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[一例表现为进行性截瘫的亚急性脊髓疾病尸检病例]

[An autopsy case with subacute spinal cord disease showing progressive paraplegia].

作者信息

Shimura Toshiro, Mori Osamu, Yamazaki Mineo, Takagi Toru, Aoyama Sumio, Hashizume Yoshio, Yamada Noritaka, Sugisaki Yuichi, Katayama Yasuo, Teramoto Akira

机构信息

Department of Neurosurgery, Nippon Medical School, Tama Nagayama Hospital, 1-7-1 Nagayama, Tama-shi, Tokyo 206-8512, Japan.

出版信息

No To Shinkei. 2002 Jun;54(6):543-9.

PMID:12166108
Abstract

We report a 72-year-old woman who died of respitory failure. History included onset of diabetes mellitus at the age of 67 years and hypertension at the age of 72 years. The patient had been in good health otherwise until 2000, when she had onset of numbness or tingling of the bilateral lower limbs. On December 3, 2000, she was admitted to a hospital in the vicinity of her home because of the above-mentioned complaints. Neurological examinations revealed progressive paraplegia. Symptoms and signs suggested Guillain-Barré syndrome. Examinations of cerebrospinal fluids revealed cell count of 338/3 (mono 72%, poly 18%) and protein value of 100 mg/dl. Later the patient course deteriorated. On December 15, 2000, she was admitted to Hakujikai Memorial Hospital for the second time. Ten days later, MRI examination showed diffuse swelling of the spinal cord from the cervical (C 3/4) level to the thoracic level. Gd-enhanced T 1-weighted MRI performed 22 days later showed a partially enhanced lesion at the thoracic (Th 5/6) level of the spinal cord. The patient was treated with steroid therapy (methylprednisolone 500 mg/dl). She died of respiratory failure on January 6, 2001. The patient was presented in a neurological CPC. Neurological and imaging findings suggested a transverse myelopathy. However, there were several points in this case that were unusual for a typical transverse myelopathy, such as total sensory loss below spinal segments of thoracic level (Th 5) and motor weakness of the upper limbs of upper segment of the same level. A clinical neurologist concluded that the patient had subacute transverse myelopathy with fused multiple pathy pathologic lesions. We discussed whether this case was a transverse myelopathy or multiple sclerosis. Post mortem examination revealed acute necrotic myelopathy affecting the spinal cord from the second cervical to the tenth thoracic vertebrae, with conspicuous infiltration of CD 68-positive macrophages involving both gray and white matter, partially necrotic associated with scattered UCHL-1 dominants lymphocytic infiltration of T cells around vessels. There were relatively older lesions with demyelinating features in the spinal roots that were particularly dominant in the anterior roots. No demyelinated plaques in the optic chiasm, tracts and nerves, or in the cerebero-cerebellar white matter were found. Systemic pathological diagnosis was lung edema with fresh hemorrhage, pancreatic atrophy consistent with diabetes mellitus and choleductlithiasis.

摘要

我们报告一名72岁女性,她死于呼吸衰竭。病史包括67岁时患糖尿病,72岁时患高血压。该患者此前身体健康,直到2000年,她开始出现双侧下肢麻木或刺痛。2000年12月3日,因上述症状她入住家附近的一家医院。神经系统检查显示进行性截瘫。症状和体征提示格林-巴利综合征。脑脊液检查显示细胞计数为338/3(单核细胞72%,多形核细胞18%),蛋白值为100mg/dl。后来患者病情恶化。2000年12月15日,她第二次入住白菊会纪念医院。十天后,MRI检查显示脊髓从颈椎(C3/4)水平到胸椎水平弥漫性肿胀。22天后进行的钆增强T1加权MRI显示脊髓胸椎(Th5/6)水平有部分强化病变。患者接受了类固醇治疗(甲泼尼龙500mg/dl)。她于2001年1月6日死于呼吸衰竭。该病例在神经科病例讨论会上被提出。神经学和影像学检查结果提示为横贯性脊髓病。然而,该病例有几个方面对于典型的横贯性脊髓病来说并不寻常,比如胸段脊髓节段(Th5)以下完全感觉丧失以及同一水平上段上肢的运动无力。一位临床神经学家得出结论,该患者患有伴有融合性多病理病变的亚急性横贯性脊髓病。我们讨论了该病例是横贯性脊髓病还是多发性硬化。尸检显示急性坏死性脊髓病累及从第二颈椎到第十胸椎的脊髓,CD68阳性巨噬细胞显著浸润灰质和白质,部分坏死并伴有血管周围散在的UCHL-1主导的T淋巴细胞浸润。脊髓神经根有相对较陈旧的具有脱髓鞘特征的病变,在前根中尤为明显。在视交叉、视束和神经以及大脑-小脑白质中未发现脱髓鞘斑块。全身病理诊断为肺水肿伴新鲜出血、符合糖尿病的胰腺萎缩和胆管结石。

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