55例脑卒中患者的康复治疗观察

为了探讨康复治疗对脑卒中患者功能预后的影响，笔者对55例脑卒中患者进行了康复训练观察，现将结果报道如下。     

他汀类药物治疗出血性脑卒中研究进展

出血性脑卒中是脑卒中的一种，该病发生率比缺血性脑卒中低，但该病发生往往更为凶险，患者功能转归更差，病死率与致残率均更高。故而出血性脑卒中发生后，需引起重视，及时给予患者有效治疗。出血性脑卒中患者多患有脑动脉硬化、高血压，主要症状为意识障碍、呕吐、头痛、感觉障碍、偏瘫等，又称之为脑淤血。出血性脑卒中多以药物治疗，而他汀类药物为临床一线降血脂药，该药对脑卒中患者神经具有一定保护作用，而多项研究指出他汀类药物可较好改善缺血性脑卒中患者病情，对患者预后具有较为积极影响。而对于出血性脑卒中患者，他汀类药物治疗相关报道较少。目前，关于他汀类药物对出血性脑卒中患者预后影响仍存在较大争议，患者在住院期间是否可应用他汀类药物进行临床治疗至今仍然无法确定。故而本文对出血性脑卒中疾病病因及临床表现进行总结分析，综述了他汀类药物的分类与作用机制，以探究他汀类药物对出血性脑卒中患者的影响，为出血性脑卒中的临床治疗提供参考。     

急性脑卒中患者肺部感染的预后及影响因素研究

目的探讨合并肺部感染的急性脑卒中患者的预后,并分析影响预后的因素。方法选取2014年8月-2016年5月132例急性脑卒中合并肺部感染患者为研究对象,根据预后情况分为预后良好组与预后不佳组,筛选出影响预后的相关因素,应用logistic回归分析进行多因素回归分析。结果 132例脑卒中合并肺部感染患者中有24例(18.2%)患者预后不佳,包括死亡7例(5.3%),108例(81.8%)预后良好;出院时预后良好组NIHSS评分明显低于预后不佳组,差异有统计学意义(P<0.05);预后良好组住院时间明显短于预后不佳组,差异有统计学意义(P<0.05);脑卒中类型、意识障碍、吞咽困难、糖尿病史、冠心病史、年龄及卧床时间与患者预后存在相关性,差异有统计学意义(P<0.05);logistics回归分析显示,出血性脑卒中、存在意识障碍、既往糖尿病史及高龄是影响患者预后的危险因素(P<0.05)。结论临床上对于出血性脑卒中、存在意识障碍、既往糖尿病史及高龄的急性脑卒中患者,需早期预防,不仅避免肺部感染的发生,且可提高急性脑卒中合并肺部感染的预后。     

老年脑卒中与青年脑卒中病因及其临床预后的临床观察

目的:通过对老年脑卒中与青年脑卒中病因及其临床预后进行临床观察,对这两个不同年龄层次的患者的脑卒中病情进行分析,讨论解释其患脑卒中病症的原因,从而为我国各大医院的脑卒中病情治疗提供理论依据与实际操作方案,促进脑卒中病情治疗医学领域的发展。方法:选取自2015年度至2016年度入住我院的70例脑卒中患者,将20至50岁的患者定为青年脑卒中患者,将50至75岁的患者定为老年脑卒中患者.其中青年脑卒中患者为35例,老年脑卒中患者为35例,对这两组患者患脑卒中病症的原料以及临床预后进行临床观察。结果:老年脑卒中患者的发病原因则大多是由所患的高血脂,高血压等病症引起的。结论:老年脑卒中与青年脑卒中的病因有所差异,且经过临床预后,青年患者比之老年患者更容易恢复病发前的健康状态。     

急性缺血性脑卒中患者人院时血尿酸水平与预后分析

目的 探讨急性缺血性脑卒中患者入院时血尿酸水平与其预后的关系。方法 58例急性缺血性脑卒中患者，根据其入院时（起病〈24h）检测的血尿酸水平分为两组：高尿酸组及正常尿酸组。回顾性分析急性缺血性脑卒中患者起病初尿酸水平与其病灶范围及预后的关系。结果高尿酸组梗死灶直径〉2cm者居多（P〈0．05），治疗好转率明显低于正常尿酸组（P〈0．05），病死率高于正常尿酸组，但差异无统计学意义。结论 急性缺血性脑卒中患者入院时血尿酸水平可作为评估预后的参考指标之一。     

制约脑卒中二级预防作用有效发挥的因素分析及对策

目的分析制约脑卒中二级预防作用有效发挥的因素,并探讨脑卒中的二级预防对策。方法收集脑卒中患者320例,按照预后效果分为预后良好组与预后不良组,统计对比影响脑卒中二级预防作用的影响因素。结果性别、高龄、营养摄入过多、不良生活习惯、康复工作水平以及入院时体温均为影响脑卒中二级预防的危险因素。结论针对脑卒中发病危险因素制定切实可行的二级预防措施,加强对患者的健康教育,提高康复护理质量及二级预防效果,可有效降低脑卒中对患者的影响。     

早期康复护理对脑卒中吞咽障碍患者的影响

目的:探讨早期康复护理对脑卒中吞咽障碍患者的影响.方法:选取我院2015年2月至2016年11月收治的40例脑卒中吞咽障碍患者作为研究对象,随机分为对照组和训练组,对照组进行常规护理,训练组在常规护理基础上施行吞咽训练为中心的康复护理,比较两组患者的护理效果.结果:治疗前两组各项指标无差异,训练组治疗6周后患者吞咽功能康复优于对照组(P<0.05).结论:脑卒中导致吞咽障碍患者给予早期康复护理干预可以使患者提高代偿性进食技巧,最终恢复患者的吞咽功能,预防营养障碍及肺炎等并发症的发生.     

青年脑卒中预后影响因素分析

目的了解青年脑卒中预后的影响因素,为改善其预后,提高生活质量提供依据。方法通过抄录医院病历记载信息及电话随访收集保定市发病年龄在18～45岁的青年脑卒中患者的资料,采用Rankin(modifiedrankin scale,mRS)量表对患者的预后进行评定,利用2检验进行单因素分析,Logistic回归进行多因素分析。结果共调查80例,男性53例占66.25%,女性27例占33.75%。预后良好者43例占53.75%,预后不良者37例占46.25%。病死7例,病死率为8.75%。单因素分析表明,文化程度低、入院首次收缩压高的患者预后较差,入院诊断出血性脑卒中的患者预后比缺血性患者差。多因素分析结果表明入院诊断、人均月收入影响患者的预后,出血性脑卒中、经济收入较低的患者预后更差。结论对于出血性脑卒中患者应尽早介入康复治疗,加强对低收入、低文化程度青年脑卒中患者的关注,采取有效措施改善预后。     

站立重心偏移对脑卒中偏瘫患者功能预后的临床探讨

研究站立时重心的偏移对脑卒中偏瘫患者功能预后的影响。46例脑卒中偏瘫患者根据站立时重心的偏移情况分为重心偏向健侧组（即非瘫痪侧组，简称健侧组）和重心偏向患侧组（即瘫痪侧组，简称患侧组），对两组患者的平稳能力、步行能力、日常生活能力和住院时间进行评测。健侧组的平衡能力、步行能力和日常生活能力明显优于患侧组；健侧组住院时间明显比患侧组短（P〈0．001），说明健侧组比患侧组的功能恢复快。结论患者站立时重心的偏移与脑卒中偏瘫患者功能预后密切相关，可作为功能恢复预后的一个重要指标。     

营养不良与脑卒中

营养障碍与脑卒中的不良结局与病死率显著相关;而脑卒中又加重病人营养不良的发生率。高龄和吞咽功能障碍是脑卒中后营养不良的独立危险因素。脑卒中后肠内营养(EN)和肠外营养(PN)相结合的序贯营养支持能减少或避免其并发症。脑卒中(特别是重症)病人应该营养支持。     

高峰发病年龄女性外阴营养不良发生的相关因素研究

目的:研究高峰发病年龄女性外阴营养不良发生的相关危险因素。方法:以40～60岁之间经病理活检确诊的外阴营养不良患者87例作为病例组,以非外阴营养不良患者98例为对照组,分析外阴营养不良的发生与工作生活环境、饮食习惯、卫生习惯、疾病情况、妇女月经史及生育史等因素之间关系。结果:过敏体质、饮食习惯中偏咸辣烫、生活习惯中喜久坐、洗衣粉洗涤内裤及白带色黄等可能是外阴营养不良发生的危险因素(OR>1),而个性开朗、饮食偏素和喜食猪肝等可能是外阴营养不良的保护性因素(OR<1)。结论:过敏体质、不合理饮食习惯、卫生生活习惯及反复阴道炎等与女性外阴营养不良的发生关系较大。     

From gene to disease: from the ABCA4 gene to Stargardt disease,cone-rod dystrophy and retinitis pigmentosa

Autosomal recessive Stargardt disease is caused by mutations in the ABCA4 gene. Mutations in ABCA4 are also found in two-thirds of cases with autosomal recessive cone-rod dystrophy, and a small fraction of patients with autosomal recessive retinitis pigmentosa. Patients with autosomal recessive retinitis pigmentosa, the most severe of these three phenotypes, invariably carry ABCA4 inactivating mutations; patients with autosomal recessive cone-rod dystrophy and Stargardt disease carry combinations of mutations that do not completely inactivate the retina specific 'ATP-binding cassette transporter' (ABCR) protein. DNA diagnostics is complicated by the high allelic heterogeneity and the uncertainty as to whether some ABCA4 variants are pathological. Nevertheless, ABCA4 mutation analysis is particularly important for patients with cone-rod dystrophy to confirm the autosomal recessive mode of inheritance.     

Dystrophia myotonica and pregnancy]

We describe the maternal and neonatal complications of pregnancy in two patients with myotonic dystrophy. The disease leads to an increased spontaneous abortion rate, hydramnios, prolonged first and second stages of labour, retained placenta, postpartum haemorrhages and anaesthetic sensitivity in the mother. The neonatal problems are caused by the congenital form of the disease. The major clinical features of congenital myotonic dystrophy are bilateral facial weakness, hypotonia, neonatal distress, feeding difficulties, talipes, tent-shaped mouth, mental retardation and delayed motor development. Relatives of a known myotonic dystrophy patient should be advised to let themselves be examined for this disease. If the disease is diagnosed, information should be given regarding possibilities for prenatal diagnosis. Pregnancy in myotonic dystrophy patients should be monitored by a gynaecologist. Labour has to take place in a hospital with intensive care facilities for mother and child.     

The clinical symptoms in post-traumatic dystrophy

The clinical signs and symptoms in 441 patients with post-traumatic dystrophy were recorded. This form of dystrophy occurred three times as often in females as in males and twice as often in the arms as in the legs. In 5% of the patients the first symptom is a cold extremity; these patients run a greater risk of major sequelae. Exacerbation of the symptoms under influence of muscular exercise proved to be an important prognostic factor. A large proportion of the referred patients proved to have undergone a treatment aimed at elimination of the sympathetic nervous system, without success. Post-traumatic dystrophy can impede all structures and functions of the affected extremity and may result in major sequelae and disablement. The pseudoparalysis of the later phase may be mistaken for conversion.     

General symptoms of cornea Salzmann-type degeneration

After a brief review of the symptoms of Salzmann's nodular corneal dystrophy the authors report on 28 patients. As found by radiological examination, arthrosis deformans was present in all of the patients and, in nearly all cases, osteoporosis has been diagnosed. Histological examination revealed a regressive change in 3 patients' costal cartilage of identical macular character. 22 patients had cataracta and 20 an impaired hearing. Therefore, the disease belongs to the keratochondrosises. Conclusions: 1. The regressive change of the corneal stroma, the hyaline cartilage and the bones relies assumably on an abnormality of their mutual component, the mucopolysaccharides. 2. Experimental and therapeutic experiences have shown that prolonged mucopolysaccharide administration (heparin) will induce osteoporosis. This explains the relationship between osteoporosis and metabolic disorder of mucopolysaccharides. 3. The remarkably high occurrence of cataracta, in comparison to a normal population of the same age, is presumably related to the metabolic disorder of mucopolysaccharides. 4. The aforesaid seem to confirm that Salzmann's nodular corneal dystrophy, corneal dystrophy is not induced by repeated inflammations but conversely, pathological metabolism of the cornea in consequence of dystrophy is responsible for serial keratitis.     

Treatment of posttraumatic dystrophy

Reflex sympathetic dystrophy, posttraumatic dystrophy or complex regional pain syndrome is a particular type of chronic pain. Although the origin is unknown, some believe that wide-dynamic range neurons located in the dorsal horn of the spinal cord play an essential role. Others consider the role of psychogenic factors underestimated. Until now, controlled clinical trials mainly directed at modulation of the sympathetic system have not revealed clearly effective therapies. Tests with guanethidine, phenylephrine, phentolamine or lidocaine have essentially been negative. Use of low dose glucocorticoids, dimethylsulphoxide, biphosphonates and epidurally applied clonidine require confirmation in studies of larger size. There are indications that invasive electrical spinal cord stimulation may have some effect; randomized studies in patients with posttraumatic dystrophy are needed. So far, only application of physical therapy at an early stage has clearly shown effective pain relief and would also lead to cost reduction.     

18 years experience with mechanical ventilation in patients with Duchenne muscular dystrophy

OBJECTIVE: To find out which patients with Duchenne muscular dystrophy are eligible for starting home mechanical ventilation and what the survival rate is. DESIGN: Retrospective. METHOD: In 48 patients with Duchenne muscular dystrophy who were treated with home ventilation from 1987, the results were assessed in the follow-up visit in February 2005. Initially, ventilation was only given through a tracheotomy (TPPV), but after starting up a multidisciplinary neuromuscular consultation, non-invasive ventilation (NIPPV) was offered in an earlier stage of the disease. The following data were derived from the outpatient medical record: indication for ventilation, vital capacity (VC), arterial blood gas values, duration of ventilation up to February 2005, survival and causes of death. RESULTS: 15 patients died. The 5-year survival rate was 75% from the start of mechanical ventilation and 67% (18/27) of the patients were still living at home at the time of the follow-up visit. The most important causes of death were cardiomyopathy (5/15) and tracheal bleeding (3/15). The group of patients who started ventilation before 1995 (n = 17) had a significantly smaller VC than the group (n = 31) who started after the neuromuscular consultation was set up. The PaCO2 during daytime was significantly higher in the group that started ventilation before 1995 compared to the group that started later. CONCLUSION: Home mechanical ventilation can be implemented effectively in patients with Duchenne dystrophy, with a 5-year survival of 75%.     

Diagnosis and therapy of myotonic dystrophy in our practice of neuromuscular care

INTRODUCTION: Myotonic dystrophy is the most frequent, autosomal dominantly inherited muscular dystrophy. The typical neurological picture (facial myopathy, myotonia, muscle atrophy) may be associated with cardial, endocrine and ocular symptoms. METHODS: The diagnosis is based on electromyography, muscle biopsy and genetical tests. Muscle histology is characterized by high frequency of central nuclei. Genetical tests detect CTG repeat expansion of the involved gene. AIMS: Authors summarize 9 cases found in the Neurology Clinic of Pecs University in the last three years. RESULTS: The prevalence is lower than expected, therefore some cases might be unrecognized. After recognizing the typical clinical picture, electrophysiological, muscle biopsy, brain MRI, psychologic and molecular genetic studies were performed. Six patients belonged to 3 families and 3 sporadic cases were found. In all except one patient mild neurocognitive deficit was detected. Three patients had cataract and cardiac involvement. CONCLUSIONS: The authors emphasize that in cases of cardial, endocrine and central nervous system involvement myotonic dystrophy must be considered and detailed examinations are necessary for early detection of the specific organ manifestations.     

Three novel serum biomarkers,miR-1, miR-133a,and miR-206 for Limb-girdle muscular dystrophy,Facioscapulohumeral muscular dystrophy,and Becker muscular dystrophy

Objectives

Muscular dystrophies are a clinically and genetically heterogeneous group of inherited myogenic disorders. In clinical tests for these diseases, creatine kinase (CK) is generally used as diagnostic blood-based biomarker. However, because CK levels can be altered by various other factors, such as vigorous exercise, etc., false positive is observed. Therefore, three microRNAs (miRNAs), miR-1, miR-133a, and miR-206, were previously reported as alternative biomarkers for duchenne muscular dystrophy (DMD). However, no alternative biomarkers have been established for the other muscular dystrophies.

Methods

We, therefore, evaluated whether these miR-1, miR-133a, and miR-206 can be used as powerful biomarkers using the serum from muscular dystrophy patients including DMD, myotonic dystrophy 1 (DM1), limb-girdle muscular dystrophy (LGMD), facioscapulohumeral muscular dystrophy (FSHD), becker muscular dystrophy (BMD), and distal myopathy with rimmed vacuoles (DMRV) by qualitative polymerase chain reaction (PCR) amplification assay.

Results

Statistical analysis indicated that all these miRNA levels in serum represented no significant differences between all muscle disorders examined in this study and controls by Bonferroni correction. However, some of these indicated significant differences without correction for testing multiple diseases (P < 0.05). The median values of miR-1 levels in the serum of patients with LGMD, FSHD, and BMD were approximately 5.5, 3.3 and 1.7 compared to that in controls, 0.68, respectively. Similarly, those of miR-133a and miR-206 levels in the serum of BMD patients were about 2.5 and 2.1 compared to those in controls, 1.03 and 1.32, respectively.

Conclusions

Taken together, our data demonstrate that levels of miR-1, miR-133a, and miR-206 in serum of BMD and miR-1 in sera of LGMD and FSHD patients showed no significant differences compared with those of controls by Bonferroni correction. However, the results might need increase in sample sizes to evaluate these three miRNAs as variable biomarkers.

Electronic supplementary material

The online version of this article (doi:10.1007/s12199-014-0405-7) contains supplementary material, which is available to authorized users.     

Protein and energy metabolism in patients with progressive muscular dystrophy.

Studies were made on whether body weight loss in patients with muscular dystrophy is due to reduced intake and/or abnormal expenditure of energy. For this, food intakes and various physiological variables were surveyed in totals of 310 patients with Duchenne muscular dystrophy (DMD) of 11 to 29 years old and 28 patients with limb-girdle muscular dystrophy (LGMD) of 30 to 47 years old. Energy and protein intakes, expressed on a unit body weight basis, in DMD patients were comparable to, or higher than the allowances for age-matched healthy controls, whereas those in LGMD patients were 92 and 94% respectively of these allowances. The basal metabolic rate (BMR), expressed as kcal/kg/day, of DMD patients of all ages was higher than that of controls, the difference increasing with age, and being about 20 to 30% higher than that of controls in older patients with DMD. The BMR of LGMD patients was nearly normal. The maintenance requirements of conventional dietary protein in DMD and LGMD patients were 1.26 and 0.84 g/kg/day, respectively. These values were about 68 and 12% higher than the normal adult value (0.75 g/kg/day), indicating decreased protein utilization and increased protein catabolism. Daily excretion of urinary 3-methylhistidine (3MH) per unit muscle mass (micrograms/mg creatinine) by MD patients was significantly higher than that by controls, indicating increased degradation of muscle protein. The BMR, maintenance protein requirement and 3MH excretion of DMD patients suggest that DMD is a hypercatabolic disease. Comparison of the energy and protein intakes with the allowances estimated in consideration of increased requirements showed deficiencies of energy and protein in DMD patients. Thus, we conclude that the underweight of the DMD patients resulted from nutrient deficiencies due to hypercatabolism, despite their considerably high intakes of energy and protein, expressed as per kg body weight. These deficiencies were confirmed by demonstrating decreased concentrations of free essential amino acids, particularly branched chain amino acids, in their serum. The values of variables of LGMD patients were intermediate between those of DMD patients and control subjects.