肝性脊髓病27例临床分析

目的探讨肝性脊髓病的临床特点、预后和治疗方法。方法收集2001-01—2012-09我院收治的肝性脊髓病27例,完善相关实验室检查,并给予内科综合治疗,1例行肝移植治疗,1例行自体骨髓干细胞移植治疗。结果内科综合治疗可暂时减轻病情,但远期预后差。1例患者行同种异体肝移植,术后下肢肌力改善。1例行自体骨髓干细胞移植,术后肝功能明显改善,下肢肌力改善。结论肝性脊髓病预后不良,目前尚无特殊治疗方法,肝移植和干细胞移植可能是治疗该病的有效方法。     

肝性脊髓病合并肝性脑病13例临床影像特点与预后

目的探讨肝性脊髓病合并肝性脑病的发病机制、临床表现、影像特点、治疗和预后。方法回顾性分析13例肝性脊髓病合并肝性脑病患者的临床表现、影像特点及预后,并结合文献进行分析。结果本病多发生于肝硬化自发性或手术门体分流后,临床主要表现可逆的脑病症状,进行性以双下肢为主的痉挛性瘫痪,仅1例有括约肌功能障碍,无肌萎缩、感觉障碍。9例血氨增高,9例脑电图表现弥漫性θ波,其中2例有三相波,8例MRIT1像显示基底神经节高信号,脊髓MRI阳性率低。所有患者均限制蛋白摄入量,给予护肝、降血氨、神经营养等治疗,3例加用糖皮质激素。9例患者脑病症状缓解,但瘫痪改善不明显;死亡3例,仅1例肝移植患者预后良好。结论脊髓症状与脑病症状并不平行消长,可能存在不同的发病机制。神经影像学研究为慢性肝病神经系统并发症的诊断提供了新思路。常规治疗脑病症状好转,但脊髓病变预后不良,肝移植为慢性肝病神经系统并发症带来曙光。     

酒精性肝硬化并发肝性脊髓病的临床分析

目的 探讨酒精性肝硬化并发肝性脊髓病的临床特点。方法 回顾性总结8例酒精性肝硬化并发肝性脊髓病患者的临床资料。结果 8例患者均为男性,缓慢起病,有酒精性肝硬化病史,主要表现为痉挛性截瘫,肝功能异常,血氨均增高,头颅MRI、颈椎、胸椎、腰椎MRI未见明显异常。仅2例患者经治疗后症状稍好转。结论 酒精性肝硬化并发肝性脊髓病的实验室检查多呈轻度异常,CT和MRI检查无器质性病变,早期营养神经治疗可能有效。     

24例肝性脊髓病的临床诊治体会

目的 探讨肝性脊髓病(hepatic myelopathy,HM)的临床特征、误诊原因及防范措施.方法 回顾性分析2009年11月-2019年6月西京医院收治的24例HM患者的临床资料,并复习相关文献.结果 24例患者均有肝硬化病史,临床表现为双下肢肌力减低,部分实验室检查和影像学检查异常.目前HM尚无统一的诊断标准,...     

肝性脊髓病临床诊治分析

目的 分析肝性脊髓病的临床特点及治疗方法.方法 对临床确诊的6例肝性脊髓病的临床资料进行回顾性分析.结果 6例患者均表现为双下肢痉挛性瘫痪,虽经积极降氨等治疗,但恢复差.随访2例死于肝昏迷、消化道大出血,余2例2 a内未好转.结论 HM患者以下肢痉挛性瘫痪为主要表现,多为神经功能不可逆损害,内科治疗效果不佳,肝移植可能为目前最好的治疗方法.     

肝性脊髓病1例报告及文献复习

肝性脊髓病临床上少见,相关文献报道也不常见.本院近期收治1例肝性脊髓病患者,现报告如下,并查阅国内外相关个案报道资料,就肝性脊髓病的临床特点和诊治进行总结.     

肝炎后肝硬化伴肝性脊髓病1例报告

肝性脊髓病(hypatic myeloathy,HM)是多种肝病引起的颈髓以下脊髓侧索脱髓鞘病变,呈现肢体缓慢进行性对称性痉挛性瘫痪,多见于有多次肝性脑病发作或施行了门体分流术的肝硬化患者.本病的脊髓损害是不可逆的,痉挛性瘫痪进行性加重,预后不良.HM临床上少见,相关文献报道也不常见.     

硬脊膜动静脉瘘28例误诊及预后分析

目的总结硬脊膜动静脉瘘的误诊原因及对预后的影响,提高其早期诊断和治疗比例,提高治疗效果。方法搜集并回顾性分析本单位近6年收治的硬脊膜动静脉瘘患者52例,并予以随访,将28例误诊患者和另外24例比较,分析患者的误诊和延误治疗原因、诊治方法和术后随访结果。结果 52例患者中有15例(28.85%)曾被诊断为"脊髓炎、脊髓空洞症、蛛网膜炎或脱髓鞘疾病等",13(25%)例曾被诊断为"椎间盘突出或椎管狭窄",其中8例接受了颈椎或腰椎手术治疗,3例(5.77%)曾被诊断为"前列腺增生症,前列腺炎"。52例均经手术和栓塞治疗治愈;随访患者术后症状多得到不同程度的改善,但早期得到确诊和治疗的患者预后较误诊的患者改善更显著。结论硬脊膜动静脉瘘临床症状不典型,易于和椎间盘突出、脊髓病混淆,容易误诊误治,早期诊断和及时治疗可以更好地改善预后。     

脑与脊髓同时受累的瘤样炎性脱髓鞘病一例

目的了解瘤样炎性脱髓鞘病(TIDD)的临床表现、诊断及治疗方法。方法介绍我院收治的1例脑与脊髓同时受累的TIDD患者的临床表现、影像学特点及治疗方法。结果本病临床症状多种多样,出现头晕、头痛及肢体无力等症状,进展迅速,头颅与脊髓影像学检查可见异常信号。给予大剂量注射用甲泼尼龙琥珀酸钠冲击治疗,效果显著,预后较好。结论本病为中枢神经系统脱髓鞘疾病,临床表现多种多样,易与颅内肿瘤相混淆,激素冲击治疗有效。     

乙型肝炎后肝硬化并肝性脊髓病1例报告

正肝性脊髓病(HM)又称门-腔分流性脊髓病,是肝硬化门静脉高压致门腔静脉分流引起的一种特殊的以脊髓病变为主的综合征,主要表现为肢体缓慢进行性痉挛性截瘫为特征~([1])。本病最早由Leigh和Card于1949年共同报告~([2]),临床上比较少见,预后不佳,目前尚无诊断金标准,容易误诊或漏诊。现就我院收治的1例HM患者报告如下。1病例男,48岁,因"反复双下肢水肿5年,加重伴双下肢乏力1周"收治我院消化内科。患者自2013年开始反复出现双下肢凹陷性水肿,无腹痛、腹胀等不适,多次就诊于当地人民医院,诊断为"乙型肝炎后肝硬化失代偿期",予抗病毒、补充白蛋白、利尿等治疗后症状可缓解。1周前患者再次出现双下肢水肿,伴双下肢乏力感,能自行行走,无言语不清、吞咽困难,无四肢麻木及大小便障碍。既往史及个人史:发现乙型     

肝性脊髓病7例临床分析

目的 探讨肝性脊髓病(HM)的临床特点及治疗方法.方法 对7例确诊HM患者的临床资料进行回顾性分析.结果 7例患者均具有典型的痉挛性截瘫的临床表现,无感觉障碍;2例(28.57%)曾接受门腔静脉分流术;5例(71.43%)血氨轻中度增高,2例血氨检测正常(28.57%);6例(85.71%)内科治疗无明显效果.结论 HM患者以痉挛性截瘫为主要表现,血氨增高可能不是唯一致病因素,常规内科治疗效果不佳,肝移植为HM的治疗带来希望.

Abstract：

Objective To analyze the clinical features of hepatic myelopathy ( HM ) , and to explore the possible treatment measures for HM patients. Methods Clinical data of 7 definite HM patients were analyzed retrospectively. Results All 7 HM patients (100% ) had typical clinical features of spastic paraparesis but without sensory dysfunction. 2 of 7 cases (28.57% ) received porto-systemic shunts. The blood ammonia level of 2 cases(28. 57% )was normal and was slightly or moderately increased in the other 5 patients (71.43% ). All 7 HM patients received medicine treatment. There was no significant effect in 6 cases ( 85. 71 % ) . Conclusions HM is characterized clinically by spastic paraparesis without sensory dysfunction. The increased blood ammonia level is not the only cause and routine medicine treatment is proved to be useless. Liver transplantation may bring hope for HM patients.     

Hepatic myelopathy with spastic paraparesis

Progressive myelopathy is a rare neurological complication of chronic liver disease with portal hypertension and there is no special diagnostic tool for hepatic myelopathy. Neuropathological studies of the patients with hepatic myelopathy have demonstrated demyelination of the lateral corticospinal tracts with various degree of axonal loss. Transcranial magnetic stimulation (TMS) is widely utilized as an indicator of changes in exitability and conductivity of the motor pathways. TMS studies are also used for the diagnosis of hereditary spastic paraparesis in the literature. In this study, we described two patients who presented with spastic paraparesis; TMS studies suggested that they had myelopathy and diagnosed as hepatic myelopathy when all the other possible diagnoses were ruled out.     

Myelopathy - Sjogren's syndrome association: analysis of clinical and radiological findings and clinical course]

Myelopathies associated with Sj?gren's syndrome has been rarely described especially concerning magnetic resonance imaging (MRI) and treatment aspects. The aim of this study was to determine the clinical, laboratory and radiological features of myelopathies occurring in Sj?gren's syndrome. Eleven patients were studied, 7 with an acute myelopathy and 4 with a chronic form. Acute myelopathy were clinically severe with a feature of transverse myelitis necessitating immunosuppressive drugs. On the other hand, chronic forms were closely similar to progressive multiple sclerosis (MS), for clinical and laboratory data. In 7 cases optic neuritis was found associated with myelopathy and fulfilled the diagnostic criteria of Devic's syndrome in 4 cases. The diagnosis of myelopathy associated with Sj?gren's syndrome may be difficult especially compared with MS, HTLV1 or HIV myelopathy and sarcoidosis, in the chronic form but also with other vasculitis, MS or viral infection in the acute forms. However, in this last form, magnetic resonance imaging and cerebrospinal fluid data should bring to the diagnosis of Sj?gren syndrome and confirmed by appropriate tests. This diagnosis will have direct consequences for an early treatment by immunosuppressive drugs.     

寰枕畸形伴发脊髓型颈椎病患者一期手术治疗的临床效果评价

目的 根据寰枕畸形伴发脊髓型颈椎病患者的不同临床特征、影像学特点采取不同手术方式组合对颈脊髓进行减压,并评价探讨其疗效和预后.方法 北京大学第三医院自2002年1月至2007年7月共收治22例寰枕畸形伴发脊髓型颈椎病患者,在了解患者首发症状及病程演变情况后,以日本矫形外科学会(JOA)评分系统对手术前后体征进行分析比较,常规行X片、CT、MRI等影像学检查.一期以不同术式组合(枕颈减压、枕大池成形3例,枕颁减压、枕大池成形和空洞穿刺引流1例,枕颈减压、单开门椎管扩大成形5例,枕颈减压、局限性单侧椎板切除减压1例,枕颈减压、钛板内固定1例,枕颈减压+颈前路间盘摘除、植骨、钛板内固定1例,枕颈减压、枕大池成形+单开门椎管扩大成形3例,枕颈减压、枕大池成形+局限性单侧椎板切除减压4例,枕颈减压、枕大池成形和空洞穿刺引流+局限性单侧椎板切除减压3例)对两种病变造成的颈脊髓等神经系统压迫进行允分减压.术后全部患者均随访调查,并以改善、稳定、进展时患者术后中期状况进行评价.结果 术后效果优6例(手术前后JOA分值差≥2),好13例(JOA分值差=1),一般3例(JOA分值差为0).随访2～48月,进一步改善20例,稳定2例.结论 根据不同临床表现、体征和影像学特点,采取不同术式组合一期手术治疗寰枕畸形伴发脊髓型颈椎病可获良好的治疗效果.     

An autopsy case with adult onset type II citrullinemia showing myelopathy

Hepatic myelopathy is a rare neurological complication in patients with chronic liver failure and most patients who suffered from this disorder were demonstrated to have portal-systemic shunt. A 31-year-old man who was diagnosed as having adult-onset type II citrullinemia (CTLN2) and had a six-year history of recurrent hepatic encephalopathy showed progressive spastic paraparesis with no involvement of sensation and sphincter function. Examinations of cerebrospinal fluid and spinal MRI were normal. He suddenly died of acute exacerbation of hepatic encephalopathy with severe brain edema. The pathology of the spinal cord disclosed a localized degeneration of both lateral columns, the lesion being more remarkable in the lower segments of the cord. These clinical and pathological findings of hepatic myelopathy have not been noted in the many patients with CTLN2 previously reported, and our patient is unique in developing hepatic myelopathy without porto-caval shunting. Thus, repeated attacks of encephalopathy with hyperammonemia might secondarily have induced the myelopathy in this patient.     

30例Ramsay-Hunt综合征临床分析

目的探讨Ramsay-Hunt综合征的药物治疗以及影响预后的因素。方法回顾性分析2015-01—2019-01在我科诊治的30例Ramsay-Hunt综合征患者的临床资料,面神经功能评估采用House-Brackman分级。结果所有患者于发病1周内就诊,可见耳甲腔或外耳道疱疹,同侧面瘫均Ⅲ级及以上,其中伴声嘶2例,伴眩晕5例,伴面部疱疹4例。经过2周药物治疗后,2例伴声嘶患者面神经功能为Ⅲ级,1例治疗后2周复发,其余患者面神经功能均恢复正常。结论Ramsay-Hunt综合征早期药物治疗面瘫恢复较好,但累及迷走神经者预后较差。     

Devic's neuromyelitis optica: A clinicopathological study of 8 patients

We report the clinical, imaging, and laboratory features of 8 patients with Devic's neuromyelitis optica. All patients had severe myelopathy and optic neuritis. In no patient was the brain, the brainstem, or the cerebellum affected, even after several years of disease. Various immunosuppressive treatments failed to benefit the patients, 5 of whom died. Autopsies of these 5 patients demonstrated a severe necrotizing myelopathy with thickening of blood vessel walls and no lymphocyte infiltrates. In the appropriate clinical setting, the lack of white matter abnormalities demonstrated by magnetic resonance imaging of the head facilitates the recognition of Devic's syndrome during life. Inasmuch as Devic's myelopathy is necrotizing, rather thatn demyelinating, the prognosis of this syndrome is poor.