Fusion of the cerebellar hemispheres ventral to the brainstem: a rare hindbrain-related malformation

Introduction New variations of cerebellar malformations are being increasingly recognized using modern neuroimaging techniques. Until now only dorsal cerebellar fusion syndromes, such as the rhombencephalosynapsis and its variations have been recognized.Case report We report on a 17-month-old male infant presenting with an exceptional hindbrain related malformation characterized by midline fusion of the cerebellar hemispheres ventral to the brainstem and causing symptoms due to brainstem compression.Discussion and conclusion A more detailed understanding of the cerebellar embryogenesis is required to unravel the underlying mechanisms leading to this type of cerebellar malformation, which cannot easily be integrated into the common classification systems. Both the morphological features and the clinical presentation are different from those of other cerebellar structural abnormalities. If this type of congenital malformation is detected more frequently in the future, it seems reasonable that it should be added to the list of cerebellar malformations as a distinct type.     

Meningovascular form of neuroborreliosis: similarities between neuropathological findings in a case of Lyme disease and those occurring in tertiary neurosyphilis

Summary Recent observations have delineated the neurological manifestations of Lyme disease, but, to our knowledge, no detailed neuropathological study from autopsy cases has been reported. In this report we describe the neuropathological findings in a case of Lyme neuroborreliosis. The chronic meningitis, the occlusive meningo vascular and secondary parenchymal changes that we found are similar to those occurring in the meningovascular form of neurosyphilis. Thus, we suggest that the case described here represents the meningovascular form of tertiary Lyme neuroborreliosis.     

石家庄地区中枢神经系统感染的住院资料分析

目的探讨中枢神经系统感染性疾病的发病特点。方法对我院1996-2005年间收治的中枢神经系统感染性疾病的住院病例进行回顾性分析。结果10年间我院共收治中枢神经系统感染性疾病的患者3929例，其中病毒性脑（膜）炎2506例（63．8％），脑囊虫病682例（17．4％），结核性脑膜炎（简称结脑）411例（10．4％），化脓性脑膜炎（简称化脑）303例（7．7％），隐球菌性脑膜炎（简称隐脑）27例（0．7％）。2001-2005年，病毒性脑（膜）炎、结脑、化脑、隐脑的病例数较前5年分别增加了53．5％、21．3％、35．3％、77．3％。脑囊虫病病例数较前5年下降了32．4％。病毒性脑（膜）炎主要集中在7～9月份，结脑主要集中在4～6月份。病毒性脑（膜）炎、脑囊虫病、结脑、化脑、隐脑的病死率分别为8．1％、6．9％、22．4％、12．9％、33．3％。结论近5年来，中枢神经系统感染性疾病在河北省石家庄市附近局部有增加的趋势，应该提高警惕，做好防治工作。     

手足口病患儿合并中枢神经系统损害临床分析

目的：探讨手足口病患儿合并中枢神经系统损害的临床特点及治疗方法。方法选取合并中枢神经系统损害的手足口病患儿137例,随机分为2组。回顾分析所有患者的临床特点、试验时间、检查结果,并进行统计。2组患儿均实施综合治疗,在此基础上,对照组给予胞二磷胆碱静滴,观察组给予神经节苷脂静滴。对2组患儿神经系统好转时间、发热缓解时间及痊愈时间进行统计。于治疗10 d时测定2组患儿神经元特异性烯醇化酶水平,评价2组患儿治疗效果。结果发热、皮疹、易惊是手足口病患儿合并中枢神经系统损害最为常见的临床表现;脑脊液细胞数升高是实验室检查中最常见的异常。观察组患者神经系统好转时间、发热缓解时间、痊愈时间及神经元特异性烯醇化酶水平均明显低于对照组。结论手足口病患儿合并中枢神经系统损害的临床表现缺乏特异性,治疗时应在综合治疗的基础上加用神经节苷脂静脉注射。     

获得性免疫缺陷综合征神经系统损害临床分析

目的 提高对获得性免疫缺陷综合征(艾滋病)合并神经系统损害临床特点的认识,以减少漏诊.方法 对28例人类获得性免疫缺陷病毒(HIV)感染和(或)艾滋病患者中的12例合并神经系统损害患者的临床资料和机会感染性疾病情况进行回顾,并结合文献分析总结.结果 12例患者分别诊断为艾滋病脑病(5例)、慢性脑膜炎(3例)、周围神经病...     

鼠神经生长因子治疗手足口病合并中枢神经系统损害的研究

目的 探讨鼠神经生长因子在手足口病合并中枢神经系统损害患者治疗中的应用.方法 收集北京地坛医院自2008年5月至2010年9月收治的手足口病合并中枢神经系统损害并使用鼠神经生长因子治疗的48例患者的临床资料,采用回顾性分析方法总结其特点.结果 5岁以下尤其是1～3岁儿童是手足口病合并中枢神经系统损害高危人群.患者急性弛缓性麻痹症状出现时间平均在病程第(7.1±3.6)天,肌力恢复平均在第(17.5±6.0)天;呕吐症状出现和消失的平均时间分别为病程第(4.29±3.10)天和第(5.74±2.90)天;易惊症状出现和消失的平均时间为病程第(2.67±1.76)天和第(7.80±2.76)天.实验室检查显示患者白细胞及CK值均有不同程度升高,脑脊液压力、蛋白表达不同程度升高MRI提示延髓部位最常受累,其次为脑干等部位.结论 鼠神经生长因子应用于手足口病合并中枢神经系统损害患者时有助于改善患者的神经系统症状及预后.

Abstract：

Objective To investigate the application of mouse nervous growth factor (mNGF) on patients with hand-foot-and-mouth disease (HFMD) combined with central nervous system damage. Methods Clinical data of 48 patients with HFMD combined with central nervous system damage, admitted to our hospital and received treatment of mNGF from May 2008 to September 2010, were analyzed retrospectively. Results Children under 5 years old, especially between 1-3 years old, were the high-risk group of having HFMD combined with central nervous system damage. The average time of appearing of acute flaccid paralysis (AFP) and recovering of muscle strength were (7.1±3.6) d and (17.5±6.0) d, respectively. A total of 31 patients (64.58%) appeared vomiting, with its average times of appearance and disappearance as (4.29±3.10) d and (5.74±2.90) d, respectively. A total of 33 patients (68.75%) tended to appear panic symptoms, which appeared on (2.67±1.76) d and disappeared on (7.80± 2.76) d averagely. Laboratory test showed number of white blood cells and CK values were elevated of certain degree, and cerebrospinal fluid pressure and protein expression level were increased to varying degrees. MRI showed that bulbus medulla was the most likely to be involved, followed by brain stem and other parts. Conclusion Application of mNGF on patients with HFMD combined with central system damage may help to improve the neurologic symptoms and prognosis.     

手足口病致迟缓性麻痹合并中枢神经系统感染的肌电图分析

目的探讨手足口病致迟缓性麻痹合并中枢神经系统感染患儿的肌电图特点。方法对75例手足口病致迟缓性麻痹患儿按是否合并中枢神经系统感染分为2组,即感染组45例和未感染组30例。结果感染组肌电图异常44例,异常率98%;未感染组肌电图异常18例,异常率60%。结论手足口病致迟缓性麻痹患儿合并有中枢系统感染者肌电图异常率明显高于未合并感染者。     

不典型性中枢神经系统Whipple病:一例报告并文献复习

目的结合文献探讨中枢神经系统Whipple病的诊断与治疗特点,以提高对该病的认识。方法回顾分析1例以头痛、左侧肢休无力,伴记忆力减退为首发症状的不典型性中枢神经系统Whipple病的临床诊断与治疗经过,并进行文献复习。结果女性患者,35岁。首发症状表现为头痛、肢体无力及记忆力减退,但不伴发热、癫癎发作。病程进展过程中相继出现阵发性四肢抽动、右侧下肢无力、小便失禁、多食、体质量增加、停经、体温波动,大剂量糖皮质激素及青露素、复方磺胺甲噁唑等抗炎药物治疗无效,随着颅内压逐渐升高,脑疝形成。腰椎穿刺脑脊液检测仅蛋广白定量显著升高。脑电图提示右侧前额颞区慢波。MRI呈以右侧大脑半球、额顶颢叶、半卵圆中心及基底节为主的大片长T₁、长T₂信号,并不均匀疏松团状强化,病灶周围水肿,占位效应明显,并累及左侧大脑半球。病理学检查呈现大片状坏死,脑组织及血管周围大量淋巴细胞和浆细胞浸润,伴大量格子细胞渗出,胞质丰富,内含大量六胺银和PAS染色阳性的细小颗粒状物质。排除中枢神经系统肿瘤、脱髓鞘病变及炎性假瘤等疾病。结论中枢神经系统Whipple病极为罕见,临床及影像学表现复杂多样,病理学检查仅能提示特殊感染,治疗困难,误诊率及病死率高。早期进行组织活检,结合临床表现及病理学特征可以明确诊断,经规范的抗生素治疗,患者可获得良好预后。     

Role of cytokines and chemokines in prion infections of the central nervous system

Prion infections of the central nervous system (CNS) are characterised by a reactive gliosis and the subsequent degeneration of neuronal tissue. The activation of glial cells, which precedes neuronal death, is likely to be initially caused by the deposition of misfolded, proteinase K-resistant, isoforms (termed PrP(res)) of the prion protein (PrP) in the brain. Cytokines and chemokines released by PrP(res)-activated glia cells may contribute directly or indirectly to the disease development by enhancement and generalisation of the gliosis and via cytotoxicity for neurons. However, the actual role of prion-induced glia activation and subsequent cytokine/chemokine secretion in disease development is still far from clear. In the present work, we review our present knowledge concerning the functional biology of cytokines and chemokines in prion infections of the CNS.     

中枢神经系统表面铁沉积症一例

目的 讨论中枢神经系统表面铁沉积症的病例特点及诊断.方法 对1例患者进行临床相关资料分析.结果 中枢神经系统表面铁沉积症是由于铁沉积在中枢神经系统表面而产生.临床典型特征为:进行性感音性听力下降,小脑性共济失调和锥体束征,磁共振的梯度回波序列T2*加权像上可见颅脑及脊髓表面显著低信号带,脑脊液中铁蛋白含量增高.结论 中枢神经系统表面铁沉积症容易误诊,通过病史及体格检查并结合相关检查可以在疾病的早期确诊.磁共振的梯度回波序列T2*加权像及脑脊液的相关检查有助于诊断.     

遗传性压力易感性周围神经病的中枢神经系统改变

遗传性压力易感性周围神经病(hereditary neuropathy with liability to pressure palsies,HNPP)是一种常染色体显性遗传疾病,由De Jong于1947年首先描述.Chance等[1]1993年发现HNPP与含有PMP22基因的染色体17p11.2的缺失有关.HNPP临床上表现为肢体轻微受压即可发生反复发作的单神经或多神经麻痹,可有运动和感觉障碍,压迫性麻痹常累及尺神经、桡神经和腓总神经,多数病例可自行好转,一般在数周或数月后缓慢恢复.     

微孢子虫脑炎一例临床和病理表现

目的研究微孢子虫脑炎的临床与病理形态学表现。方法对1例微孢子虫脑炎患者的临床、脑活体组织检查组织病理特点进行研究。结果此病呈亚急性或慢性起病，体温大多正常或37．5℃以下，升高见于病情加重出现昏迷时；患者不伴有人类免疫缺陷病毒感染但免疫力低下；全脑多发病灶，有脑膜刺激征；骨髓检查呈感染骨髓象；发病初期类风湿因子升高；中期以后出现间接胆红素升高现象；脑脊液蛋白、免疫球蛋白IgG、IgA升高，细胞学检查示淋巴细胞反应。患者伴有胸膜炎、腹膜炎及膀胱炎。脑MRI表现为双侧大、小脑半球脑白质区见斑片状等长T1、长T2信号影，压水扫描（FLAIR）呈高信号，无明显占位效应，强化扫描病灶呈斑点状及环形强化；神经系统损害症状呈永久性，治疗无效。脑组织病理显示神经元变性、核固缩，有血管周围炎现象，神经元内胞质内可见病原体。Wister大鼠腹腔内注射患者脑脊液2周后出现多脏器内炎症反应，腹腔灌洗液内发现大量病原体。结论患者伴有多脏器病变，脑内多发病灶，脑组织病理呈炎症反应，Kltiver-Barrer-PAS染色法可显示神经元内病原体。动物腹腔注射培养可增加病原体的检出率。     

Whipple''s disease and the central nervous system a case report and a review of the literature

A 65-year-old man was suffering from recurrent manic psychosis accompanied by weight loss. He also had a history of pleural effusion, aspecific migratory non-deforming seronegative polyarthritis, sensorineural hearing loss and semicircular canal paresis. Whipple's disease (WD) had been diagnosed at the age of 63 years. On admission to hospital)he had weight loss, diarrhoea in combination with an organic, brain syndrome, hemiparesis and ophthalmoplegia, including internuclear ophthalmoplegia (INO). A clinical diagnosis of central nervous system (CNS) WD was made. MRI revealed a thalamus lesion that halved in size during sulfamethoxazole-trimethop:rim treatment. The organic brain syndrome and ophthalmoplegia diminished also, as did the cerebrospinal fluid (CSF) IgG level. A review of CNS WD is presented and implications for treatment are discussed.     

我国人类免疫缺陷病毒和梅毒螺旋体感染中枢神经系统研究进展

获得性免疫缺陷综合征(亦称艾滋病)和梅毒在全球范围内广泛流行,严重危害国家公共卫生安全。我国对人类免疫缺陷病毒(HIV)相关中枢神经系统损害及神经梅毒的研究日益增多。本文检索目前国内学者发表的HIV和梅毒螺旋体感染中枢神经系统的相关文献,总结其流行病学特征、发病机制、临床特点、诊断与治疗策略,以为临床诊断与治疗提供新的思路。     

Recurrent acute obstructive hydrocephalus as clinical onset of cerebral Whipple's disease

Whipple's disease is a rare multisystemic infection caused by the intracellular bacteria Thropheryma whippelii. Central nervous system (CNS) involvement is not rare. The most frequent CNS manifestations are cognitive and behavioural changes, sopranuclear ophtalmoplegia, myoclonus, epilepsy, ataxia, meningitis and focal cerebral palsy.     

Isolated central nervous system Whipple's disease causing reversible frontotemporal-like dementia

We report a patient with a syndrome resembling frontotemporal dementia (FTD); however, on further diagnostic testing, the diagnosis was Whipple's disease. Because Whipple's disease is treatable, it should be considered in the workup of patients with a FTD-like behavioural and cognitive syndrome.     

Central nervous system fungal infections in tropics

Fungal infections of the central nervous system range from chronic indolent forms to acute fulminant forms causing significant morbidity and mortality. They often show atypical and variable neuroradiologic findings because of the absence of typical inflammatory response. The neuroradiologist must have high degree of suspicion in immunocompromised patients regarding the possibility of central nervous system fungal infections and keep in mind the appearances of various fungi even when immune response is intact. Next is to identify the pattern of involvement whether hematogenous or direct sinonasal and then make a well-informed speculation regarding the type of the pathogen based on the clinical features and imaging appearance.     

Neuropathology and general autopsy findings in AIDS during the last 15 years

A retrospective study of 450 consecutive AIDS autopsy cases (397 males, 53 females; mean age at death 38.4 years) in Vienna, Austria, between 1984 and 1999 compares the central nervous system (CNS) findings in three cohorts: 1984–1992 (190 cases), 1993–1995 (162 cases) and 1996–1999 (98 cases, after introduction of triple antiretroviral therapy) and the relationship of CNS findings to systemic AIDS pathology in the latter two cohorts. In these two groups, following involvement of the lung (85% and 75%, respectively), the brain continued to be the second most frequently involved organ (decrease from 80% to 60%, respectively). Extracerebral protozoal (Pneumocystis carinii, toxoplasmosis), Mycobacterium avium complex, viral [e.g., cytomegalovirus (CMV)], multiple opportunistic organ and CNS infections, and Kaposi sarcoma significantly decreased over time. There was less decrease in fungal infections, while bacterial organ and CNS infections (except for mycobacteriosis), lymphomas, HIV-associated CNS lesions (around 30%), non HIV-associated changes (vascular, metabolic, etc.) and negative CNS findings (10–11%) remained unchanged. Nonspecific CNS changes (e.g., meningeal fibrosis) increased. Extracerebral pathology in subjects with advanced HIV-related CNS lesions showed more frequent but decreasing systemic bacterial and CMV infections than those with negative or nonspecific neuropathology, while other opportunistic and multiple organ infections and lymphomas showed no differences between both groups. In a cohort of drug abusers, HIV encephalitis, progressive multifocal leukoencephalopathy, bacterial infections, hepatic encephalopathy, and negative CNS findings were more frequent than in non-users who showed increased incidence of CMV, toxoplasmosis, or other opportunistic CNS infections, and nonspecific CNS findings; the frequency of lymphomas was similar in both drug abusers and non-users. Similar to a recent autopsy study from San Diego, these data suggest that despite the beneficial effects of modern antiretroviral combination therapy, involvement of the brain in AIDS subjects continues to be a frequent autopsy finding, while the increased incidence of HIV encephalitis in our small cohort of drug users was less than observed in other recent autopsy studies. Received: 7 March 2000 / Revised, accepted: 24 March 2000     

Disappearance of a middle cerebral artery aneurysm associated with Moyamoya syndrome after revascularization in a child: case report

Introduction  Pediatric Moyamoya disease is rarely associated with intracranial aneurysms. We report a case of a 7-year-old girl with an antecedent of persistent craniopharyngeal canal, who presented with a history of choreiform movements. Materials and methods  A Moyamoya disease was found with an unruptured left middle cerebral artery aneurysm on her first angiography. Conservative treatment was chosen for the aneurysm and she underwent indirect revascularization by encephalosynangiosis using the multiple bur-hole technique for her Moyamoya disease. Abnormal movements were improved. Control angiogram at 6 months showed development of intracranial–extracranial anastomoses with complete resolution of the aneurysm. Aneuryms including the major arteries of the basal arterial circle occur as a by-product of the high velocity and blood flow secondary to the arterial stenosis. Blood flow modification after revascularization often lead to spontaneous regression and disappearance of these aneurysms. Conclusion  Therefore, a conservative treatment of these proximal aneurysms must be chosen after encephalosynangiosis.