.
Intractable Disease Care Center, Shinshu University Hospital, Matsumoto, Japan.
Neuropathology. 2021 Oct;41(5):406-411. doi: 10.1111/neup.12768. Epub 2021 Sep 19.
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.
本研究中的一名日本男性在两岁时右腿出现急性无力。腿部力量逐渐恢复并发展,在50岁左右时他能够打高尔夫球和爬山。大约从55岁起,他无法从弯腰姿势站立起来。肌肉无力和萎缩蔓延至右臂,肌电图显示其上下肢呈神经源性模式。该患者被诊断为患有小儿麻痹后遗症(PPS)。60岁后出现双腿麻木和臀部疼痛。当时的计算机断层扫描和磁共振成像显示下胸椎有骨质增生和骨赘突出,压迫脊髓第二腰椎节段。他80岁时死于恶性淋巴瘤和急性间质性肺炎。病理检查显示脊髓第二腰椎节段有横贯性脊髓病和完全坏死。右侧脊髓第三和第四腰椎节段的前角和中间带萎缩并弥漫性胶质化。在脊髓第三和第四腰椎节段的右侧前角观察到一个椭圆形斑块状病变。斑块状病变中的神经元和突触素免疫反应性完全消失。在病变部位发现波形蛋白免疫反应性细胞有明显扩散,而胶质纤维酸性蛋白免疫反应性星形胶质细胞在脊髓第三和第四腰椎节段两侧的前角和中间带均匀存在。使用尸检材料进行的病毒学检查未检测到脊髓灰质炎病毒。脊髓中未发现43 kDa反式激活反应DNA结合蛋白免疫反应性包涵体或布尼纳小体。本文展示了小儿麻痹后遗症中特殊“斑块状病变”内波形蛋白免疫反应性细胞值得注意的新发现。