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.     

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,...     

9例肝性脊髓病诊治回顾分析

目的探讨肝性脊髓病（Hepaticmyeloathy,HM）的临床特点及治疗方法。方法对9例确诊HM患者的临床资料进行回顾性分析。结果9例患者均具有典型的痉挛性截瘫的临床表现,1例曾接受门腔静脉分流术,8例血氨轻中度增高,血氨检测正常者1例（6．6％）,8例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,血氨增高可能不是惟一致病因素,常规内科治疗效果不佳,肝移植为HM的治疗带来希望。     

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。     

磁共振弥散张量成像在基底节区高血压脑出血致皮质脊髓束损伤中的应用研究

目的 探讨磁共振弥散张量成像(DTI)技术评价基底节区高血压脑出血(HICH)患者皮质脊髓束(CST)受损程度的意义及其与肌力恢复的关系.方法 徐州医学院附属医院神经外科自2006年11月至2009年5月行小骨窗开颅血肿清除术治疗单侧基底节区HICH患者35例,术后10 d应用3.0T磁共振DTI技术检测患者和10例健康志愿者CST,应用Functool软件进行图像分析观察CST损伤程度,HICH患者康复治疗2月后采用Brunnstrom标准进行肢体肌力检查,分析CST损伤程度与肢体肌力的相关性.结果 10例健康志愿者CST显示清晰.35例HICH患者CST受损的模式有3种:纤维束显示达正常侧的2/3或相仿(11例),患者肢体肌力恢复最好;纤维束显示小于正常侧的2/3(18例),患者肢体肌力恢复较好;纤维柬显示小于正常侧的1/3(6例),患者肢体肌力恢复最差.CST受损患者患侧的FA值均较健侧降低,差异有统计学意义(P<0.05).3种模式CST损伤患者患侧的FA值、肢体肌力不同,差异均有统计学意义(P=0.000).患者CST损伤程度与肌力恢复水平存在负相关关系(r=0.931,P=0.000).结论 应用磁共振DTI技术可显示脑内白质纤维束的走形及分布,能够早期检测HICH患者CST的损伤程度,对患者肢体运动功能损伤的评估、判断预后有重要的临床意义.     

自发性脊髓硬膜外血肿临床特点分析

目的 探讨自发性脊髓硬膜外血肿(SSEH)的发病特点、临床表现及诊治方法. 方法 总结珠江医院骨科自1995年1月至2010年12月收治的12例SSEH患者临床表现、诊治经过及随访情况.12例患者中采用经椎管减压血肿病灶清除术10例,非手术治疗2例.所有患者均随访6-18个月. 结果 12例患者发病前均无明显诱因,发病后均表现为受累肢体肌力丧失或减弱、大小便功能障碍.手术治疗患者中2例术后受累肢体运动、神经功能完全恢复,8例患者除大小便功能略微改善外,肢体的运动感觉功能无恢复.非手术治疗的患者中1例神经功能完全恢复.1例神经功能部分恢复. 结论 SSEH进展迅速,脊髓运动、神经功能障碍是其主要临床表现,及时的手术治疗可有效改善预后,是SSEH首选的治疗方式.     

扩散张量成像技术对急性脑梗死的诊断价值

目的应用MR扩散张量成像(DTI)及扩散张量纤维束成像(DTT)技术对急性脑梗死患者进行检查,研究患者肢体活动障碍的表现及预后与皮质脊髓束的关系及ADC图对急性脑梗死的诊断价值。方法对33例急性脑梗死患者(发病时间<72h)行常规MRI检查及DTI检查,并进行皮质脊髓束三维DTT成像及b=1000的ADC成像,将患者运动障碍的程度分为无瘫痪,治疗后瘫痪恢复,治疗后瘫痪不恢复3种情况,将常规MRI图、DTI中的ADC图影像表现及DTT图皮质脊髓束形态表现与患者临床肌力表现及治疗结果相比较。结果无瘫痪13例,治疗后瘫痪恢复者9例,治疗后瘫痪不恢复者11例,DTT图显示皮质脊髓束分别为无受压,受压无中断及中断破坏。所有病例ADC图均表现为低或稍低信号。结论DTI及DTT技术,对判断脑梗死患者运动障碍的程度及预后有重要价值。     

向脊髓引流的硬脑膜动静脉瘘诊治体会

目的探讨向脊髓表面引流的硬脑膜动静脉瘘（DAVF）的临床特点及治疗。方法回顾性分析2009年2月。2012年10月诊治的5例向脊髓引流DAVF患者的临床资料。4例单纯行栓塞治疗,1例行栓塞后手术切除。结果术后DSA示5例患者的瘘口均完全消失,达到解剖治愈。4例随访6个月-2年,肢体肌力及感觉均有一定恢复,MRI示脊髓表面迂曲血管流空影消失,DsA示瘘口消失、无复发。结论向脊髓引流的DAVF患者脊髓血管造影常显示正常,应注意行脑血管造影,避免漏诊、误诊,治疗主要以血管内栓寨为丰。     

恩经复治疗手足口病合并急性弛缓性麻痹患儿临床分析

目的探讨恩经复治疗儿童手足口病合并急性弛缓性麻痹的临床疗效。方法收集79例手足口病合并急性弛缓性麻痹患儿的临床资料,其中恩经复治疗组49例,对照组30例,回顾性分析2组患者的临床特点,根据诊断学肌力级别为评定标准,记录患肢肌力恢复情况,分为显效、有效、无效。结果 79例手足口病合并急性弛缓性麻痹患儿中,26例合并单下肢瘫痪患儿下肢肌力恢复较好,可达Ⅲ～Ⅳ级,40例合并双侧肢体瘫痪及单上肢瘫痪患儿,肢体肌力分别较前增加Ⅰ～Ⅱ级,7例单侧肢体瘫痪患儿上肢肌力恢复至Ⅱ～Ⅲ级,下肢恢复至Ⅲ～Ⅳ级,6例四肢瘫痪者中,2例双侧上肢肌力恢复至Ⅰ级,双侧下肢肌力恢复至Ⅱ级,4例双上肢恢复至Ⅱ级,双侧下肢肌力恢复至Ⅲ～Ⅳ级。对于HFMD合并AFP患儿,在综合康复治疗的基础上尽早给予恩经复注射治疗肌力恢复明显。结论对于HFMD合并AFP患儿,早期给予恩经复治疗,能够促进受损的神经功能早日恢复,减少瘫痪的发生,降低患儿致残率。     

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.     

Corticospinal involvement in patients with a portosystemic shunt due to liver cirrhosis

Hepatic myelopathy (HM) is a rare complication of chronic liver diseases usually associated with a portosystemic shunt, causing a progressive spastic paraparesis, and is likely to be overlooked. Thirteen patients with liver cirrhosis associated with surgical or spontaneous portosystemic shunts were studied to determine the frequency and gravity of HM. Six patients exhibited clear-cut signs of spinal cord involvement and four of them exhibited varying degrees of disability. Neurological examination did not reveal any abnormalities in the other patients. Motor evoked potentials (MEPs) were measured in all patients; in five of them the examinations were done before and after orthotopic liver transplantation (OLT). The patients with clinical signs of spinal cord involvement exhibited severe neurophysiological abnormalities, whereas milder but unequivocal MEP abnormalities were found in four of the seven patients with normal clinical examination. The clinical and neurophysiological features of patients with slight MEP abnormalities improved after OLT, whereas the patients with a more advanced stage of disease (severe MEPs abnormalities) did not. Our findings indicate that MEP studies may disclose an impairment of the corticospinal pathways even before HM is clinically manifest and provide evidence that early diagnosis of HM and subsequent immediate liver transplantation have to be recommended.     

The role of cardiac disease parameters in predicting the results of Holter monitoring in patients with acute ischaemic stroke

There is limited evidence supporting the routine use of Holter monitoring (HM) in patients with acute ischaemic stroke. This study aimed to assess the diagnostic yield of HM and determine whether any cardiac disease parameter(s) would permit more focused targeting of HM. We performed a retrospective evaluation of HM in patients with acute ischaemic stroke admitted to our hospital over a one-year period to assess diagnostic yield and whether certain cardiac disease parameters were correlated with HM results. The diagnostic yield was 9%, the number needed to screen was 11, and the cost to detect one clinically significant case was AUS$1,300. Apart from age, stratifying patients by cardiac disease parameters did not predict HM result. This strengthens the use of HM in all patients presenting with acute ischaemic stroke of unknown aetiology.     

Systematic review on the efficacy and safety of herbal medicines for Alzheimer's disease

A systematic review was conducted to assess the efficacy and safety of herbal medications (HM), as either monotherapy or adjunct to orthodox medications (cholinesterase inhibitors and nootropic agents, OM) for Alzheimer's disease (AD). Sixteen studies testing different HM were included. Out of the 15 HM monotherapy studies, 13 reported HM to be significantly better than OM or placebo; one reported similar efficacy between HM and OM. Only the HM adjuvant study reported significant efficacy. No major adverse events for HM were reported and HMs were found to reduce the adverse effects arising from OM. Imbalance in ethnicity among participants was observed; gender distribution was unclear. Heterogeneity in diagnostic criteria, interventions and outcome measures hindered comprehensive data analysis. Studies comparing HM with OM suggest that HM can be a safe, effective treatment for AD, either alone or in conjunction with OM. Methodological flaws in the design of the studies, however, limited the extent to which the results could be interpreted. Among various HMs, the safety and tolerability of EGb761 was best established. Further multi-center trials with large sample size, high methodological qualities and standardized HM ingredients are necessary for clinical recommendations on the use of HM in treating AD.     

Spontaneous peritoneal and retroperitoneal hemorrhage, rare serious complication following carotid angioplasty with stent

Hemiplegic migraine (HM) is a rare and severe subtype of migraine with aura, characterized by some degree of hemiparesis and other aura symptoms. Mutations in three genes (CACNA1A, ATP1A2 and SCN1A) have been detected in familial and, more rarely, in sporadic cases. The disease can be complicated by permanent neurological deficits, the most frequent one being a cerebellar syndrome; in addition, mental retardation has been recognized as part of the phenotypic spectrum. Here, we report a Caucasian male with a novel CACNA1A mutation and an unusual clinical phenotype: the patient, who had had a history of only two HM attacks, sought medical advice at age 49 primarily because of increasing cognitive decline accompanied by cerebellar dysfunction. While common neurodegenerative causes were excluded, neuropsychological evaluation revealed a distinct profile of deficits of a subcortico-prefrontal type as previously reported in patients with cerebellar dysfunction. This suggests a possible causal link between cerebellar and cognitive disturbances in this patient; in addition to these pathophysiological aspects, we review of the role of the cerebellum in cognition.     

Sneddon syndrome: cerebral perfusion studies by Tc99m HM-PAO and SPECT

Sneddon syndrome is a non-inflammatory, non-atherosclerotic disease involving small and medium-sized arteries of the brain and of the skin. The arteriographic examination is often negative despite progressive impairment of the neurological status. In 3 patients with Sneddon syndrome cerebral perfusion was assessed with single photon emission computerized tomography (SPECT) and technetium⁹⁹m-hexamethylpropylenamineoxime (Tc⁹⁹ HM PAO). A correlation between clinical and SPECT finding was found, with significant focal reduction of regional cerebral blood flow (rCBF) in the two more severely affected patients.

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Sommario La sindrome di Sneddon è una malattia a carattere non infiammatorio e non aterosclerotico che colpisce le arterie cerebrali e cutanee di piccolo e medio calibro. L'esame angiografico è spesso negativo nonostante il carattere progressivo della malattia. In tre pazienti con diagnosi clinica di malattia di Sneddon è stata studiata la perfusione cerebrale mediante SPECT e tecnezio⁹⁹m-esametilpropilenamineossima (Tc⁹⁹m HM PAO). La SPECT ha dimostrato una migliore correlazione con il quadro clinico rispetto alla tomografia computerizzata, evidenziando riduzioni focali di perfusione nei due casi più gravi.

     

Enhanced Skills Training and Health Care Management for Older Persons with Severe Mental Illness

This report describes a combined skills training (ST) and health management (HM) intervention for older adults with severe mental illness (SMI) and one-year pilot study outcomes. Findings are reported for twelve older persons with SMI (age 60+) who received ST+HM and twelve who received only HM. ST addressed interpersonal and independent living skills. HM included promotion of preventive health care. ST+HM was associated with improved social functioning and independent living skills, whereas functioning remained constant or declined for the HM only group. Both groups receiving HM demonstrated increased use of preventive health services and identification of previously undetected medical disorders.