Shy—Drager综合征四例误诊分析

Shy-Drager综合征起病隐袭,早期不易发现,其误诊率可高达87.5％。本文报道4例长期误诊病例,供临床借鉴。 例1 男,54岁。8年前出现阳萎,3年前尿频、尿急和排尿不畅,并渐感两下肢无力及站立时头晕。曾在某医院诊为。前列腺肥大”,前后两次手术治疗,症状     

Shy-Drager综合征12例临床分析

目的探讨Shy-Drager综合征的临床表现和影像学特点,以便早期诊断并改善患者预后。方法回顾性分析经临床诊断的12例Shy-Drager综合征患者的相关资料。结果Shy-Drager综合征患者临床以小便障碍、直立性低血压、性功能障碍、共济失调最常见。8例患者头颅磁共振检查结果示脑萎缩,主要为小脑和脑干萎缩。结论Shy-Drager综合征临床以自主神经功能障碍为主要表现,合并小脑症状发生率高,头颅磁共振可表现为小脑、脑干萎缩。     

Shy—Drager综合征12例临床分析

目的探讨Shy—Drager综合征的临床表现和影像学特点，以便早期诊断并改善患者预后。方法回顾性分析经临床诊断的12例Shy-Drager综合征患者的相关资料。结果Shy-Drager综合征患者临床以小便障碍、直立性低血压、性功能障碍、共济失调最常见。8例患者头颅磁共振检查结果示脑萎缩，主要为小脑和脑干萎缩。结论Shy—Drager综合征临床以自主神经功能障碍为主要表现，合并小脑症状发生率高，头颅磁共振可表现为小脑、脑干萎缩。     

脑干梗死与眩晕关系的探讨

目的 探讨脑干梗死所致眩晕症状的临床特点、发生率及诊断注意事项。方法 对46例以眩晕为首发症状及主要症状的急性脑干梗死的临床表现进行回顾性分析。结果 脑干梗死均以眩晕发作起病，旋转性眩晕84．78％，非旋转性眩晕15．22％。结论 脑干梗死临床表现复杂。眩晕症状突出，发生率100％。行MRI检查，以防漏诊或误诊。     

Shy-Drager综合征16例临床分析

Shy-Drager综合征少见。本文报告16例临床资料。男12例，女4例，以47～60岁居多（14例）。都有典型的小脑症状（眼震、构音障碍和共济失调）、体位性低血压及排尿淋漓及男性阳萎植物神经功能障碍。部分尚有锥体外系和锥体束征。CT／MRI扫描显示小脑和脑干萎缩。本症缺乏有效的治疗方法，合理提高血压可预防体位性低血压晕厥。     

Shy-Drager综合征16例临床分析

目的 探讨Shy-Drager综合征(SDS)的临床特点.方法 对16例SDS患者的临床资料进行回顾分析.结果 16例均有发作性晕厥,立位血压明显下降,而心率变化不大;大部分有排尿障碍、性功能下降、锥体系、锥体外系及小脑损害,卧位休息多能缓解;本组早期均误诊,误诊病种多样.本组随访8例预后不良.结论 自主神经功能异常是SDS的临床特点,卧立位血压测定是避免早期误诊的主要措施.     

Shy-Dragcr综合征的临床与MRI

目的:为了探讨Shy-Drager综合征(SDS)的临床特点及MRI表现。方法:本文对8例SDS进行详细分析,并结合文献分析了SDS主要临床症状、体征及MRI。结果:头晕或晕厥是SDS的最常见症状,所有SDS病例均有植物神经系统异常,锥体系及锥体外系异常率分别为54.4％和59.7％。结论:虽然SDS的MRI缺乏特异性改变,但MRI在SDS的诊断中亦有很大价值。提示中老年人长期发作性头晕或晕厥要想到本病,并及时行头颅MRI检查。     

肌萎缩侧索硬化症的院前误诊分析

目的 回顾分析115例肌萎缩侧索硬化症患者院前误诊情况，加深对该病临床特点的认识，减少误诊，实现尽早诊断和尽早治疗，方法收集北京大学第三医院2003年1月-2005年3月全部诊断为运动神经元病住院患行的临床资料，按照肌萎缩侧索硬化症的诊断标准（修订版）对所有患者进行严格诊断，将其中“确诊为肌萎缩侧索硬化症”和“很可能肌萎缩侧索硬化症”（包括很可能和实验室支持的很可能）115例患者作为观察对象。结果 115例患者中符合“确诊”标准者74例，“很可能”标准41例；平均诊断间期为14．80个月，平均误诊间期6．40个月。其中误诊者72例，未误诊28例，余15例为我院首诊患者，无一例误诊，院前误诊率为72．00％（72／100）。“确诊”和“很可能”患者的误诊率分别为72．31％和71．43％，二者相比差异显著件意义（P〉0．05）：症状首发部位分别为球部合并上肢（5例）、单侧上肢（49例）、单侧下肢（15例）以及偏侧上下肢者（2例），共71例，其误诊率达80．28％（57／71）。在误诊病种中，以颈椎病最为多见，其次为脑血管疾病：北京市与外埠患者的误诊率分别为50．00％（13／26）和79．73％（59／74），二者相比差异具有显著性意义（P〈0．05）。80％以上误诊发生于基层医院，最终明确诊断局限于教学医院。结论 引起肌萎缩侧索硬化症误诊的原因除疾病本身具有临床较少见的特点外，医生对其认识不足亦是发生误诊的重要因素之一，故提高医生对该病的了解并加强专业修养，是降低肌萎缩侧索硬化症误诊率的有效方法和必要手段。     

以癫痫 精神异常为首发表现的糖尿病17例临床误诊分析

目的：探索以癫痫和精神异常为首发的糖尿病临床特点、误诊原因及诊断体会。方法我院2002-02-2012-02收治的各种类型癫痫76例临床资料，其中17例以癫痫样发作和精神异常为首发，完善相关医技检查后，确诊为糖尿病性癫痫、糖尿病性精神病。结果首诊误诊率为22.4％，分别被误诊为癫痫15例，精神病2例。结论临床医生应提高对以癫痫样、精神病样发作为首发的糖尿病认识，当遇到类似症状患者除想到癫痫精神病外，还应想到糖尿病的可能，及时选做尿糖、血糖监测，并给予胰岛素等降糖药治疗，可明确诊断。     

1例前庭阵发症临床诊治分析

目的报告1例前庭阵发症患者的诊治过程,并对国内外相关文献进行复习,从而提高对该病的诊治能力。方法对1例前庭阵发症患者的临床资料及国内外文献进行分析和总结。结果以"阵发性头晕7 d,再发2 h"为主诉的82岁女性患者,结合其临床症状、体征、影像学检查及治疗特点,诊断为前庭阵发症,随访未再发作。结论临床上遇到反复发作性头晕,常规治疗效果不明显且病情反复又排除其他相关疾病时,应考虑前庭阵发症可能,建议试验性应用抗癫痫药物治疗改善症状、明确诊断。     

Micturitional Disturbance in Multiple System Atrophy

Abstract: Detailed micturitional histories and urodynamic studies were conducted to investigate the micturitional disturbance in multiple system atrophy (MSA). Eighty-six patients with MSA comprised of 14 with striatonigral degeneration (SND), 42 with olivopontocerebellar atrophy (OPCA) and 30 with Shy-Drager syndrome (SDS). The results were as follows. Micturitional symptoms were noted in over 90% of patients with each type of MSA. Dominant symptoms were irritative ones in SND and OPCA, and a combination of irritative and obstructive ones in SDS. Micturitional symptoms in SDS appeared earlier than those in SND or OPCA. The degree of micturitional disturbance was severer in SDS than in SND or OPCA. Micturitional disturbance tended to become worse as the disease progressed. The responsible sites of lesions of micturitional disturbance seemed to be supra- as well as infranuclear lesions of the pelvic and pudendal nerves in MSA. Infranuclear lesions were more prominent in SDS than in SND or OPCA. Follow-up studies of some of the patients with SDS and OPCA suggested that the responsible sites of pelvic nerve lesions changed from supra- to infranuclear lesions during the course of disease.     

An autopsy case of Shy-Drager syndrome preceded by a urinary disturbance for over 20 years

An autopsy case of Shy-Drager syndrome preceded by urinary disturbance for over 20 years was reported. A 43-year-old woman was admitted to our hospital because of urinary disturbance and orthostatic hypotension. At the age of 19 she developed urinary disturbance with polyuria and retention. These symptoms were getting worse with years, and at the age of 33 she was diagnosed to have neurogenic bladder of uninhibited type. During her hospital course her symptom became worse, and by the age of 42 she showed marked dysarthria, disturbance of smooth pursuit eye movement, Horner's syndrome, marked rigidity and tremor of four extremities, generalized hyperreflexia, marked limb and truncal ataxia, neurogenic bladder and orthostatic hypotension. Serial brain CT scan revealed progressive brain stem and cerebellar atrophy with clinical course. Severe autonomic nervous system dysfunctions were also documented. She died of respiratory failure at the age of 43. On autopsy, brain stem and cerebellum showed marked atrophy macroscopically. Microscopically marked depletion of neuron was seen in the substantia nigra, pontine nuclei, inferior olive, Purkinje cells, the intermediolateral column of spinal cord and Onuf's nucleus of S2. Although numerous cases of Shy-Drager syndrome have been reported in the past, there is no case which developed this syndrome after urinary disturbance of over 20 year's duration. We should be alert to observe the cases with longstanding urinary disturbances in order to not overlook degenerative disorders as exemplified in this case.     

Shy-Drager syndrome presenting as a REM behavioral disorder

Shy-Drager syndrome (SDS) is associated with a myriad of autonomic and neurologic impairments, including sleep disturbances. A review of the literature reveals that there are no reports of SDS presenting with an abnormality of sleep. The following case report describes a patient who was diagnosed with SDS approximately one decade after initially presenting with a progressive, polysomnographically confirmed disturbance of sleep--specifically, an REM behavioral disorder (RBD).     

Immunohistochemical quantification of substance P in spinal dorsal horns of patients with multiple system atrophy.

Using a computer-assisted image analyser, an immunohistochemical quantification method of substance P-like immunoreactivity (SPLI) in laminae I + II of spinal dorsal horn was established and applied to 13 patients with multiple system atrophy (MSA) with no disturbance of pain sensation, including olivo-ponto-cerebellar atrophy and striatonigral degeneration, and 13 neurologically normal controls. To investigate whether alteration of SPLI is related to an autonomic disorder, myelinated fibre counts of the fourth thoracic ventral roots were performed. Eleven of 13 MSA patients showed a significant decrease in small and large myelinated fibres, and were diagnosed with definite Shy-Drager syndrome (SDS), with the exception of two who had no apparent history of autonomic dysfunction. SPLIs in laminae I + II in 10 of these 11 patients, when adjusted for age, were significantly decreased at both levels of the fourth thoracic and third lumbar spinal segments. The results suggest the disorder of SP-containing synapses of primary afferent neurons and/or those of interneurons in SDS.     

疑似多系统萎缩48例的临床分析

目的探讨多系统萎缩（MSA）的临床特点,为临床诊断提供依据.方法按Gilman 诊断标准,回顾性分析38例拟诊MSA和10例可能MSA患者的临床资料、肛门括约肌肌电图（EAS-EMG）和其他辅助检查.结果本组有橄榄脑桥小脑萎缩（OPCA）15例,Shy-Drager综合征（SDS）20例和纹状体黑质变性（SND）13例.29例行EAS-EMG检查,23例有自发电位.结论临床表现与EAS-EMG等辅助检查结合,可提高MSA的诊断率.     

Abnormally increased iron concentration in basal ganglia in Shy-Drager syndrome. MR imaging and autonomic study.

Report of an early case of Shy-Drager syndrome in a 67 year-old woman patient. Autonomic failure was diagnosed by functional evaluation as well as laboratory tests. MR imaging disclosed a prominent putamina hypodensity in T2-weighted images at high field strength due to iron increased depositing in this basal ganglia. MR imaging evidences confirm Shy-Drager syndrome diagnosis, and contributes for differential diagnosis of idiopathic hypotension (pure autonomic failure) in special in SDS early cases.     

Micturitional disturbance in neuro-Behçet's syndrome

BACKGROUND: Micturitional disturbance is known to occur in neuro-Beh?et's syndrome (NBS). However, its pathophysiology is uncertain and few data are available concerning urodynamic findings. We describe our findings on micturitional histories and urodynamic studies in patients with NBS. METHODS: A history of urinary symptoms was obtained from nine patients with NBS [seven male, two female, aged 26 to 54 years; mean, 41 years]. Neurological and CT/MRI findings indicated that their lesions were located mainly in the cerebral cortex, basal ganglia, cerebellum, brainstem and the spinal cord. Symptomatic patients underwent urodynamic studies, including measurement of post-micturition residuals, urethral pressure profilometry and EMG cystometry. RESULTS: Six of the nine patients (67%) had urinary symptoms that included diurnal urinary frequency in five, sensation of urgency in five, urge urinary incontinence in five, voiding difficulty in five and nocturnal urinary frequency in four. None had urinary retention. Urodynamic studies performed in six patients showed post-micturition residuals in five (50-180 ml), increased maximum urethral closure pressure in two, decrease in four and increase in one of bladder capacities, detrusor hyperreflexia in five, decreased bladder sensation in one, brisk bulbocavernosus reflex in four, detrusor-sphincter dyssynergia in two and neurogenic sphincter EMG in one. CONCLUSION: Our results indicate that micturitional disturbance is not rare in patients with NBS. Supranuclear pelvic nerve dysfunction seems to be mainly responsible for the disturbance.     

Periodic respiration in erect posture in Shy-Drager syndrome.

Tilt-table polygraphic study in four patients with Shy-Drager syndrome demonstrated periodic apnoea in the erect posture. In one patient reduced hypercapneic ventilatory response and necropsy findings of neuronal loss and astrocytosis in the pontine tegmentum suggested dysfunctional respiratory neurones in the brainstem. One patient had Cheyne-Stokes respiration during the late stage of the illness.     

Idiopathic cerebellar ataxia of late onset: natural history and MRI morphology.

Twenty eight patients with the clinical diagnosis of idiopathic late onset cerebellar ataxia were examined clinically and by magnetic resonance imaging (MRI) or computed tomography (CT). In addition, the clinical records of all patients were analysed retrospectively. On the basis of their clinical presentation they were subdivided into patients with a pure cerebellar syndrome (n = 9) and patients with a cerebellar syndrome and additional non-cerebellar symptoms (n = 13). No attempts were made to classify patients with a disease duration of less than four years (n = 6) because the retrospective analysis showed that the disease started almost invariably with a pure cerebellar syndrome and additional symptoms came later. Patients with a lasting pure cerebellar syndrome had a significantly better prognosis than patients with additional non-cerebellar involvement (annual progression rate rate: 0.40 versus 0.80). Calculated median lifetime from onset of symptoms was 20.7 years in patients with a pure cerebellar syndrome and 7.7 years in patients with additional non-cerebellar symptoms. Among the latter, disease progression was faster the earlier non-cerebellar symptoms occurred. All of them presented with Parkinsonian symptoms, whereas bulbar symptoms, vertical gaze paresis, pyramidal deficits, dementia and urinary incontinence were encountered less frequently. MRI or CT showed cerebellar atrophy without apparent involvement of brainstem structures in all patients with a pure cerebellar syndrome suggesting the diagnosis of cerebellar cortical atrophy (CA). The majority of the patients with additional non-cerebellar symptoms had evidence of an atrophy of the cerebellum and the brainstem suggesting the presence of olivo-ponto-cerebellar atrophy (OPCA). In two of them, however, MRI morphology was not compatible with the diagnosis of OPCA.