原发性干燥综合征的神经系统损害

目的　探讨原发性干燥综合征的神经系统损害。方法　从电生理、病理及其它实验室检查 ,分析 2 2例原发性干燥综合征合并神经系统损害的临床特征及病变部位。结果　 7例原发性干燥综合征合并神经系统损害的患者中 ,周围神经病变 6例 ,多发性硬化 1例。结论　原发性干燥综合征合并神经系统损害以周围神经病变为主 ,其神经系统症状可出现在原发性干燥综合征诊断之前。     

以神经系统损害为首发症状的干燥综合征3例报道并文献复习

目的探讨干燥综合征合并神经系统损害的机制、临床表现及治疗方法。方法报告3例以神经系统损害为首发表现的干燥综合征病例,并对相关文献进行复习。结果 3例患者中2例为中枢神经病变(其中1例合并周围神经病变),1例为周围神经病变。经丙种球蛋白、糖皮质激素、营养神经等药物治疗后,患者症状均得到不同程度改善。结论干燥综合征为自身免疫性疾病,因累及部位的不同,合并神经系统病变的临床症状也呈多样性,早期临床症状常缺乏典型性,应详细询问病史并结合实验室检测、影像学及唇腺活检等检查,减少误诊。     

电生理检查在原发干燥综合征合并中枢及周围神经系统损害的应用

目的探讨原发性干燥综合征(p SS)合并中枢及周围神经系统损害的电生理特征及应用。方法选取2003年-2014年住院收治的p SS患者共19例,按照中枢和周围神经系统损害进行分类,再分别对两类患者的肌电图(EMG)(包括针极EMG、感觉和运动神经传导速度)及视觉诱发电位(VEP)检查(均包括视觉、听觉及四肢体感)结果进行总结分析。结果干燥综合征合并周围及中枢神经系统损伤的电生理表现多以感觉受损为主,可以是脑神经或四肢周围感觉神经受累也可以是髓内或颅内上级感觉皮质受累,而且可以帮助发现亚临床病灶,电生理检查确定的病损部位多于临床。结论电生理检查对原发干燥综合征的诊断和治疗中具有重要意义,建议在临床实践中应给予重视和应用。     

伴有胼胝体压部可逆性病灶的临床症状轻微的脑炎/脑病1例报道并文献复习

目的探讨伴有胼胝体压部可逆性病灶的临床症状轻微的脑炎/脑病的临床特点、治疗及预后、发病机制。方法报道1例伴有胼胝体压部可逆性病灶的临床症状轻微的脑炎患者的诊治过程,总结其临床特征并复习相关文献。结果患者表现为发热、头痛、呕吐等非特异性脑膜炎症状,合并出现抗利尿激素分泌不当导致的低钠血症,头颅MRI检查发现胼胝体压部有可逆性的DWI高信号的孤立病灶,经抗病毒及对症治疗,临床痊愈。结论伴有胼胝体压部可逆性病灶的临床症状轻微的脑炎/脑病以头颅MRI上发现胼胝体压部可逆性病灶为特点,临床症状轻微,容易合并低钠血症,预后良好,病因及发病机制尚不清楚。     

原发性干燥综合征合并神经系统病变研究进展

原发性干燥综合征是一种慢性炎症反应性自身免疫性疾病,主要累及外分泌腺,亦可累及神经系统,甚至部分患者以神经系统病变为首发症状。由于目前缺乏原发性干燥综合征合并神经系统病变的统一诊断标准,易误诊和漏诊。本文拟对原发性干燥综合征合并神经系统病变的研究进展和常见临床问题进行概述。     

磁共振弥散加权成像延迟显影的缺血性脑卒中患者临床特点分析(附16例报告及文献复习)

探讨磁共振弥散加权成像(MR-DWI)显影延迟的缺血性脑卒中患者的临床特点。方法回顾性分析16例DWI显影延迟患者的临床资料,综合分析其临床特点。结果 16例患者中男性9例、女性7例,平均年龄(66.94±8.27)岁。主要临床症状为头晕、行走不稳、肢体活动无力、言语表达不清、视野缺损。查体:11/16例(68.75%)可见神经系统定位体征,5/16例(31.25%)无神经系统定位体征。首次头颅MRI检查时间距离发病的中位时间为17(6～50)h,临床症状缓解不明显或神经系统阳性体征持续不缓解;复查头颅MRI检查时间距离发病的中位时间为45(28～82)h。梗死病灶部位:15例位于后循环,1例位于前循环。结论对神经系统症状、体征持续不缓解、或出现新发症状或体征的缺血性脑卒中患者,应及时复查头颅MR-DWI检查,以尽早发现DWI延迟显影的脑梗死病灶,有助于协助临床诊断和积极治疗,改善预后。     

伴CNS炎性脱髓鞘的原发性干燥综合征8例临床分析

目的探讨伴有CNS炎性脱髓鞘的原发性干燥综合征(p SS)的临床特点。方法回顾性分析8例诊断明确的伴有CNS炎性脱髓鞘的p SS患者的临床资料。结果 8例患者中7例以CNS症状表现为首发症状。在3例出现脑损害症状的患者中,1例表现为头晕、视物重影、饮水呛咳、行走困难,其病灶位于延髓至颈髓C2段脱髓鞘病灶;1例表现为听力下降、耳鸣,病灶位于双侧额颞顶深部白质脱髓鞘病灶;1例表现为反应迟钝、听力下降,头颅MRI示双侧额顶叶深部多发脱髓鞘病灶。其余5例均为脊髓损害症状患者,单纯颈髓受累1例,单纯胸髓受累1例,颈胸腰骶髓受累1例,颈胸髓同时受累1例,胸腰段受累伴左侧颞极病灶1例。2例脊髓炎患者行血清水通道蛋白4(AQP4)抗体检测为阳性。8例患者中1例患者放弃治疗,7例患者接受治疗,其中5例好转,2例无明显疗效。结论在CNS脱髓鞘的患者中应注意筛查是否合并系统性疾病如p SS等,在p SS患者中若出现视神经脊髓炎典型表现应查AQP4抗体以尽早诊断是否二者共病。     

原发性干燥综合征的神经系统损害

本文统计了我院近１０年内的干燥综合征病例。在１８例原发性干燥综合征患者中，６例合并神经系统损害，占３３．３％。合并周围神经损害者最常见，以感觉障碍为主，其中深感觉障碍尤为突出。中枢神经系统和植物神经系统亦可受累。     

原发性干燥综合征神经精神损害28例分析

目的探讨原发性干燥综合征(pSS)的神经精神损害特点。方法选择我院1984~2004年间住院的原发性干燥综合征合并神经精神损害的病人共28例进行回顾性分析。结果周围神经系统(PNS)受累19例,中枢神经系统(CNS)7例,同时受累2例;重症肌无力2例,肌肉病变2例(其中1例同时存在周围神经病);抑郁状态1例。结论pSS的神经精神损害表现多样,PNS和CNS均可受累,并且神经系统损害表现常先于pSS症状,常缺乏腺体外表现和免疫功能异常。     

青年人中重度一氧化碳中毒致脑梗死11例临床分析

目的探讨青年人中重度一氧化碳中毒合并急性脑梗死的临床特征及预后。方法对我院近几年收治的11例一氧化碳中毒合并急性脑梗死患者的临床资料进行回顾性分析。结果 11例患者入院时均有不同程度的意识障碍,入院后经急诊处理后立即进行CT或MRI检查,均未发现有明显异常。入院后或出现神经系统定位体征后24h复查头颅CT或MRI,均发现有与症状和体征相关的梗死病灶。经综合治疗,6例治愈或基本治愈,4例有不同程度的后遗症,1例大面积脑梗死患者死于多脏器功能衰竭。结论青年人中重度一氧化碳中毒后可合并急性脑梗死的发生,治疗一氧化碳中毒的过程中要及时发现其对脑血管的损害,防止漏诊。     

干燥综合征并发帕金森综合征1例及文献复习

目的：探讨干燥综合征和帕金森综合征之间的关系。方法：经临床症状和右侧唇腺病理确诊的1例干燥综合征患者合并出现帕金森综合征样表现,结合国内外文献报道对其进行分析。结果：干燥综合征患者并发中枢神经系统病变并不少见,但合并出现帕金森综合征的不多见,国内尚未见报道。予以甲泼尼龙0.5g冲击治疗3d后,患者眼干、口干症状改善,且帕金森综合征表现也较前好转。结论：干燥综合征并发帕金森综合征样临床表现,文献报道均为女性,其发病机制有待进一步研究。治疗方法与其他帕金森综合征不尽相同,目前主张激素治疗,但疗效不一。     

Traumatic cervical Brown-Sequard and Brown-Sequard-plus syndromes: the spectrum of presentations and outcomes.

Brown-Sequard syndrome (BSS) and Brown-Sequard-plus syndrome (BSPS) are characterised by asymmetrical paresis with hypalgesia more marked on the less paretic side. This study examined the clinical features of 38 patients (30 males and 8 females; mean age = 32 years) with traumatic cervical BSS or BSPS who underwent comprehensive inpatient rehabilitation. Twenty two injuries were caused by road traffic accidents, 8 by penetrating injuries, 5 by diving injuries, and 3 by other causes. After an average of 35 days in acute care and 79 days in rehabilitation, 37 patients had increased muscle strength, all 38 patients improved functional abilities, 29 patients walked independently, 34 had spontaneous bladder emptying, 36 were discharged home, and 14 were employed. Statistically significant increases (p less than 0.001) were made in modified Barthel index functional scores between admission and discharge. Patients with BSPS had a better prognosis than did those with 'pure' BSS. Patients with predominant upper limb weakness had more favourable outcomes than did those with predominant lower limb weakness. Few other potentially predictive demographic, injury, or neurological factors were associated with functional outcome. Patients with BSS or BSPS generally have a good prognosis for neurological and functional improvement.     

Intraindividual Variability in Physical and Emotional Functioning: Comparison of Adults With Traumatic Brain Injuries and Healthy Adults

Recent research has shown that individuals with certain neurological conditions demonstrate greater intraindividual variability on cognitive tasks compared to healthy controls. The present study investigated intraindividual variability in the domains of physical functioning and affect/stress in three groups: adults with mild head injuries, adults with moderate/severe head injuries, and healthy adults. Participants were assessed on 10 occasions and results indicated that (a) individuals with head injuries demonstrated greater variability in dominant finger dexterity and right grip strength than the healthy controls; (b) increased variability tended to be associated with poorer performance/report both within and across tasks; and (c) increased variability on one task was associated with increased variability on other tasks. The findings suggest that increased variability in physical function, as well as cognitive function, represents an indicator of neurological compromise.     

肢带综合征1例及文献复习

目的:探讨淋巴瘤肌肉侵犯的临床特点。方法:报道1例肌肉病理确诊为淋巴瘤合并肌肉侵犯患者,对其临床、肌肉MRI表现和肌肉活检病理结果结合复习文献予以分析,总结淋巴瘤肌肉侵犯的临床特点。结果:淋巴瘤肌肉侵犯发生率较低,主要表现为受累肌肉肿块、疼痛和无力。MRI中可表现为局部肿块或弥漫性肿大及异常信号。肌肉活检及免疫组化有助于与肌炎鉴别和淋巴瘤的分型。以肌肉侵犯为主要表现的淋巴瘤化疗以CHOP方案为主,预后较好。结论:淋巴瘤肌肉侵犯可引起包括肢带综合征在内的肌病表现,有肌肉肿块或无力症状以及相应MRI表现的病例应考虑淋巴瘤肌肉侵犯可能。     

Schizotypal personality traits in Gilles de la Tourette syndrome

OBJECTIVES: Gilles de la Tourette syndrome (GTS) is a chronic tic disorder associated with comorbid psychopathology, including obsessionality, affective instability and attention-deficit hyperactivity disorder. Evidence linking GTS with schizophrenia-like symptoms is limited and equivocal, despite a common putative substrate involving dopaminergic dysfunction within frontostriatal circuits. The aim of this study was to quantify the prevalence of schizotypal traits in GTS and to detail the relationship between schizotypy and comorbid psychopathology. MATERIALS AND METHODS: A total of 102 subjects with GTS were evaluated using the Schizotypal Personality Questionnaire and standardized neurological and psychiatric rating scales. The predictive interrelation between schizotypy, tic-related symptoms and psychiatric comorbidities was investigated using correlation and multiple regression analyses. RESULTS: In our clinical population, 15% of the subjects were diagnosed with the schizotypal personality disorder according to the DSM-IV criteria. The strongest predictors of schizotypy were obsessionality and anxiety ratings. The presence of multiple psychiatric comorbidities correlated positively with schizotypy scores. CONCLUSIONS: Schizotypal traits are relatively common in patients with GTS, and reflect the presence of comorbid psychopathology, related to the anxiety spectrum. In particular, our preliminary results are consistent with a shared neurochemical substrate for the mechanisms underpinning tic expression, obsessionality and specific schizotypal traits.     

Whiplash injury: a reappraisal.

Few topics provoke so much controversy or heated opinion, based on so little fact as whiplash injuries. In emergency departments, orthopaedic, neurological and rheumatological clinics, and not least in the Courts, this common syndrome is shrouded in mystery and creates clinical insecurity in those who attempt to explain its mechanism, its prognosis and treatment. These problems are compounded in medico-legal practice where the potential rewards of successful litigation may colour the clinical picture.     

The neurological assessment of patients in vegetative and minimally conscious states

Patients with serious central nervous system (CNS) injuries can enter a chronic state in which there is impaired awareness, but the presence of preserved basic vegetative functions. Such cases present formidable challenges to the medical practitioner in terms of clinical assessment and thus management. This paper presents the neurological approach to such patients highlighting the clinical clues that need to be sought to decide whether the patient is in a persistent vegetative or minimally conscious state (i.e., showing any responsiveness to external stimuli), or alternatively suffering from a locked-in syndrome. This neurological clinical formulation is then assimilated with other tests and assessments from a range of medical specialities, and by so doing helps confirm the clinical impression. This multidisciplinary approach is vital in the assessment of such patients and ideally should also include neurophysiological and functional imaging paradigms.     

Neurological features in adult Triple-A (Allgrove) syndrome

Triple-A or Allgrove syndrome is a rare multisystem disease classically associated with esophageal achalasia, adrenal insufficiency and alacrima. Here, we describe the poorly understood neurological characteristics often associated with this condition, through the clinical and electrophysiological analysis of eight patients. All patients were genetically confirmed and had a mutation in the ALADIN gene. They all displayed a classical picture of Triple-A syndrome: all suffered from achalasia and alacrima and half of them from adrenal insufficiency. However, all harbored a neurological picture characterized by a recognizable pattern of peripheral neuropathy. Other neurological features included cognitive deficits, pyramidal syndrome, cerebellar dysfunction, dysautonomia, neuro-ophthalmological signs and bulbar and facial symptoms. This neurological picture was prominent in all patients and misled the initial diagnosis in six of them, which had a late onset. We then review the previous neurological reports of this disease, to improve the understanding of this rare condition. Diagnosis of late-onset Triple-A syndrome is difficult when the clinical picture is mainly neurological and when endocrine or gastrointestinal signs are minor. The characteristics of the peripheral neuropathy, among other neurological signs, can be of help.     

Sources of error in the diagnosis of Guillain-Barre syndrome

A careful monitoring of the accuracy of diagnosis in six cases of Guillain-Barre syndrome has shown that a substantial proportion of these patients initially diagnosed as having Guillain-Barre syndrome on the basis of characteristic clinical findings and an elevated level of protein in the spinal fluid had a neuropathy caused by another etiology. The pitfalls in the laboratory and clinical diagnosis of disorders that were confused with Guillain-Barre syndrome were several: the pattern of neurological dysfunction in the Guillain-Barre syndrome was not unique to that disorder; no specific laboratory test existed to confirm the diagnosis of Guillain-Barre syndrome; and the laboratory diagnosis of other causes of similar neurological disorders (especially heavy metal intoxication) depended upon tests that are very unreliable.     

Neurological and neuropsychological consequences of electrical and lightning shock:review and theories of causation

Injuries from lightning and electrical injuries involve multiple systems of the body, however neurological symptoms are very widely reported. A disabling neuropsychological syndrome is also noted.This paper presents a comprehensive review of neurological and neuropsychological symptoms. Partial theories of causation for these injuries have been advanced, however, there is no convincing explanation for both delay in onset of symptoms and also the genesis of the neuropsychological syndrome. A theory of causation is proposed which satisfies both these constraints.This theory suggests circulating hormones such as cortisol, together with nitric oxide and oxidant free radicals from glutamatergic hyper-stimulation, act on tissues remote from the injury path including the hippocampus.This theory opens a research path to explore treat-ment options.