Detection of anterior horn lesions by MRI in central European tick-borne encephalomyelitis

We report a case of central European tick-borne encephalitis with cervical myelitis presenting clinically as a lower motor neuron syndrome of the upper limbs with proximal asymmetrical pareses and atrophies. There were no sensory deficits nor signs of lesions of the spinal pathways or signs of encephalitis or meningitis. The affected motor fibers of the upper limbs were electrically inexcitable, but sensory findings were normal. Electromyography of the paralyzed muscles revealed pathological denervation activity without voluntary activation. The initial magnetic resonance imaging (MRI) showed a large hyperdense lesion in the anterior part of the cervical cord from C3 to T1. Despite the fact that MRI changes disappeared completely within 6 weeks the patient showed only little improvement in the paralyzed muscles after 6 months. To our knowledge, these MRI changes in patients with tick-borne encephalitis, consistent with an isolated anterior horn lesion, have never been reported previously. The course may have been aggravated by an initial antibiotic treatment with cephalosporins. Received: 4 May 1999 Received in revised form: 22 July 1999 Accepted: 26 July 1999     

抗N-甲基-D-天冬氨酸受体抗体脑炎复发的临床特征

目的总结复发性抗N-甲基-D-天冬氨酸受体(NMDAR)抗体脑炎的临床特点、诊断及治疗。方法回顾性分析2013年5月至2017年2月郑州大学人民医院诊治的57例抗NMDAR抗体脑炎中10例复发者的临床症状、实验室检查、影像学特点和免疫治疗。结果 10例(19%)复发中男5例,女5例,平均年龄27.5岁,10例共复发14次,其中2例复发2~3次,平均复发间隔时间7.1个月(1~19月)。复发时最常见症状为癫痫(8/14)和精神行为异常(8/14),其中1例次复发仅表现为不典型症状,如眩晕、行走不稳和复视。复发时13例次行抗NMDAR抗体检测,脑脊液抗体阳性率100%(13/13),血清抗体阳性率69%(9/13)。13例次复发再次给予免疫治疗仍有效,1例次拒绝治疗,出院后平均随访18.9月,3例完全恢复正常,4例遗留轻微后遗症,3例遗留较明显后遗症,影响日常生活。结论抗NMDAR脑炎复发时可表现为典型或不典型症状,脑脊液抗NMDAR抗体检测有助诊断,积极免疫治疗仍有效。     

Neurosarcoidosis Flare with Multifocal Restricted Diffusion: Stroke,Inflammation, or Both?

Sarcoidosis is a multisystem granulomatous disease that can cause a wide range of neurologic symptoms. Leptomeningeal enhancement is frequently described but reports of stroke-like symptoms or the appearance of ischemia on magnetic resonance imaging are rare. We present a case of a patient with both leptomeningeal enhancement and multifocal restricted diffusion in a patient with sarcoidosis.     

Stroke-like episodes in familial mitochondrial encephalomyopathy: clinical and biochemical aspects

Summary Acute episodes of focal neurological dysfunction are a well-recognized complication of the mitochondrial encephalomyopathies. Because of rapid remission, biochemical tests and other diagnostic procedures are mostly performed after the acute phase. We report the case of a patient suffering from mitochondrial disease manifesting primarily with seizures, progressive deafness and dementia, who experienced multiple stroke-like episodes. Other members of the family with evidence of mitochondrial dysfunction are presented briefly. EEG and biochemical findings in the acute stage are correlated with clinical symptoms, showing characteristics distinct from the chronic illness. The possible involvement of dietary factors in the provocation of stroke-like episodes is discussed and regulation of glucose intake suggested as a strategy in the prevention of stroke-like episodes.     

Limbic Encephalitis Investigated by 18FDG-PET and 3D MRI

Two patients with clinically probable or possible limbic encephalitis (LE) are reported, both cases with typical findings in clinical symptoms (severe neuropsychological deficits and complex partial seizures) and in routine magnetic resonance imaging (MRI) (hyperintense mesiotemporal lesions). Underlying malignancy was identified (rectal carcinoma) in one case but could not be detected in the other patient. The 2 patients were investigated by cerebral 18F-fluoro-2-deoxy-D-glucose-positron emission tomography (FDG-PET) and 3-dimensional (3D) MRI, and abnormalities in metabolic activity were mapped using coregistration of spatially normalized PET and MRI. Highly significant focal hypermetabolism in bilateral hippocampal areas was found in both cases. The authors' findings support FDG-PET coregistered to 3D MRI as a potentially valuable additional tool in the imaging diagnostics of LE. Results are discussed with respect to the clinical symptoms and previously reported imaging findings in the disease.     

l-arginine is effective in stroke-like episodes of MELAS associated with the G13513A mutation

We report a case involving a 15-year-old boy with MELAS (G13513A mutation) who developed several stroke-like episodes in a short period of time. Intravenous administration of l-arginine during the acute phase of the stroke-like episodes reduced symptoms immediately, and oral supplementation of l-arginine successfully prevented further stroke-like episodes. This is the first report on effective l-arginine therapy in MELAS associated with the G13513A mutation.     

线粒体脑肌病伴高乳酸血症和脑卒中样发作综合征一家系临床特征及线粒体基因A3243G位点点突变异质性水平

目的 调查1个疑似患有母系遗传性线粒体脑肌病伴高乳酸血症和脑卒中样发作(MELAS)综合征家系的临床表现、生物化学检测数据和影像学资料,并探索其与血细胞线粒体基因突变异质性水平的关联性.方法 收集先证者和11位其母系家系成员的一般情况、抽搐及脑卒中样发作等病史,检测家系成员的血常规和运动前后血浆乳酸水平等生化指标,并做头颅磁共振检查.用聚合酶链反应(PCR)-限制性内切酶片段长度多态和DNA测序法检测其成员是否存在线粒体基因组A3243G点突变,并用荧光实时定量PCR定量该突变的水平.结果 该家系部分成员存在抽搐、脑卒中样发作和高乳酸血症等MELAS综合征典型症状,以及身材矮小、运动不耐受和发热、偏头痛等非典型症状.发作期头颅磁共振成像符合MELAS综合征的典型特点,且普遍存在小脑萎缩.母系亲属均存在线粒体基因的A3243G位点点突变,突变异质性水平越高,症状越典型且严重.结论 该调查家系确诊母系遗传性MELAS综合征,其致病基因为线粒体A3243G点突变.外周血血细胞线粒体基因突变异质性水平与亲缘关系、抽搐早现性和血乳酸值等临床表型存在相关性.     

8例自身免疫性脑炎的病例分析并文献复习

目的探讨自身免疫性脑炎的临床表现、磁共振(MRI)图像、脑脊液特点及治疗转归。方法回顾性分析我院8例(自2012年1月-2016年1月)自身免疫性脑炎患者的临床资料。结果 8例患者平均发病年龄为44岁,其中有4例抗NMDA受体脑炎,3例LGI1蛋白抗体阳性边缘系统脑炎,1例抗GABABR脑炎,所有患者(100%)均有认知功能损害和精神症状;5例(62%)出现癫痫发作;3例(37%)出现不自主运动和自主神经功能障碍。4例(50%)患者脑电图存在异常;4例(50%)患者有磁共振检查异常信号;1例发现肺癌。结论以认知功能损害、精神行为异常、癫痫发作为主要表现的患者,要警惕自身免疫性脑炎的可能,以免误漏诊。     

发病酷似单纯疱疹病毒脑炎的抗N-甲基-D-天冬氨酸受体脑炎

目的 提高对发病酷似单纯疱疹病毒脑炎(Herpes simplex encephalitis,HSE)的抗N-甲基-D-天冬氨酸(N-methyl-D-aspartate,NMDA)受体脑炎的认识。方法 报道2例发病酷似单纯疱疹病毒脑炎的抗NMDA受体脑炎,并复习相关文献。结果 2例患者均急性起病,表现为发热、抽搐、认知功能减退、精神行为异常等。脑MRI均未见异常。病例1、病例2脑脊液白细胞最高分别为20×10⁶/L、21×10⁶/L。脑脊液蛋白、葡萄糖以及氯化物水平均正常。2例患者早期均被诊断为HSE,并接受阿昔洛韦抗病毒治疗,但病情仍进展。抗NMDA受体抗体检测显示例1脑脊液及血液均阳性,例2脑脊液阳性,从而明确了抗NMDA 受体脑炎的诊断。结论 对脑MRI无异常,脑脊液白细胞无明显升高的边缘系统脑炎,应想到抗NMDA 受体脑炎可能。     

Neuroborreliosis simulating a progressive stroke

A 55-year-old man with an onset and course of neurological symptoms of a progressive stroke was found to have a CNS infection caused by the borrelia spirochete. Almost complete recovery was seen after intravenous infusion of bencylpenicillin. Elevated borrelia IgG antibody titers could be seen for long time after recovery. The possibility of an infection due to neuroborreliosis must be considered. If CT scan doesn't show any focal ischemic or hemorrhagic area further investigation with lumbar puncture is necessary in stroke patients.     

线粒体脑肌病伴高乳酸血症和卒中样发作的诊断

目的 分析总结线粒体脑肌病伴高乳酸血症和卒中样发作(MELAS)的临床表现和辅助检查结果,探讨在MELAS诊断中存在的问题,提出MELAS早期临床诊断的可行性.方法 归纳总结34例MELAS患者的临床表现,寻找对MELAS患者相对特异的症状组合.同时总结分析各项辅助检查结果,结合文献复习解读血和脑脊液的乳酸水平、神经影像、肌肉活体组织检查(活检)和基因检查对于MELAS诊断的意义和局限性.结果 本组患者最常见的临床表现依次为:癫癎发作、头痛、智能下降、卒中样发作、发育异常、四肢无力和易疲劳、眼肌麻痹.血空腹乳酸或运动后乳酸升高23例(67.6%).32例头颅MRI异常的患者最常见的受累部位依次为枕叶、顶叶、颞叶、基底节、额叶、小脑和深部白质.32例患者行肌肉活检,有阳性发现的共24例(75%),阴性的8例(25%).14例患者行基因检查,9例发现A3243G位点突变,5例未发现该位点突变.结论 认识MELAS的各种临床表现,争取在卒中样发作出现前早期诊断是完全可行的.     

Neuroimaging of stroke-like episodes in MELAS

Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) shows sudden neurological deficits that are called ‘stroke-like episodes’. With regard to the pathophysiology of stroke-like episodes, so-called mitochondrial angiopathy and cytopathy theories have been proposed, but the subject is still controversial. To clarify this matter and to contribute to the development of a treatment for MELAS, we review here current neuroimaging research and consider the pathophysiology of stroke-like lesions. With regard to diffusion-weighted imaging findings, early reports often showed an elevated apparent diffusion coefficient (ADC) in stroke-like lesions; this was considered to be mainly vasogenic edema in the acute phase and is a different pattern than that in stroke. However, there has recently been an increase in the number of reports of a decrease in ADC; these cases are considered to be cytotoxic edema in the acute phase, which is compatible with stroke. With regard to ¹H-magnetic resonance spectroscopy findings in stroke-like lesions, a decrease in N-acetylaspartate and an increase in lactate have been reported. With regard to single photon emission computed tomography findings for stroke-like lesions in MELAS, an overall trend is hyperperfusion in the acute stage (within 1 month) of stroke-like episodes and hypoperfusion in the chronic stage (several months later). With regard to positron emission tomography, nearly all of these reports are consistent with the mitochondrial cytopathy theory. With regard to neuropathology in MELAS, the most common findings during the chronic stage of stroke-like episodes include foci of necrosis and peculiar vascular changes (abnormalities of mitochondria in small arteries). Concerning the pathology of the acute stage of stroke-like episodes, extensive petechial hemorrhage along the gyri of the cortex corresponding to acute stroke-like lesions has been reported. To clarify the true pathophysiology of stroke-like episodes, we offer three suggestions. First, we must define the precise onset of stroke-like episodes. Second, current studies are limited by the difficulty of imaging just before and just after (within a few minutes) the onset of stroke-like episodes. Third, we hope to establish an experimental animal model. We should conduct a simultaneous multimodal imaging and histological study just before and just after (within a few minutes) the onset of stroke-like episodes in an experimental animal model.     

伏格特-小柳-原田综合征脑实质损害症状的临床分析

目的 总结伏格特-小柳-原田综合征的神经系统症状损害特点。 方法 回顾性分析住院伏格特-小柳-原田综合征患者的临床资料,对患者的临床表现、神经系统症 状、实验室及磁共振成像（magnetic resonance imaging,MRI）检查结果、治疗等进行描述性分析。 结果 研究共纳入36例患者,其中30例（83.33%）患者出现神经系统症状,以头痛最为常见（29例, 80.56%）,卒中样表现包括肢体及面部麻木（1例,2.78%）、步态不稳（1例,2.78%）、意识障碍（1例, 2.78%）等。有卒中样症状的患者MRI可见脱髓鞘表现,但未见新发梗死灶。 结论 伏格特-小柳-原田综合征中可出现脑实质损害症状,在MRI上可表现为脱髓鞘改变。     

A case of neonatal human parechovirus encephalitis with a favourable outcome

Human parechoviruses (HPeVs) are a new family of neurotropic viruses that cause central nervous system (CNS) infections similar to enterovirus (EVs) meningoencephalitis in the neonatal period, resulting in white matter lesions that can be visualized with cranial ultrasonography and magnetic resonance imaging, and correlated to a large spectrum of neurological outcomes. HPeV should be suspected in neonates with signs and symptoms of sepsis-like illness or CNS disease. We report a case of neonatal HPeV encephalitis, diagnosed on the basis of clinical and radiological findings and HPeV RT-PCR, with a good neurological outcome.     

抗NMDAR脑炎临床特点及影像表现分析

目的探讨抗NMDAR脑炎的临床特点、影像表现及意义。方法回顾性分析陕西省人医院2014-01-2020-08确诊的11例抗NMDAR脑炎患者的临床及影像资料。结果11例患者为急性或亚急性起病,首发症状表现为精神症状2例,认知功能损害2例,痫性发作4例,非特异性症状3例。11例患者的脑脊液抗NMDAR抗体均为阳性,其中2例脑脊液HSV抗体阳性,1例脑脊液麻疹病毒抗体阳性。头颅MRI 7例表现为侧脑室旁、岛叶、颞叶、额叶及海马等部位高T2 FLAIR信号,4例未见明显病灶。MRI首次阳性者,病灶范围会随病情好转而缩小或加重而增大。MRI首次阴性者,复查可出现阳性或仍为阴性。结论抗NMDAR脑炎临床表现复杂多样,MRI阳性者结合临床特点可提示诊断,动态观察MRI表现可反映疾病进展、演变。     

复发性抗NMDA受体脑炎的临床及复发原因分析

目的探讨复发性抗NMDA受体脑炎的临床及复发原因分析。方法回顾我科诊治的复发性抗NMDA受体脑炎8例,分析其临床表现、神经影像、辅助检查等特点及治疗方案。结果男2例,女8例,年龄为16~60岁。首次发病的起病症状包括:精神异常3例,癫痫5例,中枢性通气障碍3例,神志不清4例,自主神经障碍4例。复发时症状:精神异常8例,癫痫4例(2例癫痫持续状态),中枢性低通气2例,视神经损害2例,意识水平下降3例,自主神经障碍1例。复发次数为1~2次,首次发病及复发期的抗NMDA受体抗体滴度阳性率为100%;首次发病及复发期脑电图均异常。头部MRI首次发病检查中3例异常,复发时原有病灶消失或减小,2例见新病灶。1例于复发期发现卵巢畸胎瘤并手术切除。8例给予一线免疫治疗。全部病例经免疫治疗后病情缓解。缓解期2例抗NMDA受体抗体滴度阳性,2例脑电图异常。结论抗NMDA受体脑炎具有复发性,其复发的原因可能与肿瘤、病毒等有关。临床表现、抗体检测和神经影像学改变等为诊断和评估复发的重要方法。长时间的小剂量免疫治疗是预防复发的关键,同时需要遵循个体化的原则。     

Cerebral Whipple's disease with a stroke-like presentation and cerebrovascular pathology

Although neurological symptoms are common in Whipple's disease, patients rarely have a purely neurological presentation and involvement restricted to the central nervous system is uncommon. A 39 year old woman presented with a meningoencephalitic illness, which responded to penicillin. Eleven months later she developed recurrent stroke-like episodes. Patchy enhancing meningeal, cortical, and subcortical lesions thought to be vascular in origin developed within nine days of the onset of symptoms. No evidence was found of a cardiovascular source of emboli, vasculitis, or thrombophilic condition. A brain biopsy showed meningoencephalitic features suspicious of Whipple's disease associated with leptomeningeal arterial fibrosis and thrombosis. DNA polymerase chain reaction confirmed Tropheryma whippelii in both blood and brain tissue. The neurological manifestations of cerebral Whipple's disease are varied and very rarely include stroke-like symptoms. The pathogenesis of cerebral infarction in Whipple's disease is not well established but arterial fibrosis and endocarditis complicated by embolisation have been reported. This case emphasises the importance of early brain biopsy in unusual cases of stroke and illustrates the clinical utility of polymerase chain reaction to confirm Whipple's disease.     

Magnetic resonance restricted diffusion resolution correlates with clinical improvement and response to treatment in herpes simplex encephalitis

Introduction: A 34-year-old man presented with herpes simplex encephalitis (HSE), with magnetic resonance imaging (MRI) showing dense foci of restricted diffusion in the temporal lobe. Case Report: With treatment and clinical improvement, follow-up MRI done 8 days later showed complete resolution of the restricted diffusion abnormalities, whereas other MRI sequences suggested interval progression. Discussion: Restricted diffusion abnormalities on MRI in patients with HSE may be more sensitive to and correlate better with disease activity in HSE.     

视神经脊髓炎合并抗N-甲基-D-天冬氨酸受体脑炎1例

目的 探讨视神经脊髓炎合并抗N-甲基-D-天冬氨酸(NMDA)受体脑炎的临床特征。方法 报道本院收治的1例视神经脊髓炎合并抗N-甲基-D-天冬氨酸受体脑炎的临床特点及诊治经过,并结合文献分析该病的特征。结果 患者为21岁女性,表现为腹泻后急性起病的下肢麻木无力、昏睡、视物模糊; MRI示双侧基底节、海马、桥臂、齿状回、延髓背侧多发异常信号,同时伴有颈髓多发条片状长T₂异常信号。脑脊液NMDA受体抗体阳性,血清和脑脊液水通道蛋白4(AQP4)抗体阳性,合并心动过缓并发症,筛查未发现肿瘤; 经激素冲击、丙种球蛋白及免疫抑制剂治疗后症状逐渐好转至痊愈。结论 视神经脊髓炎合并抗N-甲基-D-天冬氨酸受体脑炎可以在感染后起病,症状和影像学主要表现为两种疾病的叠加,可出现心动过缓并发症,早期积极行免疫治疗效果较好。     

抗N-甲基-D-天冬氨酸受体脑炎一例

目的 探讨抗N-甲基-M-天冬氨酸(NMDA)受体脑炎的临床特点、诊断及治疗.方法报道1例伴卵巢畸胎瘤的抗NMDA受体脑炎患者的临床表现及诊治过程,并总结其临床特征.结果患者为青年女性,表现为精神行为异常及抽搐,意识障碍及通气不足.早期发现畸胎瘤,脑脊液中抗NMDA受体抗体阳性.行畸胎瘤手术切除,联合静脉用免疫球蛋白和激素冲击治疗,患者临床痊愈.结论 抗NMDA受体脑炎是一种自身免疫性的、能用血清学方法诊断的疾病.早期明确诊断和给予及时治疗,预后良好.