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1.
One hundred and eight patients with spasticity of the paralytic limbs were treated successfully with hyperselective posterior rhizotomy (SPR). Of the 108 patients, 100 had cerebral palsy, 2 hemiplegia, 3 sequelae of cerebral injury, 2 paraplegia and 1 multiple sclerosis. Twelve patients received cervical SPR and 96 lumbosacral SPR. Laminectomy is performed to open the dura and to separate the posterior spinal root into several rootlets. The lower threshold rootlets were divided after electrical stimulation. Follow-up for 6 to 30 months showed that the effective rate of reducing spasticity was over 95% and functional improvement rate over 80%.
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2.
In 30 patients with intractable generalized epilepsy treated with cerebral commissurotomy, the corpus callosum was stimulated intraoperatively at a 1 cm interval with electric current, and evoked potentials (EPs) were recorded from different areas of the brain for determining the distribution patterns of functional projections from the corpus callosum to the cerebral cortex. The surface of the corpus callosum in man was 12 cm long and it was divided into 12 segments (1 cm each). Stimulation of the first segment resulted in EPs only in the frontal lobe. Stimulation of segments 2-4 produced EPs mainly in the anterior, middle and posterior frontal lobe, anterior and central temporal lobe, rarely in parietal and occipital lobes. Stimulation of segments 5-8 induced EPs mainly in the frontal and temporal lobes, but rarely in the parietal and occipital regions; on stimulation of segments 9-11, EPs occurred only in the parietal and occipital regions. These results were confirmed electrophysiologically in 5 cats and anatomically in 8 cats. These data provide an anatomical basis for selective cerebral commissurotomy in the treatment of intractable epilepsy. Hence in patients with a concentration of epileptic discharges in the frontal lobe, the 4 anterior segments of the corpus callosum should be incised. Epileptic discharges in the frontotemporal region indicates that the middle segments should be incised and epileptic activities originating from the parieto-occipital regions can be treated effectively by selective section of the posterior corpus callosum (segments 9-12).
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3.
Background Spinocerebellar ataxia type 7 (SCA7) is known as an autosomal dominant cerebellar ataxia; patients with genetically confirmed diagnoses of SCA7 have increased rapidly in recent years.However, SCA7 is a rare subtype of SCA, and most data available about SCA7 are those of white people.The aim of the present study was to systematically review the prevalence and clinical and genetic aspects of SCA7 patients in East Asian population.Methods A search for publications on SCA7 was performed by using the "PubMed" database with the published language limited in English.Publications mainly focusing on the prevalence of SCA7 in patients with SCA and the clinical and genetic features of SCA7 patients were fully reviewed and analyzed.Results The prevalence of SCA7 in SCA patients ranged from 0 to 7.7%, which was similar to those reported previously.The clinical manifestations were typically present at the 30's of its victims (median, 29 years; interquartile range (IQR),19.5-36.5 years), and the symptoms appeared 15 years ((15.17±4.22) years) earlier on average in the offspring than in the parents.Gait ataxia and visual impairment were both found in all patients of whom the clinical features were described.Mutant SCA7 alleles contained 40-100 CAG repeats, with a median of 47 repeats (IQR, 44.5-50.0); and the offspring had 13 more repeats on average compared with their parents (12.62±19.03).A strong negative correlation was found between CAG repeat size and the onset age of patients (r=-0.739, P=0.000).In addition, no significant difference was found in CAG repeat sizes between patients with visual impairment as the initial symptom and those with gait disturbance as their initial symptom (P=0.476).Conclusions The prevalence of SCA7 in SCA patients, the age at onset and CAG repeats of SCA7 patients in East Asia are consistent with those of white people.However, larger population study is needed to assess the correlation between the CAG repeat size and initial symptoms of SCA7 patients in East Asia.  相似文献   

4.
Background There are not many studies about treatment of shoulder spasticity. Although botulinum toxin injection has been reported to be effective for shoulder spasticity, the effectiveness was judged by pain and limited motion change, but not the spasticity itself. Shoulder spasticity is considered to play an important role in hemiplegic frozen shoulder. However, the subscapularis muscle, unlike the pectoralis major muscle, is located deep beneath scapula, where conventional injection is difficult to perform. As extracorporeal shock wave therapy (ESWT) has been reported to be effective for spasticity relief, and we thought spasticity of subscapularis muscle located deep beneath the scapula would be a good candidate for ESWT treatment. This study was to evaluate the beneficial effects of radial ESWT (rESWT) on spastic subscapularis muscle in stroke patients. Methods This is an uncontrolled, prospective, unicenter, clinical pilot study. Stroke patients (n=57; mean age 55.4 years) with spastic shoulders were recruited between June 2011 and February 2012 at the University Rehabilitation Hospital. rESWT was administered to each patient every two or three days for two weeks (five total treatments). Evaluation consisted of 11 measurements for each patient; at the start of each of the five treatments and once per week during the following six weeks. Spasticity was measured at external rotator muscles of the shoulder using the modified Ashworth scale (MAS), and passive range of motion (ROM) of the shoulder in external rotation was recorded. Pain was measured using a visual analogue scale (VAS) during passive ROM of the shoulder in external rotation, and was additionally recorded for patients who preserved cognitive and communicative ability (Pain group). Results Reduction in MAS and VAS and improvement of ROM during and after rESWT treatments were prominent compared to baseline. The reduction in MAS and VAS and improvement of ROM continued four weeks after the last treatment and the effects of the treatment decreased afterward. Conclusion rESWT will be able to provide stroke patients with an effective and safe procedure for the reduction of spasticity and pain as well as for the improvement of ROM of spastic shoulders.  相似文献   

5.
Here we review a new variety of leukoencephalopathy with infantile megalencephaly and discrepant clinical course (MLC, MIM: 604004). These children had megalencephaly in the first year of life, with or without mild delay of motor function and/or seizures. After a few years, motor handicap was slowly progressive with unsteady gait, serious cerebeliar ataxia and mild plasticity. Eventually most of patients were confined to a wheelchair. Meanwhile mental development was relatively preserved, although the learning problems was increased from the midway of elementary school. Most of patients had tonic-clonic seizure and some might advance to status epilepticus. Antiepileptic drugs may effectively control seizure. The disorders of known metabolic defects were excluded. Neurophysiological examination showed that EEG had interictal epileptic discharges on the generalized slow wave background in most patients. The cerebral white matter had diffuse abnormality, with swelling of white matter, and cysts in the frontoparietal and anterior-temporal lobes on MRI examination. Some central white matter structures were spared, such as corpus caliosum. The severity of lesions on MRI is inconsistent with the clinical signs. Pathogenesis of this disease was unknown. The pathological findings found a spongiform leukoencephalopathy due to myelin splitting and intramyelinic vacuole formation but without myelin loss, This disease had probably an autosomal recessive inheritance, The geneKIAA027 on 22qtel was responsible for M LC.  相似文献   

6.
Hereditary spastic paraplegia (HSP) (MIM#182600) is a group of heterogeneous neurodegenerative disorders, with 35 underlying loci recognized by the HGNC (HUGO Gene Nomenclature Committee; http://www.gene.ucl.ac.uk/nomenclature/) and 10 identified genes ( http : //www. gene. ucl. ac. uk/cgi-bin/nomenc lature/ searchgenes.pl plus NIPA1, last search July 2006). The mode of inheritance may be autosomal dominant, autosomal recessive or X-linked. Among these, autosomal dominant spastic paraplegia (AD-HSP) is the most common type, accounting for 70%-80% of all families. The disease is characterized by lower limb spasticity, hyperreflexia, progressive spastic gait and an extensor plantar response. Pes cavus is one of the commonly reported foot phenotypes.  相似文献   

7.
Schizencephaly is a neuronal migration malformation characterized by a cleft traversing the cerebral hemisphere. The cleft is lined by heterotopic gray matter, and the lips of the cleft may be fused or separated. Often associated with anomalies of the septum pellucidum or corpus callosum, and polymicrogyria, schizencephaly has characteristic CT findings. Of 17 patients with this lesion detected by CT 2 had bilateral schizencephaly.
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8.
Objective: To study the CT and MR imagirig features of spongiform leukoencephalopathy after heroin vapor inhalation. Methods: The CT and MR imaging features and pathologic findings of 13 patients with heroin-induced spongiform leukoencephalopathy were analyzed. Results: CT scanning and MR] of all the patients showed diffuse,symmetric lesion in the cercbellar and cerebral white matter, and the cerebellum was invariably involved in all cases.Symmetric round Or butterfly-like lesions lateral to the midline of the cerebellum with clear border was the most distinct feature in CT and MRI examination. The lesions were not found in the anterior limbs of the internal capsules. CT scanning showed low-density changes while MRI TIWI imaging presented low-signal and T2WI high-signal lesions without space-occupying mass. The pathologic findings showed spongiform degeneration of the white matter in the central nervous system, but necrotic lesions were not observed. Condusions: Spongiform leukoencephalopathy should be considered when acute, cerebellar signs are present in patients who admitted a history of heroin inhalation. The CT and MRI manifestation of this disease is typical and the diagnosis can thus be made.  相似文献   

9.
Background Hearing impairment has been reported to be common in patients with mitochondrial disorders,a group of diseases characterized by pleiomorphic clinical manifestations due to defects in oxidative phosphorylation of mitochondria.This study aimed to investigate the audiological characteristics in a large cohort of patients with mitochondrial disease.Methods Comprehensive audiological evaluations,including pure tone audiometry,tympanometry,speech audiometry,otoacoustic emissions,electrocochleography and auditory brainstem evoked potentials,were performed in 73 Chinese patients with mitochondrial encephalomyopathy and with confirmed mitochondrial DNA (mtDNA) defects.Results Among the patients,71% had hearing impairment.However,the incidence rate and severity of hearing impairment were much less in the chronic progressive external ophthalmoplegia (CPEO) subtype than in the mitochondrial encephalomyopathy,lactic acidosis,and stroke-like episodes (MELAS),myoclonic epilepsy with ragged red fibers (MERRF) and Kearns-Sayre syndrome (KSS) subtypes.While most of our patients had a predominantly cochlea origin for the hearing deficit,five patients had an auditory neuropathy spectrum disorder and three patients had impairment of both cochlea and auditory codex.Conclusions Various portions of the auditory system could be involved in patients with mitochondrial diseases,including cochlea,auditory nerve,auditory pathway and cortex.Hearing loss was more associated with multisystem involvement.Genotype,mutant load of mtDNA and other unknown factors could contribute to heterogeneity of hearing impairment in mitochondrial disease.  相似文献   

10.
Objective: To observe the clinical characteristics of Costello syndrome. Methods:the clinical characteristics and gene mutation types of one child with Costello syndrome were analyzed. The key words of Costello syndrome, Costello syndrome and HRAS were searched and analyzed. Results: all the children showed some special facial features, growth retardation and heterozygous mutation of HRAS gene. After diagnosis, all patients were treated with growth hormone and medical treatment. It was found that the clinical characteristics of Costello syndrome reported at home and abroad were similar to the child in our case. The main clinical manifestations of Costello syndrome were special features, heart system disease, short stature and mental retardation, with tumor susceptibility. Conclusion: the incidence rate of Costello syndrome is extremely low, involving multiple system diseases. Special signs and HRAS gene sequencing are helpful for the diagnosis of the disease.  相似文献   

11.
目的探讨胼胝体发育不良的的磁共振影像表现及其临床价值。方法回顾性分析132例胼胝体发育不良的磁共振影像及临床资料。结果总结所有入组病例的MR图像征象,单纯胼胝体发育不良者40例,其中胼胝体完全未发育者2例,部分未发育者13例,胼胝体发育不良者11例,胼胝体部分发育不良者14例。单纯性胼胝体发育异常主要表现为胼胝体部分或完全发育不良,变薄,或者胼胝体部分或完全未发育。胼胝体发育异常伴其他脑发育异常者92例。除了单纯性胼胝体的结构或形态异常,还伴有包括侧脑室增大、三脑室扩大上移,脑积水,透明隔缺如或透明隔间腔,脑白质发育不良,灰白质异位、脑裂畸形、脑回发育畸形、颅骨发育畸形,先天性表皮样囊肿及脑肿瘤等。临床上,部分患儿无明显的症状和体征,重者主要表现为不同程度发育迟缓、癫痫。单纯性胼胝体发育异常的临床症状较伴有其他脑发育异常的轻。结论 MRI能够很好的显示胼胝体发育异常,并能对伴随的其他脑发育异常做出全面的评估。  相似文献   

12.
选择性脊神经后根切断术对大脑功能的影响   总被引:5,自引:0,他引:5  
目的:探讨选择性脊神经后根切断术(SPR)治疗脑瘫下肢痉挛时对脑瘫患者大脑功能的影响及其机制。方法:对1990年5月-1998年4月间采用腰骶段选择性脊神经后根切断术治疗的痉挛型脑瘫患者进行随访,并对得到2年以上随访的825例病例进行上肢痉挛、癫痫、斜视、流涎及语言发音障碍等情况调查分析。结果:伴有上肢痉挛者328例,其中67例(20.4%)上肢痉挛减轻;伴有癫痫者35例,其中31例(88.6%)术后癫痫发作频率减少或癫痫控制药物服用量减少;伴有斜视者386例,其中132例(34.2%)减轻;伴有流涎者73例,其中49例(67.1%)流涎减少,21例(28.8%)流涎消失;伴有语言发音障碍者456例,其中72例(15.8%)发音功能改善。结论:腰骶段选择性脊神经后根切断减少外周神经传入冲动,从而降低了中枢的兴奋性,影响大脑高级中枢的调控作用;此外周-皮层-外周大环路反射可能是SPR解痉的另一机制。  相似文献   

13.
14.
The clinical features of 16 cases of tumours of the corpus callosum are described. Many presented with mental changes which were eventually obvious in all but one of the patients. The need to consider these tumours in the differential diagnosis of dementia is discussed.  相似文献   

15.
巨颅伴皮层下海绵样囊肿性脑白质病是近年来被认识的一种新的儿童脑白质病。这种脑白质病患者出生时头围增大 ,神经发育基本正常或接近正常。随后逐渐出现缓慢进展性运动功能恶化如走路不稳、共济失调、肢体痉挛和瘫痪 ,而同时智能损伤相对较轻。大多数患者可有惊厥发作但对抗惊厥治疗反应良好。除脑电图上可有痫样放电外其他神经电生理检查基本正常。在影像学上所有患儿的脑白质均受累 ,早期患者MRI上表现出脑组织水肿 ,特别是额叶以及颞叶和顶叶的前部十分明显 ,后期脑皮层下出现囊肿样变化。病理学显示这些囊肿样结构主要是由单一五层膜结构所构成的髓鞘板层覆盖的空泡。目前已知的、常见的遗传代谢物筛查在该病患者中无阳性发现。该病是一种常染色体隐性遗传病 ,位于 2 2qtel上的KIAA0 0 2 7基因被认为是该脑白质病的致病基因  相似文献   

16.
目的 探讨脑性瘫痪(CP)患儿胼胝体面积与神经运动发育之间的关系.方法 选取脑性瘫痪患儿63例作为研究组,正常儿童60例作为对照组,应用《0~6岁儿童神经心理发育量表》评价脑性瘫痪患儿神经运动发育.对受试儿童进行头颅MRI检查,正中矢状位测量胼胝体总面积及分区面积,分析胼胝体面积与神经运动发育之间的关系.结果 研究组胼胝体总面积及分区面积小于对照组,差异具有统计学意义;研究组胼胝体总面积与神经运动发育评分存在正相关性,其中粗大运动及适应能力相关明显.结论 脑性瘫痪患儿胼胝体的发育受影响,患儿临床症状越重,胼胝体面积越小,经头颅MRI对其检测可评估脑性瘫痪病情的轻重及治疗效果.  相似文献   

17.
《中医杂志(英文版)》2014,34(5):609-615
ObjectiveTo determine the characteristics and advantages of acupoint-to-acupoint penetrative needling (AAPN) treatment for post-stroke spastic paralysis (PSSP) to improve the clinical outcomes of this disease in the future.MethodsRandomized, controlled trials of PSSP patients receiving AAPN treatment were searched from MEDLINE, EMBASE, and China National Knowledge Infrastructure Database between January 2006 and June 2013. Key words included: clinic or clinical, acupuncture, needling, acupoint-to-acupoint, penetrative or penetration or penetrating, stroke or apoplexy or cerebral infarction or cerebral hemorrhage, spastic paralysis or spasticity or palsy, and hypermyotonia. Language was limited to English and Chinese. Case series reports, review articles, and animal studies were excluded.ResultsAAPN showed better clinical results on PSSP than other acupuncture treatments, especially when combined with adjunct therapies such as electroacupuncture, bloodletting, and rehabilitation. The greatest benefit was achieved with rehabilitation combined with penetration from Yang-channel acupoints to Yin-channel acupoints in the upper limbs, and from Yin-channel acupoints to Yang-channel acupoints in the lower limbs with a reinforcing maneuver.ConclusionAAPN is an effective treatment for PSSP, and it can accelerate and enhance functional repair of PSSP patients.  相似文献   

18.
首发精神分裂症患者的胼胝体及小脑中脚MR扩散张量研究   总被引:1,自引:0,他引:1  
目的 探讨磁共振扩散张量成像(DTI)对首发精神分裂症(SP)患者胼胝体和双侧小脑中脚细微异常的诊断价值.方法 首发SP患者组20人,正常组18人,均行DTI检查,测量胼胝体膝部、压部.双侧小脑中脚的局部各向异性(FA)、平均扩散参数(ADC)值.所测得数值进行两独立样本t检验.结果 首发SP患者双侧小脑中脚的FA值低于正常对照组(P<0.05),而胼胝体膝部、压部的FA值无统计学差异(P>0.05),两组胼胝体膝部、压部.双侧小脑中脚ADC值无统计学差异(P>0.05).结论 小脑与大脑神经纤维联系的细微异常,可能是引起SP早期症状的主要原因.  相似文献   

19.
目的:探讨低场MRI中胼胝体梗塞的影像学表现及其临床应用价值。方法:对28例胼胝体梗塞患者MRI图像进行分析,并与临床治疗相结合。结果:胼胝体膝部梗塞9例、胼胝体干部梗塞7例、胼胝体压部梗塞15例;其中早期梗塞5例,陈旧性梗塞23例。结论:低场MRI对诊断胼胝体梗塞很有价值,可以清晰显示梗塞的部位、范围、形态,并能对梗塞新旧分期,指导临床治疗。  相似文献   

20.
目的;分析苯丙酮尿症/颅脑MRI异常表现,方法:用0.5T超导磁共振机对临床检查和生化测定确诊的73例苯丙酮尿症患儿行头颅扫描。结果:MRI发现异常71例。T2WI上脑白质内散在斑片状高信号灶最为常见(69人次);沿胼胝体向明后延伸并向双侧放射冠和半卵圆中心白质对称性扩展的弥漫性高信号病灶在T2WI上也很常见(51人次);MRI玩赏发现无胼胝体(3人次)、胼胝体发育不良(17人次)、无透明隔(2人  相似文献   

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