Bickerstaff脑干脑炎和Miller Fisher综合征临床及预后的对比研究

目的探讨Bickerstaff脑干脑炎(BBE)和Miller Fisher综合征(MFS)的临床特征,研究两者的症状学特点及疾病分类学关系。方法对湘雅二医院2003-01-2013-12符合BBE(21例)和MFS(67例)诊断标准的患者的临床资料进行回顾性分析。结果 BBE和MFS患者均存在以上呼吸道感染为主的前驱感染症状〔10例(48%)、36例(54%)〕,并具有眼外肌麻痹和共济失调的共同症状〔21例(100%)、67例(100%)〕,头痛〔8例(42%)、20例(30%)〕、眼内肌麻痹〔8例(38%)、17例(25%)〕、延髓麻痹〔8例(50%)、32例(48%)〕、面瘫〔6例(35%)、20例(30%)〕、浅表感觉障碍〔4例(25%)、28例(42%)〕等症状在两者中均常见且发生率相近;两者均有脑脊液蛋白-细胞分离现象〔5例(25%)、23例(38%)〕和头部影像学检查〔10例(59%)、2例(4%)〕异常。两者主要的不同点在于BBE患者的中枢神经系统受累表现如意识障碍〔16例(76%)〕、腱反射亢进〔11例(52%)〕、Babinski征〔6例(32%)〕、头部MRI脑干部位异常信号〔10例(59%)〕、脑电图异常〔7例(78%)〕较MFS患者〔0例、0例、8例(12%)、2例(4%)、0例〕常见。两组患者预后均较好,随访期间(≥1年)均未见复发病例,MFS患者59例(88%)症状完全消失,BBE患者10例(48%)症状完全消失,3例(14%)明显改善,6例BBE死亡。结论BBE与MFS临床特点相似且存在交叉重叠,两者可能形成同一连续性自身免疫性疾病谱。     

Bickerstaff's脑干脑炎继发肥大性下橄榄核变性的临床及影像学特点(附1例报告)

目的 探讨Bickerstaff's脑干脑炎(BBE)继发肥大性下橄榄核变性(HOD)的临床及影像学特点.方法 回顾性分析1例BBE继发HOD患者的临床资料.结果 本例患者先以复视、构音障碍、口角歪斜等多脑神经受损症状及共济失调为主要表现,后出现饮水呛咳,伸舌受限,软腭、舌肌、面肌震颤及肌张力增高等延髓及锥体外系症状;头颅MRI示入院后第2 d时脑干及小脑异常信号并强化,入院后第17 d时出现延髓双侧下橄榄核区局限性肥大,且双侧下橄榄核区异常信号较前明显增大.结论 BBE继发HOD的临床表现以多脑神经受损症状、共济失调及锥体外系症状为主,影像学改变为脑干、小脑及下橄榄核区异常信号和下橄榄核区局限性肥大.     

Bickerstaff脑干脑炎和Miller Fisher综合征免疫调节治疗疗效的对比研究

目的探讨免疫调节治疗在Bickerstaff脑干脑炎(BBE)和Miller Fisher综合征(MFS)中的疗效。方法回顾性分析湘雅二医院和湘雅医院2003~2013年符合BBE(32例)和MFS(67例)诊断标准的患者临床资料,比较免疫调节治疗对两组患者症状的改善及预后的影响。结果与对照组对比,IVIg联合激素治疗可以加快BBE患者意识障碍的恢复(P<0.05);IVIg、激素单独治疗在BBE患者意识障碍、眼外肌麻痹及共济失调症状的开始改善时间上无明显差异(P>0.05)。各免疫调节治疗措施与对照组相比,对MFS患者眼外肌麻痹和共济失调症状的恢复均无明显加速作用(P>0.05)。两组平均随访时间均>1 y,未见复发病例,绝大多数的BBE(66%)和MFS(98%)患者症状完全缓解,9例BBE患者死亡。结论 IVIg联合激素治疗可加快BBE患者意识障碍的恢复,改善早期出现意识障碍患者的预后。免疫治疗措施对MFS患者疾病病程及预后无明显影响,可能与该病的具有较好的自然病程有关。     

Bickerstaff脑干脑炎

Bickerstaff脑干脑炎(BBE)是一种罕见的炎性的脱髓鞘疾病,临床表现通常为意识障碍、眼外肌麻痹、对称性迟缓性四肢瘫、共济失调、双侧面瘫、Babinski征、瞳孔异常和球麻痹等,但是Bickerstaff脑干脑炎一般预后比较好.     

Miller-Fisher综合征的临床特点分析

目的探讨Miller-Fisher综合征的临床特点。方法回顾分析12例Miller-Fisher综合征患者的临床特点。结果主要神经系统症状:头昏,视物成双,四肢无力,手足发麻感,头痛。主要神经系统体征:瞳孔直接及间接对光放射消失,双侧眼外肌麻痹,水平眼震,周围性面瘫,共济失调,深、浅感觉减退,腱反射减弱或消失。首发症状主要表现为视物成双和四肢无力,脑脊液蛋白升高10例,脑脊液细胞升高2例。肌电图检查示神经源性损害3例,CT和MRI检查均未发现异常。甲基强的松龙和人血免疫球蛋白同时使用可有效缓解病情,10例患者出院后随访3个月内症状消失。结论Miller-Fisher综合征主要累及周围神经系统,大多数患者预后良好。     

儿童Bickerstaff脑干脑炎重叠Guillain-Barré综合征1例

正Bickerstaff脑干脑炎(BBE)是一种罕见的以眼外肌麻痹、共济失调、意识障碍为特征的自身免疫性脑炎,1957年由Bickerstaff首次报道并命名为"脑干脑炎"[1]。因其可能和Guillain-Barré综合征(GBS)、Miller-Fisher综合征(MFS)的病理生理过程相互关联,临床表现类似,临床上称之为GBS谱系疾病[2-3]。目前国际上针对BBE重叠GBS的大宗流行病学数据较少,     

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。     

Bickerstaff's脑干脑炎4例并文献复习

目的分析Bickerstaff脑干脑炎(BBE)的临床特点,以提高临床医生对其认识,提高诊治水平。方法对瑞金北院神经内科收治的4例BBE患者的临床资料进行回顾性分析,并综合相关文献,总结其临床特点、发病机制、治疗方法及预后。结果 4例患者均为急性起病,发病前有上呼吸道感染者2例,疱疹病毒感染1例,鼻窦炎1例。临床主要表现为急性眼肌麻痹(眼内肌麻痹3例,眼外肌麻痹1例),共济失调4例,意识障碍1例,腱反射亢进4例,病理征阳性2例,1例患者同时累及脑神经(面神经、前庭神经及听神经)。4例患者脑脊液检查及头颅MRI检查结果基本正常。2例行血清抗GQ1b抗体检查者均为阴性。4例患者使用糖皮质激素或联合免疫球蛋白治疗预后较好。结论 BBE的确诊主要依据临床表现、血清抗体、影像学及神经电生理作为辅助诊断,激素或激素联合免疫球蛋白治疗有效。     

Miller-Fisher综合征的临床特点(附3例报告)

目的 探讨Miller-Fisher综合征的临床特点.方法 回顾性分析3例Miller-Fisher综合征患者的临床资料.结果 本组男1例,女2例,年龄43～56岁;均急性起病,且存在前期呼吸道症状.临床表现均有复视及行走不稳,眼睑下垂2例,手足麻木1例.本组均有双侧眼肌麻痹、腱反射减弱(或消失)及共济失调征,有感觉减退2例、自主神经功能障碍1例.脑脊液检查示蛋白-细胞分离2例.神经电生理检查均有H反射异常.经大剂量丙种球蛋白静脉滴注治疗,3例患者均预后良好.结论 MFS患者以眼肌麻痹、腱反射减低及共济失调为主要表现,还可伴有感觉和自主神经的症状;大剂量免疫球蛋白治疗的效果较好.     

A型肉毒中毒1例报告

1　资料患者男性 ,36岁、农民 ,以“视物成双 4天 ,饮水呛咳 ,四肢无力 2天”之主诉入院。4天前晨起后感视物成双 ,伴头昏 ,全身疲乏无力 ,无头痛、呕吐、意识障碍及二便障碍 ,无发热。病后 2 4小时头颅CT未见异常 ,按“上感”对症治疗 (具体用药不详 )后症状消失。 2天前家人发现其饮水呛咳、吞咽困难 ,四肢无力 ,尚能持物行走 ,上述症状渐加重 ,入院时不能持物行走 ,伴张口困难 ,以“吉兰 巴雷综合征”收住院。病前 1天曾服臭豆腐 ,量约 4 0～ 5 0g,近期家族中先后 10人有程度不等类似症状 ,病前均食数量不等臭豆腐 ,其中 3人已死亡 ,余…     

Miller Fisher syndrome with transient coma: comparison with Bickerstaff brainstem encephalitis

We herein report a 4-year-old boy with Miller Fisher syndrome (MFS) who presented with transient coma in addition to the typical triad of internal and external ophthalmoplegia, cerebellar ataxia and areflexia after an influenza type B infection. The electroencephalogram findings revealed intermittently generalized slow wave bursts. The cerebrospinal fluid revealed high protein and a lack of any cellular response. The serum anti-GQ1b IgG antibody was elevated in the acute phase and disappeared in the convalescent phase. The transient coma with the triad of MFS in this patient indicated an extended brainstem lesion including a reticular formation, which is also the responsible lesion of Bickerstaff brainstem encephalitis (BBE), but the magnetic resonance imaging repeatedly showed no abnormal finding. Our patient suggested the involvement of central nervous system in addition to the peripheral nerve injury in MFS. He also suggested that MFS and BBE may belong to the same group of disorders as syndrome of ophthalmoplegia, ataxia and areflexia (SOAA).     

Involvement of the central nervous system in Miller Fisher syndrome: a case report

Miller Fisher syndrome (MFS) is characterised by ophthalmoplegia, ataxia and areflexia. Reports on cerebellar ataxia and supranuclear oculomotor derangement in MFS suggested an additional involvement of the central nervous system (CNS), resembling Bickerstaff's brainstem encephalitis (BBE). In the present report, a patient with a monophasic acute illness, early recovery and specific clinical-laboratory findings suggested both intrinsic brainstem and peripheral nerve disease (MFS and BBE). In pons and medulla oblangata, blurred to discrete T2-lesions were revealed by cranial MRI, while involvement of peripheral nerves was detected with EMG. The CSF showed no increase in protein or cell content, such as occurs in brainstem encephalitis.     

Bickerstaff's brainstem encephalitis -- an analysis of clinical and MRI findings

BACKGROUND AND PURPOSE: Bickerstaff's brainstem encephalitis (BBE) is a very uncommon central nervous system disease. Its etiology remains unknown but it is postulated that BBE has an autoimmunologic origin. BBE is characterized by acute ophtalmoplegia, ataxia, pyramidal paresis and disturbance of consciousness. A special pattern of changes in magnetic resonance imaging (MRI) is characteristic of BBE. Lesions are located mainly in the brainstem. The aim of this paper is to analyze the clinical course and MRI changes in our own cases of BBE. MATERIAL AND METHODS: We have analyzed medical records, clinical findings and MRI images of the patients with BBE diagnosis established in our Department of Neurology in the years 1998-2004. RESULTS: During that period 4 cases of BBE were diagnosed with clinical sings of acute brainstem lesion and MRI imaging suggestive of BBE. In our patients we have observed ophtalmoplegia, ataxia, disturbances of consciousness and pyramidal signs. Two patients had a monophasic and 2 remitting-relapsing course of the disease. In all four cases we have seen on T2-weighted images large, irregular hyperintense lesions in the brainstem, especially in the pons, midbrain and medulla, but also in one case in the thalamus and vermis of the cerebellum. Patients were treated with steroids, plasmapheresis or acyclovir or a combination of these methods. We observed a complete recovery in two cases and significant improvement in the other two lasting more than two years confirming a relatively good prognosis in BBE. CONCLUSIONS: BBE despite severe initial presentation usually has a good prognosis. MRI plays critical role in the diagnosis of BBE.     

A case of Bickerstaff's brainstem encephalitis; the evidence of cerebellum involvement by SPM analysis using PET

Although the clinical manifestations such as drowsiness, brisk reflexes, extensor plantar responses and hemisensory disturbance usually are considered to suggest Bickerstaff's brainstem encephalitis (BBE) rather than Miller Fisher syndrome (MFS), the nosological relationship between BBE and MFS has yet to be established. Herein, we report upon a 58-year-old man who showed ophthalmoplegia, ataxia and consciousness disturbance. In the absence of any abnormality on brain MRI, electrophysiological studies and SPM analysis using (18)F-FDG PET showed evidence of brainstem and cerebellum involvements.     

High-dose intravenous immunoglobulin therapy in a child case of Bickerstaff's brainstem encephalitis

We report an 11-year-old boy with Bickerstaff's brainstem encephalitis (BBE). He had gait disturbance, disturbed consciousness, and diplopia after upper respiratory tract infection. On admission, he showed multiple cranial nerve palsy, muscle weakness of arms, cerebeller ataxia and generalized areflexia. The cerebrospinal fluid on day 7 revealed albuminocytologic dissociation. IgG antibodies against GQ1b and GT1a were detected in the serum. Immunoglobulin was administered intravenously from day 11, and then his symptoms gradually diminished. When he was discharged on day 27, he had neither conscious disturbance nor limb weakness. There still were mild ophthalmoparesis and diminished deep tendon reflexes, but they disappeared by 10 months after the onset. Effective therapy for BBE has yet to be established. Our case had features of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome, such as an acute monophasic course, limb weakness with areflexia, albuminocytologic dissociation in the cerebrospinal fluid, detection of serum anti-ganglioside antibodies and efficacy of intravenous immunoglobulin, indicating that BBE and GBS are closely related. Our case suggested that intravenous immunoglobulin therapy, an established treatment for GBS, should be considered in some patients with BBE.     

Anti-GQ1b IgG antibody syndrome: clinical and immunological range

OBJECTIVES: To clarify the nosological relation among Miller Fisher syndrome (MFS), Guillain-Barré syndrome (GBS) with ophthalmoplegia, Bickerstaff's brain stem encephalitis (BBE), and acute ophthalmoparesis without ataxia. Serum samples from patients with each condition often have anti-GQ1b IgG antibody. METHODS: Information on antecedent illness, initial symptoms, neurological signs during the illness, and CSF findings were reviewed in 194 patients with anti-GQ1b IgG. It was determined whether overlapping MFS and GBS (MFS/GBS), as well as overlapping BBE and GBS (BBE/GBS), is explained by the combined action of anti-GQ1b IgG and anti-GM1 or anti-GD1a IgG, serological markers of GBS. RESULTS: Based on the diagnostic criteria, all the patients with acute ophthalmoparesis, MFS, MFS/GBS, BBE/GBS, and BBE had external ophthalmoplegia; all the patients with MFS, MFS/GBS, or GBS had hyporeflexia or areflexia; and all those with MFS and BBE showed ataxia. Tendon reflexes were decreased or absent in 91% of those with BBE/GBS, 67% of those with BBE, and 53% of those with acute ophthalmoparesis. Ataxia was present in 68% of the patients with MFS/GBS and 45% of those with BBE/GBS. Antecedent illness caused by upper respiratory tract infection had occurred in 60% to 80% of these patients, and CSF albuminocytological dissociation in 25% to 75%. Anti-GM1 or anti-GD1a IgG was present in 50% of those with GBS, 35% of those with MFS/GBS, 27% of those with BBE/GBS, 16% of those with MFS, and 8% of those with BBE. CONCLUSIONS: These findings together with the common autoantibody (anti-GQ1b IgG) suggest that a common autoimmune mechanism functions in the pathogenesis of these illnesses. In a larger study, it was confirmed clinically that MFS, GBS, BBE, and acute ophthalmoparesis are closely related, forming a continuous range. This is supported by the immunological findings. The term "anti-GQ1b IgG antibody syndrome" is not intended to be used as a clinical diagnosis, but recognition of this syndrome is useful for understanding the aetiological relation among the various illnesses and for introducing the established treatments of GBS for use with other conditions.     

Bickerstaff’s brainstem encephalitis with overlapping Guillain-Barré syndrome: Usefulness of sequential nerve conduction studies

Bickerstaff's brainstem encephalitis (BBE) is a rare immune-mediated disorder characterized by ophthalmoplegia, ataxia and disturbance of consciousness, which may overlap with Guillain-Barré syndrome (GBS) if there is additional limb weakness. We report a 7-month-old boy presented with ophthalmoplegia followed by a rapidly ascending paralysis of all four limbs and disturbance of consciousness. The initial impression was BBE with overlapping GBS. This was supported by sequential nerve conduction study (NCS) findings compatible with an acute inflammatory demyelinating polyneuropathy (AIDP). He received intravenous pulse methylprednisolone, intravenous immunoglobulin and plasmapharesis with complete clinical recovery after 6?weeks of illness and improved NCS findings from week 16. This is the first case of paediatric BBE with overlapping GBS with an AIDP subtype of GBS. It expands the clinical spectrum of this condition in children. Our case highlights the importance of sequential NCS in paediatric BBE with overlapping GBS for accurate electrophysiological diagnosis and prognosis particularly if the first NCS findings are not informative.     

Delayed motor and sensory neuropathy in a patient with brainstem encephalitis

Bickerstaff's brainstem encephalitis (BBE), Miller Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS) are thought to be closely related and to form a continuous spectrum. However, chronic polyneuropathy in BBE has not been reported. We report the temporal profile of anti-ganglioside antibody titer in a case of BBE-like brainstem encephalitis complicated with chronic polyneuropathy. A 71-year-old Japanese woman presented with drowsiness and cerebellar ataxia in addition to mild weakness in distal limb muscles. Anti-GalNAc-GD1a IgG and anti-GalNAc-GM1b IgG antibodies were positive in her serum. Brain magnetic resonance imaging revealed high-intensity signals in the midbrain, pons, and middle cerebellar peduncles on T2-weighted images. Central nervous system manifestations improved after immunomodulating therapy that included prednisolone, plasmapheresis and intravenous immunoglobulin. Nevertheless, the distal muscle weakness was exacerbated when the anti-GalNAc-GD1a IgG titer was elevated. Nerve conduction study indicated motor and sensory neuropathy which was developed motor dominant axonal damage. These findings suggest that anti-ganglioside antibodies, including anti-GalNAc-GD1a IgG, may be involved in a common autoimmune mechanism in BBE-like brainstem encephalitis and chronic motor dominant axonal neuropathy. However, the fact that the latter manifestation exacerbated after the improvement of former one possibly indicates different thresholds of neurologic symptoms mediated by anti-ganglioside antibodies in the present patient.     

Bickerstaff's brainstem encephalitis and Fisher syndrome: their relationship and treatment]

The nosological position of Bickerstaff's brainstem encephalitis (BBE) was not eatablished, and its etiology was not clear until 1993. Because anti-GQ1b IgG antibody frequently occurs in patients with Fisher syndrome (FS) and there are clinical similarities between FS and BBE, we investigated anti-ganglioside antibodies in sera from three patients with BBE. High anti-GQ1b IgG antibody titers were present in their sera, but decreased with the clinical course of the illness. The findings that BBE and FS shared common autoantibody suggest that autoimmune mechanism common to FS is likely in BBE, and that both conditions form a continuous spectrum. To clarify the clinical features of BBE, we reviewed detailed clinical profiles and laboratory findings for 62 cases of BBE diagnosed by the strict criteria of progressive, relatively symmetric external ophthalmoplegia and ataxia by 4 weeks, and disturbance of consciousness or hyperreflexia. A striking feature was the association with flaccid symmetric tetraparesis seen in 60%. Limb weakness in the BBE cases studied was considered the result of overlap by the axonal subtype of Guillain-Barré syndrome. These indicates that the two disorders are closely related and form a continuous spectrum, and that treatment for Guillain-Barré syndrome should be applied for BBE.