Relapse of herpes simplex encephalitis

This report describes a child with herpes simplex virus (HSV) encephalitis who improved dramatically while being treated with acyclovir but subsequently had neurological deterioration and died. A severe necrotizing process was present in the brain at autopsy but there were no focal areas of demyelination and poor inflammatory response. HSV was not cultured from brain biopsy during relapse or autopsy. Fourteen previous cases of relapsing herpes encephalitis are reviewed and treatment regime and mechanisms of relapse are discussed.     

Meningioangiomatosis: report of two cases and literature review

Meningioangiomatosis (MA) is a rare benign intracraneal lesion. The majority of cases are sporadic although the association of this lesion with familial neurofibromatosis (NF) type 2 is well known. NF-associated MA may be multifocal and is often asymptomatic and diagnosed at autopsy. Non-associated cases are usually symptomatic, occurs in children and young adults and frequently arise in leptomeninges and underlying cerebral cortex. In the present work, we describe two new non-associated cases of MA in two boys, seven and one year old with seizures that disappeared after surgical excision. Histopathologically, the lesion was predominantly cellular in one case and more fibrous in the other. From the literature review we concluded that sporadic cases present as single lesions which manifest by seizures or persistent headaches. Rarely MA has been described to coexist with meningiomas. Histopathologically, MA is characterized by a plaque-like proliferation of meningothelial and fibroblast- like cells surrounding small vessels and trapping islands of gliotic cortical tissue. The lesion does not show significant atypia, mitosis or necrosis. Although all cases of MA share unifying features, there are different degrees of histological presentation with cases predominantly cellular and others more fibrous and calcified. This could correspond to different stages in the evolution of the MA. Symptoms disappear with the complete excision of the lesion.     

Combined therapy with acyclovir and adenosine arabinoside in herpes simplex encephalitis

We report a case of herpes simplex encephalitis (HSE) with initial coma and severe left-sided hemiparesis in which combined treatment with adenine arabinoside and acyclovir was followed by complete recovery. This favorable result is discussed in view of the literature of HSE treatment including experimental studies on antiviral activity of both drugs. Combined treatment may be useful in severe cases of HSE.     

烫吸海洛因致白质脑病2例报告及文献复习

目的 研究烫吸海洛因蒸汽引起海绵状白质脑病的临床表现及影像学特征，探讨吸毒方式与病理改变的关系。方法 对2例烫吸海洛因引起的海绵状白质脑病的患者进行了临床和MRI观察，复习中外文献，对该病的临床表现、影像学特征、病理组织改变、吸毒方式与病理机制之间的关系进行了总结与讨论。结果 2例患者均采用烫吸的方式摄入海洛因，时间为1．5年、8年，均在戒毒数日后发病，以小脑性共济失调、智力减退为主要临床表现。MRI显示对称性小脑齿状核、双大脑半球半卵圆中心、胼胝体压部、内囊后肢、内侧丘系、红核、黑质、脑桥长T1T2信号改变，给予地塞米松、脑多肽、胞二磷胆碱、B族维生素、高压氧、低激光血疗等治疗有近期疗效。结合文献资料显示本病可能与海洛因的烫吸方式有关，提出突然戒断与发病的可能关系。结论 烫吸海洛因引起海绵状白质脑病临床表现、影像学改变具有特征性，从而使本病能够得到早期确诊。     

Meningo encephalitis revealing Q fever: two cases and a review of the literature

Q fever is a zoonosis caused by Coxiella burnetii. The acute Q fever is usually characterized by a self-limited flu-like syndrome, fever, pneumonia and hepatitis. Symptoms of the chronic Q fever (evolution>3 months) mainly consist of endocarditis with negative culture. Focal neurological symptoms are rarely observed. Neurological symptoms of acute Q fever consist of meningitis or meningo-encephalitis. Neurological symptoms of chronic Q fever are cerebral embolisms from Coxiella burnetii infected heart valves. We herein report two patients with meningoencephalitis revealing acute Q fever.     

Intraparenchymal schwannomas: report of two new cases studied with MRI and review of the literature

Intraparenchymal schwannomas are very rare tumours. We present two young adult patients operated for this type of lesion who show no signs of recurrence 2 years after surgery. These tumours have a bimodal peak of presentation: most occur in young patients under 25 years, and the rest present in the elderly. Characteristically they show both Antoni A and Antoni B areas, intense inmunoreactivity to S-100 and Vimentin protein, and none to EMA or CD34. Electron microscopy is diagnostic when basal membrane is found around the cytoplasmatic processes. MRI spectroscopy depicts increased myoinositol, choline and lipids, and perfusion MR demonstrates high rCBV with a characteristic curve due to the total absence of blood brain barrier. An origin in the Schwann cells of the perivascular nervous plexus in the subarachnoid space is the most accepted theory for the histogenesis of these tumours. We propose to perform the characterization of a series of markers such as SOX-10 in every new case in order to prove that theory.     

尿毒症患者透析后舞蹈样并发症二例分析及文献复习

目的探讨尿毒症患者透析后舞蹈样并发症的临床表现和治疗方法。方法选择2例典型患者进行临床表现及影像学结果回顾性分析。结果尿毒症血透患者维生素B_1缺乏可由多种原因引起,常见于摄入过低和透析中丢失过多。维生素B_1不足,三羧酸循环紊乱,血乳酸增加,从而导致机体出现一系列相关症状。其临床表现多为威尼克氏脑病。结论对尿毒症血透发生基底神经节功能紊乱所引发的舞蹈样症的患者,诊断时应加强对本病的认识。血乳酸和脑基底节影像学检查可为本病提供诊断依据。维生素B_1静脉疗法对本病有明显的缓解作用。     

Radio-induced low-grade glioma: report of two cases and review of the literature

With the increasing number of cancer survivors, we can observe a population that will present a higher risk of developing secondary long-term toxicities related to adjuvant chemo and radiotherapy regimens. Among these, children surviving from acute lymphoblastic leukemia (ALL) that were treated with prophylactic cranial irradiation represent a group of patients at a high risk of developing secondary brain tumors. Radiation-induced intracranial tumors have been documented since 1950, and today, more than one-hundred cases have been described. We report our experience with two young patients who were hospitalized for low grade gliomas and had a positive anamnesis for ALL and consequent radiotherapy.     

Acute herpes simplex encephalitis treated with acyclovir. Report of 5 cases]

5 cases of acute herpes simplex encephalitis were verified by specific IgG antibodies in cerebrospinal fluid and serum among 31 cases with 'sporadic encephalitis' within one year. All were treated with acyclovir, 4 with remarkable response--2 cured and 2 significantly recovered, except one died due to delayed specific treatment. The dosage of acyclovir used by the author was 5mg/kg/12-24 hours.     

复发性Fisher-Bickerstaff综合征2例临床分析

目的探讨复发性Fisher综合征(FS)叠加Bickerstaff脑干脑炎(BBE)的临床特点和疾病命名。方法分析2例复发性Fisher-Bickerstaff综合征的患者的临床资料,并复习相关文献。结果例1表现为复发性睡眠增多、走路不稳、视物成双,肌电图检查提示下肢神经源性损害;例2表现为复发性视物成双、走路不稳,第2次发病出现四肢无力,查体双下肢远端肌力3级,双侧Babinski征阳性,肌电图提示四肢神经源性损害;2例患者除具有眼肌麻痹和共济失调的症状外,都有中枢和周围神经受损的表现;依据患者的病史、查体、临床检测和疾病经过,诊断为复发性Fisher-Bickerstaff综合征,第2例患者叠加吉兰-巴雷综合征。结论具有周围神经损害的BBE和具有中枢神经损害的FS从临床表型上不能截然分开,Fisher-Bickerstaff综合征的概念更能准确地表述这类患者的表型特点。     

抗N-甲基-D-天冬氨酸受体脑炎2例报道并文献复习

目的探讨抗N-甲基-D-天冬氨酸受体脑炎患者的临床特点及诊治。方法收集2例我院诊治的抗N-甲基-D-天冬氨酸受体脑炎患者的临床资料,并通过计算机检索中国知网、万方数据库,收集2010年至2013年在我国各种期刊上公开发表的关于抗N-甲基-D-天冬氨酸受体脑炎患者的文献。结果本文报道的2例及9篇文献检索的23例患者主要表现为发热、精神行为异常、智能减退、抽搐、不自主运动及表现为睡眠减少、心率快、汗液分泌异常等自主神经功能障碍。本文报道的2例患者男女各1例,年龄分别为15.5岁、14岁,均未发现畸胎瘤;文献检索的23例患者女性14例、男性9例,合并畸胎瘤5例。所有病例均经化验血液和(或)脑脊液抗NMDA受体抗体阳性而确诊,脑脊液可见以淋巴细胞为主的白细胞数轻度升高。头MRI检查及脑电图无特异性所见。本文报道的2例患者尚发现血液抗链球菌溶血素O均升高。本文报道的2例患者及文献检索的20例患者经大剂量激素联合丙种球蛋白治疗后(20/23,86.9%)症状明显改善,2例症状改善不明显,1例死亡。结论抗N-甲基-D-天冬氨酸受体脑炎临床表现无特异性,早期易误诊为精神障碍性疾病,脑脊液抗NMDA受体抗体检测有助于早期诊断和治疗,大部分患者免疫治疗有效。本文报道的2例病例尚合并ASO升高,文献中未见报道。     

自身免疫性脑炎再发7例并文献复习

目的分析自身免疫性脑炎(Autoimmune encephalitis;AE)再发患者的临床特点、诊治及再发原因。方法回顾2016年1月~2017年12月吉林大学第一医院神经内科收治的所有AE,分析2次及以上症状再发者的临床表现、神经影像学及脑脊液(CSF)等的特点、治疗方案、疾病转归,并与其他患者进行比较。结果符合标准的AE共47例,包括抗NMDAR脑炎17例,平均年龄36岁(17~69岁);抗GABABR抗体相关脑炎11例,平均年龄61岁(47~80岁);抗LGI1抗体相关脑炎19例,平均年龄57岁(33~78岁)。其中再发者7例,男5例、女2例,包括抗NMDAR脑炎3例(占17.6%),抗GABABR抗体相关脑炎3例(占27.3%),抗LGI1抗体相关脑炎1例(占5%)。首次发病的起始症状包括癫痫发作5例、记忆力减退1例、精神行为异常1例。6例患者为1次再发,1例2次再发,多数为多症状再发。发病间隔0.5~4 m,平均3.5 m。再发的起始症状为:发作性抽搐4例、记忆力减退2例、意识水平下降及双下肢麻木各1例。首次发病时头MRI异常者5例,再发时原有病灶消失或出现新病灶。两次发病时脑电图除2例未完成外均异常。首次发病时均未筛查到肿瘤,1例于再发时发现中心型肺癌伴转移。CSF或血清相关抗体阳性率为100%,经一线免疫等治疗后病情缓解。结论 AE中抗NMDAR脑炎、抗GABABR抗体相关脑炎、抗LGI1抗体相关脑炎症状好转或消失后均可再发,平均间隔时间为3.5 m,多数为多症状再发。判断复发主要依据症状学、CSF相关抗体检测、神经影像学等。AE复发的危险因素可能为免疫治疗不规范、首次未发现肿瘤、病情严重、激素减停过快、合并多重抗体及伴有炎性脱髓鞘改变者。     

Delayed brain abscess after embolization of arterio-venous malformation: report of two cases and review of literature

Infections of central nervous system are rare complications of endovascular procedures. Review of literature revealed only four reported cases of brain abscesses after embolization of intracranial arteriovenous malformations (AVMs). We report two new cases of delayed brain abscess after embolization of AVM. In one of the patients, it was due to an unusual organism, Burkholderia caeci.     

Neurosarcoidosis mimicking meningioma en-plaque: report of two cases and review of the literature

We describe two patients who presented with seizures and in whom imaging studies showed extra-axial disease which was simulating meningioma en-plaque. The diagnosis of neurosarcoidosis was obtained by meningeal biopsy. The clinical and MRI features of neurosarcoidosis presenting as an extra-axial mass lesion are reviewed. In cases in which clinical or radiological features place the diagnosis of meningioma in doubt, frozen section biopsy is advocated to avoid unwarranted excision of a lesion caused by sarcoidosis.     

Isolated vestibular nuclear infarction: report of two cases and review of the literature

Cerebral infarction presenting with isolated vertigo remains a diagnostic challenge. To define the clinical characteristics of unilateral infarctions restricted to the vestibular nuclei, two patients with isolated unilateral vestibular nuclear infarction had bedside and laboratory evaluation of the ocular motor and vestibular function, including video-oculography, bithermal caloric irrigation, the head impulse test (HIT) using magnetic scleral coils, and cervical and ocular vestibular-evoked myogenic potentials (VEMPs). We also reviewed the literature on isolated vertigo from lesions restricted to the vestibular nuclei, and analyzed the clinical features of seven additional patients. Both patients showed spontaneous torsional-horizontal nystagmus that beat away from the lesion side, and direction-changing gaze-evoked nystagmus. Recording of HIT using a magnetic search coil system documented decreased gains of the vestibular-ocular reflex for the horizontal and posterior semicircular canals on both sides, but more for the ipsilesional canals. Bithermal caloric tests showed ipsilesional canal paresis in both patients. Cervical and ocular VEMPs showed decreased or absent responses during stimulation of the ipsilesional ear. Initial MRIs including diffusion-weighted images were normal or equivocal, but follow-up imaging disclosed a circumscribed acute infarction in the area of the vestibular nuclei. Infarctions restricted to the vestibular nuclei may present with isolated vertigo with features of both peripheral and central vestibulopathies. Central signs should be sought even in patients with spontaneous horizontal–torsional nystagmus and positive HIT. In patients with combined peripheral and central vestibulopathy, a vestibular nuclear lesion should be considered especially when hearing is preserved.     

Primary intradural extramedullary ependymoma: report of two cases and review of the literature

Primary intradural extramedullary ependymomas are very rare. They are called primary in this location as they do not have any connection with the central nervous system. They usually develop from an ectopic ependymal cell nest. To the best of our knowledge, only 10 cases have been described in the literature. We report two cases of large sausage-shaped intradural extramedullary ependymoma in the lumbosacral area.