Rasmussen脑炎的研究进展

Rasmussen脑炎(Rasmussen’s encephalitis,RE)是一种罕见的神经系统慢性疾病,又称为Rasmussen综合征。该病多起源于儿童期,常累及一侧大脑半球,主要以药物难治性癫痫、部分性癫痫持续状态、进展性神经功能缺损(言语、运动及认知功能障碍等)为主要特点。目前神经病理学及免疫学机制尚不明确。影像学检查对其早期诊断有指导意义。该病症状重,致残率高,抗癫痫药物治疗的疗效较差,激素及免疫抑制剂等药物治疗不能从根本上阻止疾病的进展,大脑半球切除术仍是目前唯一治愈该病的方法。     

Rasmussen脑炎临床病理研究

目的 探讨Rasmussen脑炎的的发病机制、临床表现、神经影像学表现和病理特征.方法 结合临床、影像、HE和免疫组化方法对6例大脑半球切除的Rasmussen脑炎患者的资料进行回顾分析(手术时年龄3.5 ～11.0岁).结果 患者均为难治性癫痫,患病时间1-4年,均有不同程度偏瘫.术后随访4个月-3年8个月,预后均良好,正规服用抗癫痫药物,均无癫痫发作.组织病理学上所有6例均可见局灶软脑膜下星形胶质细胞增生,脑实质淋巴细胞及小胶质细胞结节散在分布,5例脑实质血管周围慢性淋巴细胞浸润,淋巴套袖形成,所有淋巴细胞以T淋巴细胞为主(CD3+,CD5+,CD7+),且有细胞毒性T淋巴细胞为主(CD8+,GranzymeB+,CD4-),B淋巴细胞罕见(CD79a-,CD20-).未见病毒包涵体.结论 Rasmussen脑炎中淋巴细胞为细胞毒性T细胞来源,患侧大脑半球切除是治疗Rasmussen脑炎、阻止疾病进展的有效的方法.     

Rasmussen脑炎的发病机制研究进展

Rasmussen脑炎以难治性癫(癎)、进行性偏瘫及智力减退为临床特征,多见于儿童,影像学主要表现为病变侧大脑半球的进行性弥漫性萎缩,主要病理学特点是神经细胞脱失、胶质细胞增生及血管周围淋巴细胞浸润.该病病因及发病机制未明,病毒感染、机体自身抗体的产生及脑组织血管周围T淋巴细胞浸润可能与其发病及疾病进展过程相关.本文即就以上几个方面对该病的发病机制研究进行综述.     

结核性脑膜脑炎的临床及影像学特点(附1例报道及文献复习)

目的探讨结核性脑膜脑炎(TBM)的临床及影像学特点。方法通过1例结核性脑膜脑炎的临床表现、MR I改变,并结合相关文献进行讨论分析。结果患者临床表现较轻且不典型,影像学上表现为病灶弥漫分布,累及幕上脑实质、脑干、小脑多个部位。结论结核性脑膜脑炎临床表现多样影像学表现根据时期不同也各有差异,通过常见临床及影像学特点综合分析,结合实验室检查及诊断性抗结核治疗有效有助于诊断。脑脊液镜下发现或培养出结核杆菌是诊断金标准。     

Rasmussen脑炎(附1例报告及文献复习)

目的 探讨Rasmussen脑炎的临床特点及诊断。方法 回顾分析1例Rasmussen脑炎患儿的临床资料,并复习相关文献进行分析。结果 女性,7岁。起病时表现为间断右侧眼睑抽动伴右侧肢体抖动,起病后有认知损害,予抗癫■药及免疫抑制剂等治疗病情好转。病程约6个月时加重,出现累及右侧面部及右侧肢体的局灶性癫■持续状态和语言功能障碍。患儿有定向障碍,记忆力减退,右侧肢体肌力4级,右侧巴氏征阳性。头颅MRI病初时仅有左侧额叶局部异常信号,一过性累及左侧颞叶,逐渐发展为左侧大脑半球萎缩。脑电图可见左侧半球弥漫性慢波,夹杂尖波、棘波发放。行左侧额、顶、岛叶癫■灶切除术及大脑半球离断术后,患儿再无癫■发作。能独走,右侧肢体肌力4级,精细动作差,能说简单句。结论 临床表现为局灶性癫■持续状态、进行性神经功能和认知损害,头颅影像学表现为大脑皮质进行性局部萎缩,脑电图表现为一侧大脑半球慢波可伴尖波、棘波,应考虑Rasmussen脑炎。     

Rasmussen脑炎1例并文献复习

对我科室2014年年底收入的1例青年女性Rasmussen脑炎患者的诊疗经过及治疗措施进行分析,总结自身不足并结合文献复习Rasmussen脑炎。Rasmussen脑炎是一种好发于儿童、青少年的罕见疾病,其病因和发病机制不清,实验室检查结果无特异性,确诊主要依据临床表现、磁共振检查(MRI)、脑电图(EEG)以及脑组织活检。该病总体预后不佳,免疫治疗可能有用,大脑半球切除术是目前唯一治愈Rasmussen脑炎引起癫痫的方法。对于相对大龄的青年患者,不应因未出现脑萎缩而过早排除RE的诊断。     

核磁共振表现为颞叶内侧及海马异常信号的神经梅毒1例及相关文献复习

目的 探讨核磁共振表现颞叶内侧信号异常的神经梅毒的临床特征。方法 通过总结本院近期收治的1例及国内外文献报道确诊的28例核磁共振表现为颞叶内侧信号异常患者的临床资料,分析该病的临床特点。结果 患者主要表现为认知障碍、癫痫、精神行为异常等。影像学检查与边缘叶脑炎典型征象无明显差别。血清及CSF梅毒特异性检查阳性。结论 神经梅毒与边缘叶脑炎有相似临床表现及影像学征象,确诊需检查梅毒特异性抗体。尽早青霉素或头孢曲松治疗可显著改善预后。     

脑炎性假瘤的临床、影像学及病理特征(附1例报告)

目的探讨脑炎性假瘤的临床、影像学及病理特征。方法回顾性分析1例脑炎性假瘤患者的临床资料。结果本例脑炎性假瘤患者临床表现为头痛、恶心、呕吐、发热、抽搐发作等,头部MRI显示右侧额叶大片长T1、长T2信号,中线移位。病理检查可见脑组织肿胀,正常神经元轮廓消失,血管周围大量炎细胞浸润。经手术、脱水、抗感染等治疗无效,死亡。结论脑炎性假瘤病例少见,临床表现和影像学改变无特异性;病理学检查是诊断的主要依据;本病手术治疗效果不好。     

以智能下降为首发症状合并松果体区肿瘤的Rasmussen脑炎1例并文献复习

正Rasmussen脑炎(Rasmussen encephalitis,RE)又称Rasmussen综合征(Rasmussen syndrome,RS),是一种罕见的后天获得性进展性慢性炎症性脑疾病,以一侧大脑半球损害引起的难治性癫痫、进行性偏瘫和智能减退为主要临床特征~([1])。典型RE通常以癫痫为首发临床表现,以难治性部分发作性癫痫(包括癫痫持续状态,Epilepsia partialis continua,     

假瘤型病毒性脑炎

目的 提高对假瘤型病毒性脑炎的认识。方法 报告11例经手术或活检病理证实的假瘤型病毒性脑炎,并结合文献对本病的临床、病理、影像学表现、诊断与鉴别诊断以及治疗进行讨论。结果 本病临床经过和影像学特征与脑肿瘤相似,本组11例患者误诊为脑胶质瘤9例,病理证实均为病毒性脑炎。结论 本病的确诊有赖于是学检查。手术切除病灶和综合性药物治疗是治疗本病的有效手段。     

Rasmussen encephalitis with active inflammation and delayed seizures onset

Severe focal epilepsy is regarded as a clinical hallmark of Rasmussen encephalitis (RE). The authors report two children with progressive hemiparesis, contralateral hemispheric atrophy, and pathologic features characteristic for RE. At histologic diagnosis and over several months, neither patient experienced seizures. The report enlarges the clinical spectrum of RE and suggests that seizures are not an obligatory presenting symptom of the disorder.     

Absence of seizures in Rasmussen encephalitis with active inflammation

Severe focal motor epilepsy is considered a clinical hallmark of Rasmussen encephalitis (RE). The authors report a 6-year-old girl with progressive right sided hemiparesis, loss of language skills, left sided hemispheric atrophy, and brain pathologic features characteristic for RE. The patient did not experience seizures over a 2 year period after symptom onset and for several months during follow-up. This report expands the clinical spectrum of RE and suggests that seizures are not a universal symptom of RE. Our patient’s quite remarkable neurologic deficits along with active inflammation in the absence of epilepsy supports that, at least in some individuals, unilateral hemispheric progressive inflammation can occur without active seizure activity.     

Finding on brain MRI mimicking focal cortical dysplasia in early Rasmussen's encephalitis: a case report and review

Introduction  

Rasmussen's encephalitis (RE) is one of the important causes of refractory seizure. The most impressive clinical manifestation of RE is epilepsia partialis continua (EPC). Others include progressive hemiparesis and neuropsychological deterioration. Currently, the best approach to RE is hemispherectomy.     

Dual pathology in Rasmussen's encephalitis: A study of seven cases and review of the literature

Dual pathology has previously been reported in less than 10% of cases of Rasmussen's encephalitis (RE). Given the rarity of RE, it appears unlikely that dual pathology in RE is merely a coincidence. We therefore reviewed all cases of RE experienced in our institution to assess for an additional/associated pathology. A total of seven patients with RE were identified in our archives. Seven children (4 boys and 3 girls, age range: 3–16 years, mean: 9.5 years) with medically refractory epilepsy underwent surgical resection for intractable seizures. The surgical specimens were examined with routine neurohistological techniques, and immunohistochemistry was performed with an extensive panel of antibodies for viruses, lymphocytes, microglia/macrophages, human leukocyte antigen (HLA)‐DR, astrocytes, and neurons. Relevant literature was reviewed. Microscopically, all seven cases demonstrated the inflammatory pathology of RE in the cortex and white matter with leptomeningeal and perivascular lymphocytic infiltration, microglial nodules with/without neuronophagia, neuronal loss and gliosis. The HLA‐DR antibody was extremely helpful in highlighting the extent of microglial cell proliferation/activation that was not appreciable with standard histology. An unexpected finding in all seven cases was the presence of cortical dysplasia. In our series of seven cases, there was co‐occurrence of the inflammatory/destructive pathology of RE with malformative/dysplastic features in cortical architecture in 100% of cases, raising questions about the possible relationships between the two entities. Awareness of the possibility of dual pathology in RE is important for clinical and pathological diagnosis, and may affect the management and outcome of these patients. Immunohistochemistry is very helpful to make a definitive diagnosis of both pathologies.     

Can head trauma trigger adult-onset Rasmussen's encephalitis?

Rasmussen's encephalitis (RE) is a rare unilateral inflammatory brain disorder that causes progressive neurocognitive deterioration and refractory epilepsy including epilepsia partialis continua (EPC). We describe a patient with a unique presentation, where right upper limb EPC due to RE began within 2 weeks of a concussive left frontal head injury, in a 36-year-old female without other identifiable etiology, no prior neurological deficit nor suggestion of intracranial pathology or infection, and no preceding seizures. The diagnosis of RE followed extensive investigation, excluding confounding diagnoses, with supportive histopathology, and her EPC has proven refractory to treatment. In the absence of a satisfactory alternative etiology and exclusion of differential diagnoses, the most likely cause or precipitant of this patient's RE was head trauma.     

Probable Rasmussen's syndrome. Case report

The syndrome of chronic encephalitis with epilepsy (Rasmussen's syndrome) typically occurs in children and is characterized by the development of intractable focal seizures, progressive hemiparesis and intellectual deterioration. The etiology is unknown, and the pathological abnormalities vary. We report the case of a 17-year-old boy, presenting with clinical features typical of probable Rasmussen's syndrome. We discuss the etiology and treatment for this pathology.     

Development of anaplastic ependymoma in Rasmussen’s encephalitis: review of the literature and case report

Background Rasmussens encephalitis (RE) is a form of chronic encephalitis presenting with intractable seizures and progressive neurological deficits in children. The occurrence of anaplastic ependymoma in a diagnosed case of RE has not been reported in the literature.Case report We report an eight and a half year-old boy, a diagnosed case of RE, suffering from intractable right-sided focal seizures, who developed features of raised intracranial pressure during follow-up. Repeat imaging revealed left fronto-temporoparietal mass for which near-total decompression was done. Histopathology of the mass revealed anaplastic ependymoma.Conclusion The etiology of such a co-existence or development of malignancy in a pre-existing RE is yet to be completely understood. Both these entities could have a common etiology of viral- or autoimmune-mediated process, but no definite conclusion can be drawn with all the literature available at the moment.     

Electrical status epilepticus "invisible" to surface EEG in late-onset Rasmussen encephalitis

Rasmussen's encephalitis (RE) is a chronic, progressive disease that typically occurs in childhood, rarely in adulthood. When it does occur in adulthood, it may be associated with atypical electro-clinical features, and neuroimaging alterations usually help diagnosis. Unlike childhood forms, in which epilepsia partialis continua is usually observed, the EEG pattern in adult variants may be aspecific. We describe a highly interesting case of late-onset RE in which an electrical status epilepticus was not detected by the surface EEG, but by a recording with subdural electrodes.