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伴有痉挛性截瘫的肝性脊髓病:两例报告及文献复习

Hepatic myelopathy with spastic paraparesis: report of two cases and review of the literature.

作者信息

Ben Amor Sana, Saied Mohamed Zakaria, Harzallah Mohamed Salah, Benammou Sofiene

机构信息

Department of Neurology, Sahloul Hospital, Belt drive, 4054, Sahloul city, Sousse, Tunisia,

出版信息

Eur Spine J. 2014 May;23 Suppl 2:167-71. doi: 10.1007/s00586-013-2828-z. Epub 2013 Jun 1.

Abstract

PURPOSE

The present report illustrates two men aged for 59 and 43 years, respectively, who presented with slowly progressive spastic paraparesis.

STUDY DESIGN

Two case reports and review of literature.

METHODS

The patent's history, clinical examination, biology, magnetic resonance imaging (MRI) findings and treatment are reported. We also discuss the pathogenesis and various treatment options.

RESULTS

Neurologic examination showed spastic paraparesis without other neurological disorders. MRI of the spinal cord and brain were normal. Cytologic examination of cerebrospinal fluid from each patient was normal. Hemogram disclosed a pancytopenia. Partial thromboplastin time was prolonged. Liver function tests revealed raised serum bilirubin, normal alanine aminotransferase and aspartate aminotransferase, reduced total protein and albumin. Plasma ammonia was elevated. Blood vitamin B12 and folate values were in normal ranges. Serum antibodies to human T cell lymphotropic virus, human immunodeficiency virus, syphilis and hepatitis C virus were absent. Hepatitis B virus antibody assay was positive. The electromyographic evaluation for second motor neuron involvement was also normal. Endoscopy revealed esophageal varices and a spleno-renal shunt.

CONCLUSIONS

Hepatic myelopathy remains a default diagnosis assigned only after the exclusion of other causes of spastic paraparesis and partial transverse myelopathy. An accurate history, along with appropriate imaging and laboratory findings, is crucial.

摘要

目的

本报告阐述了两名分别为59岁和43岁的男性患者,他们均表现为缓慢进展的痉挛性截瘫。

研究设计

两例病例报告及文献综述。

方法

报告了患者的病史、临床检查、生物学检查、磁共振成像(MRI)结果及治疗情况。我们还讨论了发病机制和各种治疗选择。

结果

神经系统检查显示痉挛性截瘫,无其他神经系统疾病。脊髓和脑部的MRI检查正常。每位患者脑脊液的细胞学检查正常。血常规显示全血细胞减少。部分凝血活酶时间延长。肝功能检查显示血清胆红素升高,丙氨酸转氨酶和天冬氨酸转氨酶正常,总蛋白和白蛋白降低。血浆氨升高。血液维生素B12和叶酸值在正常范围内。血清中不存在人类嗜T细胞病毒、人类免疫缺陷病毒、梅毒和丙型肝炎病毒抗体。乙肝病毒抗体检测呈阳性。对第二运动神经元受累情况的肌电图评估也正常。内镜检查发现食管静脉曲张和脾肾分流。

结论

肝性脊髓病仍是一种仅在排除痉挛性截瘫和部分横贯性脊髓病的其他病因后才做出的默认诊断。准确的病史以及适当的影像学和实验室检查结果至关重要。

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