9例原发性中枢神经系统血管炎临床、影像及病理特点研究

目的  总结原发性中枢神经系统血管炎临床、磁共振成像（magnetic resonance imaging,MRI）和病理学特点。 方法  收集2012年3月～2014年12月首都医科大学附属北京天坛医院神经病学中心收治的原发性中枢神经系统血管炎患者资料,采用描述性方法对其临床表现、MRI及病理学特点进行分析。 结果  共收集9例患者,其中男性5例（55.56%）,女性4例（44.44%）,年龄范围10～47岁,中位年龄30岁。痫性发作4例（44.44%）,行为认知异常3例（33.33%）,局灶性感觉运动异常5例（55.56%）,头晕2例（22.22%）,钝性头痛2例（22.22%）,面部疼痛1例（11.11%）,视物模糊1例（11.11%）,行走不稳1例（11.11%）。腰穿异常4例（44.44%）。MRI表现为双侧病灶6例（66.67%）,单侧病灶3例（33.33%）,其中受累部位分别为额叶9例（100%）,顶叶5例（55.56%）,颞枕叶4例（44.44%）,合并皮层下白质受累6例（66.67%）,合并脑膜/脊膜受累3例（33.33%）,合并基底节受累1例（11.11%）,合并脊髓受累1例（11.11%）;病变边界不清8例（88.89%）,边界清1例（11.11%）;皮层萎缩伴脑室扩大3例（33.33%）;病灶及脑脊膜强化6例（66.67%）;7例患者行磁共振T2*或磁敏感加权序列（susceptibility weighted  imaging,SWI）,其中病灶表现低信号4例（57.14%）。2例行脑病理学检查,表现为脑实质水肿,血管周围炎性细胞浸润,血管壁坏死和胶质增生。 结论  原发性中枢神经系统血管炎临床表现及影像学多样,病灶合并脑膜和（或）脊膜强化,以及磁共振T2*或SWI序列低信号是其重要影像学特征,脑活检仍是目前重要的确诊手段。     

内囊预警综合征的临床特征和预后

目的 分析内囊预警综合征（capsular warning syndrome，CWS）的临床特点、影像学特征及治疗预后， 以提高对此病的认识。 方法 回顾性分析战略支援部队特色医学中心神经内科2013年1月-2018年12月收住院治疗的CWS的 临床资料、影像学特征及治疗预后情况。 结果 共12例患者，男性8例（66.67%），中位年龄为60岁。高脂血症9例（75.0%），高血压8例 （66.67%），糖尿病4例（33.33%），肿瘤病史3例（25.0%）。纯运动性卒中4例（33.33%），运动伴有构 音障碍者4例（33.33%），运动及感觉性障碍3例（25.00%），纯感觉障碍1例（8.33%）。影像学示豆纹 动脉供血区新发脑梗死6例（50%）。所有患者给予口服双联抗血小板治疗，其中3例症状复发患者又 给予静脉溶栓和静脉抗血小板治疗。3个月随访，所有患者均未再出现症状发作，10例mRS评分为0 分，2例mRS评分分别为4分及3分。 结论 CWS主要表现为运动障碍，发作刻板，常见原因为动脉粥样硬化性。在本组病例中发现双重 抗血小板治疗有效，静脉溶栓效果不肯定。     

Abnormal cervical vestibular-evoked myogenic potential in anterior inferior cerebellar artery territory infarction: frequency, pattern, and a determinant

BackgroundThere have been few magnetic resonance imaging (MRI) studies of the spinal cord in large multiple sclerosis (MS) patient cohorts and little is known about correlations between cord lesions and human leukocyte antigen (HLA) alleles.ObjectiveTo investigate the spectrum of MRI changes in the spinal cord in MS and associations with the HLA-DRB1 genotype.MethodsTwo hundred and fifty two consecutive MS patients from the Perth Demyelinating Diseases Database had MRI of the spinal cord and brain and high-resolution HLA-DRB1 genotyping. The numbers, locations, shape and segmental extent of cord lesions were analysed and were correlated with carriage of individual HLA-DRB1 alleles and diplotypes.ResultsFocal cord lesions were present in 82.9% of cases, with numbers being maximal in the cervical cord and increasing with disease duration. Focal lesions were usually round or oval in shape but in 35% of cases subpial wedge-shaped lesions were present. Diffuse cord involvement was present in 10% of cases and correlated with carriage of HLA-DRB1*1501 and with higher disability. Carriage of the minor allele HLA-DRB1*0701 was significantly associated with numbers of wedge-shaped lesions and lesions in the cervical cord, while HLA-DRB1*1104 and DRB1*0103 were significantly associated respectively with higher and lower numbers of thoracic cord lesions. HLA-DRB1*1501 and the HLA-DRB1*11 sub-alleles DRB1*1101 and DRB1*1104 were significantly associated with the segmental length of cord lesions.ConclusionsOur study is the first to investigate the frequency of subpial wedge-shaped lesions in the cord in vivo and has provided preliminary evidence that HLA-DRB1 alleles may play a role in determining the severity and extent of spinal cord involvement in MS.     

内囊预警综合征的临床特征和预后

目的 分析内囊预警综合征（capsular warning syndrome，CWS）的临床特点、影像学特征及治疗预后，
以提高对此病的认识。
方法 回顾性分析战略支援部队特色医学中心神经内科2013年1月-2018年12月收住院治疗的CWS的
临床资料、影像学特征及治疗预后情况。
结果 共12例患者，男性8例（66.67%），中位年龄为60岁。高脂血症9例（75.0%），高血压8例
（66.67%），糖尿病4例（33.33%），肿瘤病史3例（25.0%）。纯运动性卒中4例（33.33%），运动伴有构
音障碍者4例（33.33%），运动及感觉性障碍3例（25.00%），纯感觉障碍1例（8.33%）。影像学示豆纹
动脉供血区新发脑梗死6例（50%）。所有患者给予口服双联抗血小板治疗，其中3例症状复发患者又
给予静脉溶栓和静脉抗血小板治疗。3个月随访，所有患者均未再出现症状发作，10例mRS评分为0
分，2例mRS评分分别为4分及3分。
结论 CWS主要表现为运动障碍，发作刻板，常见原因为动脉粥样硬化性。在本组病例中发现双重
抗血小板治疗有效，静脉溶栓效果不肯定。     

Les manifestations neurologiques de la maladie de Behçet : étude de 67 patients

IntroductionNeurological manifestations of Behçet disease (BD) are polymorphic and serious. The purpose of this study was to analyze the clinical patterns and outcome of neurological involvement in BD.MethodsThe medical records of patients with neurological manifestations of BD were reviewed retrospectively. All patients fulfilled the International Study Group Criteria for the Diagnosis of BD. Patients with headache and normal findings (neurological examination, cerebrospinal fluid, computed tomography scan, magnetic resonance imaging) were excluded.ResultsSixty-seven patients had clinical evidence of neurological involvement. There were 53 men and 14 women. A male/female sex ratio was 3.78. The average age of onset of neurological involvement was 31.5. The meningoparenchymal (MP) central nervous system involvement was found in 83.58%. The most common findings were pyramidal signs. Eleven patients (16.41%) without parenchymal central nerouvous system involvement were noted. In this group, there were six cases of intracranial thrombosis, one case of intracranial aneurysm and four cases of idiopathic intracranial hypertension. Other clinical features were reported: extrapyramidal signs and isolated spinal cord involvement. The course of disease was favorable in 70% of cases, and unfavorable in 30%.ConclusionClinical patterns of neurological involvement in BD are various and particularly serious in the MP group.     

原发性干燥综合征继发中枢神经系统血管炎临床及影像分析

目的   探讨原发性干燥综合征继发中枢神经系统血管炎的临床和影像特征。 方法   回顾性分析35例原发性干燥综合征继发中枢神经系统血管炎患者临床表现和影像学资料。 结果  男性5例,女性30例;年龄18～76［平均（52±14）］岁;中枢神经系统首发症状24例（68.57%）。其中以急性脑血管病发病患者8例（22.86%）,表现为蛛网膜下腔出血2例（5.71%）,短暂性脑缺血发作2例（5.71%）,动静脉瘘2例（5.71%）,脑出血1例（2.86%）,静脉窦血栓1例（2.86%）,其余的患者临床表现形式分别为：脑白质病变10例（28.57%）,视神经脊髓炎6例（17.14%）,脊髓炎5例（14.29%）,帕金森综合征3例（5.57%）,三叉神经痛2例（5.71%）,脑脊髓炎1例（2.86%）。影像学表现动脉瘤3例（8.57%）（2例蛛网膜下腔出血）;动脉狭窄2例（5.71%）,其中椎动脉狭窄1例（2.86%）,大脑中动脉狭窄1例（2.86%）;颞顶枕动静脉瘘2例（占5.71%,三叉神经痛合并动静脉瘘1例）;上矢状窦血栓1例（2.86%）。室旁白质受累20例（57.14%）,皮层下白质受累16例（45.71%）,脑干8例（22.86%）,脊髓11例（31.43%）;脑室扩大2例（5.71%）;其中脊髓/脑干单病灶累及多个脊髓阶段（≥3个脊髓阶段）患者10例（28.57%）。 结论  中枢神经系统原发性干燥综合征可以急性脑血管病形式起病,但以急慢性小血管受累的脑白质病和脊髓病最常见;单病灶多个脊髓阶段是脊髓病变的影像特点。     

Fatal neurologic involvement in pediatric Wegener's granulomatosis

Wegener's granulomatosis is a potentially life-threatening vasculitis with widely variable presentation. Only three pediatric cases with severe central nervous system involvement are reported in the literature. Early fatal outcome as described here is exceptional. This report describes a 13-year-old female with typical skin lesions, proteinuria, and renal failure initially misdiagnosed as a Schoenlein-Henoch purpura. A kidney biopsy revealed severe extracapillary proliferation in 70% of the analyzed glomeruli but no granuloma. In spite of methylprednisolone pulse therapy and oral high-dose prednisone, end-stage renal failure was reached 4 months later, necessitating peritoneal dialysis. Three months later she presented with pulmonary hemorrhage and positive antineutrophil cytoplasmic antibodies suggesting Wegener's granulomatosis. This episode was controlled by methylprednisolone pulses. Seven months later she presented generalized seizures and coma, suggesting central nervous system involvement confirmed by magnetic resonance imaging. Methylprednisolone pulses and intravenous immunoglobulins led to neurologic improvement. Oral methotrexate was then introduced for long-term disease control. Another severe relapse of central nervous system vasculitis did not respond to any applied therapies and led to death 16 months after initial symptoms. This case emphasizes the need for activity scores to identify patients at risk for progressive systemic vasculitis requiring early and long-term aggressive immunosuppressive therapy.     

Cytotoxic lesions of the corpus callosum in children: Etiology,clinical and radiological features,and prognosis

ObjectivesCytotoxic lesions of the corpus callosum (CLOCCs) are secondary lesions associated with entities like infection manifested by restricted diffusion on diffusion-weighted cranial magnetic resonance imaging. Our objectives are to evaluate the clinic-radiological spectrum of pediatric patients with cytotoxic lesions of the corpus callosum (CC).MethodsChildren (0–18 years) admitted between February 2017 and May 2020 with splenial lesions showing diffusion restriction on MRI, either isolated or within involvement of other parts of the brain, were included retrospectively. The primary lesions of the CC (e.g. acute disseminated encephalomyelitis, acute ischemic infarction, and glioblastoma multiforme) were excluded. CLOCCs were divided into infection-associated, metabolic disorder–associated, and trauma-associated lesions, as well as CLOCCs involving other entities. Data were collected from the medical databases.ResultsForty-one patients were determined to have CLOCCs. Twenty-five (61%) were infection-associated, nine (22%) were trauma-associated, and three (7%) were metabolic disorder–associated cases, including 2 inherited disorders of metabolism. There were four (10%) patients with other entities, three with epilepsy, and one had an apparent life-threatening event. Six patients had a known etiology among the infection-associated group; one had multisystem inflammatory syndrome caused by COVID-19 and one had been infected by COVID-19 without any complications. All the infection-associated patients with isolated splenial lesions recovered totally, although six patients required intensive care hospitalization. Four trauma-associated patients had sequela lesions.ConclusionsCLOCCs are associated with a spectrum of diseases, including the new coronavirus, COVID-19 infection. Infection-associated CLOCCs has the best prognosis, although severe cases may occur. Sequelae are possible based on the etiology.     

成人贫血并发急性脑梗死的临床特点分析

目的 探讨成人贫血并发急性脑梗死的临床特点。方法 回顾性分析2014年1月～2015年12月本院神经内科收治的18例成人贫血并发急性脑梗死患者的临床资料,总结其贫血特点、脑卒中危险因素、临床表现和脑MRI特点。结果 成人贫血并发急性脑梗死患者占同期所有住院急性脑梗死患者(205例)的比例为8.78%。其中男11例,女7例,平均年龄(60.56±12.50)岁(35～87岁),平均血红蛋白浓度(74.06±14.95)g/L(52～102 g/L); 轻度贫血3例(16.66%),中度贫血12例(66.67%),重度贫血3例(16.66%); 平均血小板计数(311.33±101.48)×10⁹/L(136～455×10⁹/L); 小细胞低色素性贫血14例(77.78%)(9例确诊为缺铁性贫血),缺铁性贫血患者平均血小板计数(375±73.78)×10⁹/L(251～455×10⁹/L)。无脑卒中危险因素者6例(33.33%),脑卒中危险因素1～2项者7例(38.89%),3项以上危险因素者5例(27.78%); 5例有意识障碍(27.78%); 12例病灶累及前循环(66.67%),14例累及分水岭区(77.78%),10例累及放射冠及半卵圆中心(55.56%); 13例为多发性脑梗死(72.22%),15例为小梗死(83.33%)。18例患者均行扩容治疗,4例输注红细胞; 临床治愈及好转者12例(66.67%)。结论 成人贫血并发急性脑梗死患者多为中度贫血,缺铁性贫血占多数,多伴有继发性血小板增多; 患者多缺乏脑血管病危险因素; 病变多位于脑前循环分布区,分水岭区多见,病灶以多发性小梗死灶为主,且多累及放射冠及半卵圆中心。治疗应以扩容及纠正贫血为主。     

隐源性缺血性脑卒中与房间隔膨胀瘤的相关性研究

目的 探讨隐源性缺血性脑卒中(Cryptogenic ischemic stroke,CS)与房间隔膨胀瘤(Atrial septal aneurysm,ASA)的相关性。方法 选取2015年1月-2020年1月本院神经内科收治的≤55周岁CS患者137例(观察组)及同时期的体检健康人群137例(对照组),比较2组年龄、性别、体质量指数、饮酒史、吸烟史、脑卒中家族史、左室射血分数、右房横径、右房上下径、左室舒张末期内径、高同型半胱氨酸血症、卵圆孔未闭、睡眠呼吸暂停综合征、颈动脉夹层、阵发性房颤、血小板减少性紫癜、偏头痛、动脉炎、ASA分布情况及ASA膨出度、基底直径,采用Logistic回归方程分析CS的相关影响因素,采用Pearson分析ASA膨出度、基底直径与美国国立卫生研究院卒中量表(National institute of health stroke scale,NIHSS)评分的相关性,分析合并ASA的CS患者年龄、性别、神经功能缺损程度分布特点。结果 2组高同型半胱氨酸血症、卵圆孔未闭、睡眠呼吸暂停综合征、颈动脉夹层、阵发性房颤、血小板减少性紫癜、偏头痛、动脉炎、ASA分布比较均有明显差异(P<0.05); 高同型半胱氨酸血症、卵圆孔未闭、睡眠呼吸暂停综合征、颈动脉夹层、阵发性房颤、血小板减少性紫癜、偏头痛、动脉炎、ASA均是CS的相关危险因素(P<0.05); 观察组ASA膨出度、基底直径大于对照组(P<0.05); ASA膨出度、基底直径与NIHSS评分呈正相关(P<0.05); 合并ASA的CS患者年龄41～50岁占比最高,为44.44%,其次是31～40岁(25.93%)、51～55岁(18.52%)、20～30岁(11.11%); 男性占比55.56%,高于女性的44.44%; 神经功能缺损程度以中度(59.26%)、重度(33.33%)为主。结论 CS的发生与高同型半胱氨酸血症、卵圆孔未闭、睡眠呼吸暂停综合征、颈动脉夹层、阵发性房颤、血小板减少性紫癜、偏头痛、动脉炎、ASA有关,且ASA膨出度、基底直径可影响CS患者的神经功能缺损程度,CS伴ASA高发于41～50岁男性人群,以中重度病情为主。     

An enhancing pituitary lesion in a young woman: a diagnostic dilemma.

Wegener's granulomatosis is a systemic small-vessel vasculitis predominantly involving the upper airways, lungs and kidneys. Central nervous system involvement is known to occur in 10% of cases, manifesting as diffuse meningeal involvement and cerebral granulomatous lesions, but isolated involvement of the pituitary is extremely rare. We describe a case of isolated pituitary involvement with abscess formation secondary to Wegener's granulomatosis in a 21-year-old woman. To the best of our knowledge this is the first reported case of pituitary abscess formation secondary to Wegener's granulomatosis.     

卒中类型、卒中部位与卒中后癫痫的多因素关系

目的探讨卒中类型、卒中部位与卒中后癫痫的多因素关系,为卒中后癫痫的防治提供参考。方法以1804例卒中患者为研究对象,收集其性别、年龄、卒中类型、卒中部位、卒中后癫痫发生的时间等资料,根据卒中后是否发生癫痫,将患者分为卒中后无癫痫组(n=1487)和卒中后癫痫组(n=317),分析卒中后癫痫发作的危险因素。结果共317例卒中后癫痫发作患者,其中早发性癫痫141例(44.48%),迟发性癫痫176例(55.52%)。不同卒中部位及卒中类型的癫痫发病率为17.57%。多因素logistic回归分析显示,卒中部位中的顶叶合并蛛网膜下腔、额叶合并颞叶、额叶合并颞叶和枕叶、单一颞叶是卒中后发生癫痫的危险因素(P<0.01),其中单一颞叶是卒中后早发性癫痫的危险因素(P<0.01)。脑梗死患者常见早发性癫痫(23.66%),脑出血患者常见迟发性癫痫(47.95%)。结论卒中类型中的脑梗死、脑出血、蛛网膜下腔出血与卒中后癫痫有关;卒中部位中顶叶合并蛛网膜下腔、额叶合并颞叶、额叶合并颞叶和枕叶、单一颞叶与卒中后癫痫有关。     

Central Nervous System Involvement in Hemophagocytic Lymphohistiocytosis: A Single-Center Experience

BackgroundHemophagocytic lymphohistiocytosis is a rare multisystem disorder characterized by proliferation and diffuse infiltration multiple organs with histiocytes, including the central nervous system.PATIENTS and MethodsThirty children diagnosed with hemophagocytic lymphohistiocytosis between 1997 and 2010 were reviewed and analyzed. Central nervous system disease involvement was defined as the presence of neurological symptoms and signs or elevated values of cerebrospinal fluid cells and/or proteins.ResultsAmong the 30 patients, 17 (56%) had central nervous system involvement. Fourteen patients (46%) presented with neurological symptoms including seizures, irritability, bulging fontanelle, cranial nerve palsy, or disturbance of consciousness, whereas the remaining three patients developed central nervous system symptoms during the course of the disease. Seventeen patients (56%) had cerebrospinal fluid abnormalities. Neuroradiological studies were performed in nine patients. The most common findings were edema, atrophy, subcortical necrosis, and high signal intensity on T2-weighted magnetic resonance imaging. All patients were treated according to the Hemophagocytic Lymphohistiocytosis-94 and Hemophagocytic Lymphohistiocytosis-2004 protocols. Patients with central nervous system involvement had greater mortality. In prediction of the outcome, the cutoff value for cerebrospinal fluid protein was 470 mg/L. The most common neurological sequela was psychomotor delay.ConclusionCentral nervous system involvement in hemophagocytic lymphohistiocytosis is common and is associated with poor outcome.     

The clinical value of head-neck joint high-resolution vessel wall imaging in ischemic stroke

Background and PurposeTo study the feasibility and clinical utility of head-neck joint high-resolution vessel wall imaging (HNJ-VWI) in the assessment of ischemic stroke.MethodsWe reviewed our institutional HNJ-VWI database. Patients with transient ischemic attack (TIA) or ischemic stroke were included. Abnormal findings of intracranial and/or extracranial artery were assessed on three-dimensional time-of-flight magnetic resonance angiography (3D TOF MRA) and HNJ-VWI modified from high-resolution 3D T1 sequence and classified into three groups including intracranial, extracranial and coexisting based on the locations. Etiologies of stroke were recorded according to Trial of Org 10172 in Acute Stroke Treatment criteria.ResultsOne hundred and ten consecutive patients were studied. 3D TOF MRA displayed 71.8% (79/110, based on patients) abnormal arteries (stenosis or occlusion) , while HNJ-VWI displayed 96.3% (106/110) abnormal arteries (plaque,wall thickness and occlusion) including four isolated extracranial lesions and ten coexisting lesions. The etiologies of TIA/ischemic stroke included large artery atherosclerosis (80 cases), cerebral small vessel disease (6 cases), cardiogenic (2 cases), dissection (6 cases), vasculitis (4 cases), moyamoya disease (6 cases), others (2 cases) and undetermined (4 cases). For patients with atherosclerosis stroke, re-infarctions were more common in coexisting group than intracranial group (extracranial vs. intracranial vs coexisting: 0% vs. 9.1% vs. 43.7%, p = 0.001).ConclusionsHNJ-VWI is a feasible and valuable technique in assessment of ischemic stroke by detecting extracranial and intracranial artery abnormalities with one-step scan.     

Combined nerve and muscle biopsy in the diagnosis of vasculitic neuropathy. A 16-year retrospective study of 202 cases

We reviewed 202 biopsies performed on patients with suspected vasculitic neuropathy, of which 24 Churg-Strauss cases are studied separately. Specimens from the superficial peroneal nerve and peroneus brevis muscle were taken simultaneously by one incision. Without taking into account constitutional signs, systemic involvement was present in 131 patients, whereas the remaining 47 corresponded to non-systemic patients with lesions limited to peripheral nervous system and adjoining muscles. Diagnosis of panarteritis nodosa or microscopic polyangiitis, according to the size of involved vessels, was attested by an infiltration of vessel walls by inflammatory cells associated with fibrinoid necrosis or sclerosis. Microvasculitis was diagnosed when inflammatory infiltration concerned small vessels with few or no smooth-muscle fibers and without any necrosis. Microvasculitis was present in 11 of 46 non-systemic cases, and this predominance is statistically significant. Isolated perivascular cell infiltrates in the epineurium were considered not significant but allowed the diagnosis of 'probable vasculitis' if associated with at least one of the following features: regenerating small vessels, endoneurial purpura, asymmetric nerve fiber loss, and/or asymmetric acute axonal degeneration. Necrotizing vasculitis was visible in 60 cases: in nerve (16 cases), in muscle (19 cases), and both (25 cases). Microvasculitis was present in 25 cases: in nerve (19 cases), muscle (four cases), or both (two cases). Moreover, granulomatous vasculitis was found in the nerve of one non-systemic patient presenting also sarcoid granulomas in muscle. There were 24 'probable vasculitis' and 68 negative cases. Muscle biopsy improved the yield of definite vasculitis by 27%.     

Spontaneously changing MRI findings of primary central nervous system vasculitis: A case report

Spontaneously disappearing lesions on magnetic resonance imaging (MRI) has been described in primary central nervous system lymphoma. In this case, we report our experience of spontaneously changing MRI findings of primary central nervous system vasculitis (PCNSV) confirmed histopathologically. A 69-year-old man presented with sudden unsteady gait. Fluid-attenuated inversion recovery (FLAIR) images showed high-intensity areas in the right deep white matter. Contrast-enhanced T1-weighted MRI demonstrated a nodular lesion in the white matter of the left occipitoparietal lobe. On repeat MRI 7 days later, FLAIR hyperintense lesions had spontaneously disappeared and contrast-enhanced lesions had progressed, with new contrast lesions in the right corpus callosum. Repeat MRI 14 days after admission demonstrated contrast-enhancing lesions either increased or decreased in intensity in both occipitoparietal lobes. Contrast-enhancing lesions were therefore biopsied. Histopathological examination revealed vasculitis with fibrinoid necrosis. PCNSV was diagnosed without any signs of inflammation in blood vessels other than cerebral blood vessels. Spontaneously changing MRI findings may play an important role in diagnosing PCNSV.     

Neuroborreliosis-associated cerebral vasculitis: long-term outcome and health-related quality of life

Neuroborreliosis affects the nervous system after systemic infection with the spirochete Borrelia burgdorferi. Previously, cerebral vasculitis has been regarded as an extremely rare complication of neuroborreliosis. The data on the long-term outcome in patients with cerebral vasculitis due to neuroborreliosis are limited. The objective of this study was to perform a longitudinal analysis of cases of neuroborreliosis-associated cerebral vasculitis. We recruited all patients (n = 11) diagnosed with neuroborreliosis-associated in three neurological departments in an East German region. Inclusion criteria were sudden neurological deficits, magnetic resonance (MR) imaging findings that conform to cerebral ischemia or brain infarction, intrathecal synthesis of borrelia-specific antibodies, and non-atherosclerotic pathology of brain supplying arteries. Vasculitic changes were detected by digital subtraction angiography, MR angiography and/or transcranial Doppler ultrasound. Outcomes were measured by the modified Rankin scale (mRS) and EuroQoL Index. Cerebral vasculitis is a rare complication of Lyme disease (0.3 % of all cases in the endemic area). Ten out of 11 patients diagnosed with neuroborreliosis-associated vasculitis cerebral vasculitis using clinical, radiological and immunological criteria developed ischemic stroke or transient ischemic attacks (TIA), 7 patients had recurrent stroke. Vasculitic alterations could be demonstrated in 8 patients that all except one developed ischemic lesions. The median mRS was 3 (range 0–4) at admission and 2 (range 0–6) at discharge. The posterior circulation was affected in 8 of 11 patients; thrombosis of the basilar artery was detected in 2 patients, one died in the acute stage. Neuroborreliosis can cause recurrent stroke or TIA on the basis of cerebral vasculitis. Lumbar puncture is needed for detection of this potentially life-threatening condition. Early recognition and adequate therapy would possibly improve outcome.     

Chronic inflammatory demyelinating polyradiculoneuropathy associated with central nervous system involvement--as compared to multiple sclerosis

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with central nervous system (CNS) demyelinating lesions has recently been reported to mimic multiple sclerosis (MS). In this paper, a series of patients with CIDP were examined to see if they had CNS involvement. CIDP patients with CNS lesions were then compared to patients with MS with peripheral nervous system (PNS) involvement for similarities. CNS and PNS involvement were detected by clinical symptoms, neurological findings, neuro-otological and neuro-ophthalmological tests, electrophysiological examinations such as electroencephalography, evoked potentials, blink reflex, conventional peripheral nerve conduction studies and electromyography, as well as computed tomography and magnetic resonance imaging (MRI). There were 7 of 17 CIDP patients with CNS involvement, but only 2 of 59 MS patients with PNS lesions were found. The rate of CIDP with CNS involvement (41.2%) was higher than that of MS with PNS lesions (3.4%). The CNS signs and symptoms of 7 CIDP patients were not so constant as their PNS symptoms, and consisted of 1 case with optic neuritis, 4 cases with cerebellar atxia and/or nystagmus, and 3 cases with spinal symptoms. These signs and symptoms are all well known in MS. Prolonged latencies on evoked potentials and high signal white matter lesions on T2 weighted MRI, indicating demyelinating CNS lesions were also similar to those found in MS. The CNS involvement in those patients with CIDP was therefore similar in character to those found in MS, but was far less severe than the PNS finding.(ABSTRACT TRUNCATED AT 250 WORDS)     

Magnetic resonance imaging abnormalities in cat-scratch disease encephalopathy.

A 23-year-old woman who presented with a branch retinal artery occlusion followed by encephalopathy showed, by brain magnetic resonance imaging, a nonenhancing lesion in the right parietal gray matter with normal diffusion-weighted imaging. Of 64 reported cases of cat-scratch encephalopathy with documented neuroimaging findings, only 12 (18.8%) have had abnormal imaging findings. The abnormalities have included cerebral white matter lesions, basal ganglia and thalamic lesions, and multifocal lesions in immunocompromised patients, but no gray matter lesions similar to those in this patient. The variety of neuroimaging findings supports multiple pathophysiologic mechanisms of central nervous system involvement in this disorder.     

¿Es esperable que haya cuadros neurológicos por la pandemia por SARS-CoV-2?

IntroductionThere is growing evidence that SARS-CoV-2 can gain access to the central nervous system (CNS). We revise the literature on coronavirus infection of the CNS associated with neurological diseases.DevelopmentNeurological symptoms were rarely reported in the SARS-CoV and MERS-CoV epidemics, although isolated cases were described. There are also reports of cases of neurological symptoms associated with CoV-OC43 and CoV-229E infection. The presence of neurological lesions, especially demyelinating lesions in the mouse hepatitis virus model, may explain the mechanisms by which coronaviruses enter the CNS, particularly those related with the immune response. This may explain the presence of coronavirus in patients with multiple sclerosis. We review the specific characteristics of SARS-CoV-2 and address the question of whether the high number of cases may be associated with greater CNS involvement.ConclusionAlthough neurological symptoms are not frequent in coronavirus epidemics, the high number of patients with SARS-CoV-2 infection may explain the presence of the virus in the CNS and increase the likelihood of early- or delayed-onset neurological symptoms. Follow-up of patients affected by the SARS-CoV-2 epidemic should include careful assessment of the CNS.