Bickerstaff's脑干脑炎8例并文献复习
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摘要
目的:分析Bickerstaff脑干脑炎(BBE)的临床特点,以提高临床诊疗水平。方法:对8例BBE患者的临床资料进行回顾性分析,并结合相关文献,总结其临床特点、发病机制、治疗方法及预后。结果:8例患者均为急性起病,发病前有上呼吸道感染者2例,腹泻2例,水痘-带状疱疹病毒感染1例。首发症状为肢体麻木无力伴言语含混3例;意识不清、双下肢无力、声音嘶哑伴吞咽困难、面部和手指麻木、头晕伴行走不稳的各1例。临床主要表现为急性眼肌麻痹(眼外肌麻痹7例,眼内肌麻痹1例),共济失调4例,意识障碍4例,腱反射减弱或消失5例,病理征阳性8例,累及面神经4例,累及舌咽和迷走神经6例,同时累及脑神经(面神经、舌咽、迷走神经、舌下神经)3例。2例患者脑脊液检查正常,4例头颅MRI检查无特征性表现,2例在脑干内相应部位有异常病灶,1例硬脑膜广泛强化。5例行血清抗GQ1b抗体检查者均为阴性。8例患者均使用免疫球蛋白或联合糖皮质激素治疗,7例患者预后较好,1例患者死亡。结论:BBE多呈单相良性病程,确诊主要依据临床表现、血清抗体、脑脊液病原基因组、影像学及神经电生理作为辅助诊断,免疫球蛋白或联合糖皮质激素治疗有效。
Objective: To investigate the clinical features of Bickerstaff brainstem encephalitis(BBE)improving disease awareness and standards of diagnosis and treatment. Methods: Clinical data of 8 BBE patients were collected and retrospectively analyzed. Relevant literature was reviewed to summarize clinical features, pathogenesis, treatment, and prognosis of the disease. Results: All 8 patients showed acute onset.Before onset of BBE, 2 cases displayed upper respiratory infection, 2 cases diarrhea, and 1 case varicella zoster virus infection. In 3 cases, the initial symptom was numbness and weakness of the limbs with disorganized speech. There was 1 case each of unclear consciousness, weakness of both lower limbs, hoarseness with dysphagia, numbness of face and fingers, and dizziness with unsteady gait. The main clinical manifestations were as follows: acute ophthalmoplegia(extraocular n=7, intraocular n=1), ataxia(n=4), impaired consciousness(n=4), deep tendon hyporeflexia or areflexia(n=5), positive pathology(n=8), facial nerve involvement(n=4),glossopharyngeal and vagus nerve involvement(n=6), and simultaneous involvement of cranial nerves(facial,glossopharyngeal, vagus, and hypoglossal nerves, n=3). Cerebrospinal fluid examination was normal in 2 patients, head MRI examination showed no characteristic manifestations in 4 patients, abnormal lesions in corresponding parts of the brainstem were found in 2 patients, and extensive dural enhancement was seen in 1 patient. All 5 cases that underwent routine serum GQ1b antibody testing were negative. All 8 patients were treated with immunoglobulin or combined glucocorticoid; 7 patients had a good prognosis, and 1 patient died.Conclusion: BBE mostly presents a single-phase benign course. Diagnosis is mainly based on clinical manifestations, and serum antibodies, cerebrospinal fluid pathogen genome, imaging, and neuroelectrophysiology serve in auxiliary diagnosis. Immunoglobulin or combined glucocorticoid therapy is effective treatment.
Objective: To investigate the clinical features of Bickerstaff brainstem encephalitis(BBE)improving disease awareness and standards of diagnosis and treatment. Methods: Clinical data of 8 BBE patients were collected and retrospectively analyzed. Relevant literature was reviewed to summarize clinical features, pathogenesis, treatment, and prognosis of the disease. Results: All 8 patients showed acute onset.Before onset of BBE, 2 cases displayed upper respiratory infection, 2 cases diarrhea, and 1 case varicella zoster virus infection. In 3 cases, the initial symptom was numbness and weakness of the limbs with disorganized speech. There was 1 case each of unclear consciousness, weakness of both lower limbs, hoarseness with dysphagia, numbness of face and fingers, and dizziness with unsteady gait. The main clinical manifestations were as follows: acute ophthalmoplegia(extraocular n=7, intraocular n=1), ataxia(n=4), impaired consciousness(n=4), deep tendon hyporeflexia or areflexia(n=5), positive pathology(n=8), facial nerve involvement(n=4),glossopharyngeal and vagus nerve involvement(n=6), and simultaneous involvement of cranial nerves(facial,glossopharyngeal, vagus, and hypoglossal nerves, n=3). Cerebrospinal fluid examination was normal in 2 patients, head MRI examination showed no characteristic manifestations in 4 patients, abnormal lesions in corresponding parts of the brainstem were found in 2 patients, and extensive dural enhancement was seen in 1 patient. All 5 cases that underwent routine serum GQ1b antibody testing were negative. All 8 patients were treated with immunoglobulin or combined glucocorticoid; 7 patients had a good prognosis, and 1 patient died.Conclusion: BBE mostly presents a single-phase benign course. Diagnosis is mainly based on clinical manifestations, and serum antibodies, cerebrospinal fluid pathogen genome, imaging, and neuroelectrophysiology serve in auxiliary diagnosis. Immunoglobulin or combined glucocorticoid therapy is effective treatment.
引文
[1]Bickerstaff ER,Cloake PC.Mesencephalitis and rhombencephalitis[J].Br Med J,1951,2:77-81.
[2]Bickerstaff ER.Brain-stem encephalitis:further observations on a grave syndrome with benign prognosis[J].Br Med J,1957,1:1384-1387.
[3]Odaka M,Yuki N,Yamada M,et al.Bickerstaff's brainstem encephalitis:clinical features of 62 cases and a subgroup associated with Guillain-barrésyndrome[J].Brain,2003,126:2279-2290.
[4]Wakerley BR,Uncini A,Yuki N,et al.Guillain-Barréand Miller Fisher Syndromes--new diagnostic classification[J].Nat Rev Neurol,2014,10:537-544.
[5]Garg M,Kulkarni S,Udwadia Hegde A.Herpes simplex encephalitis with thalamic,brainstem and cerebellar involvement[J].Neuroradiol J,2018,31:190-192.
[6]Ricigliano VAG,Saraceno L,Cavalli M,et al.Slowly progressing varicella zoster brainstem encephalitis complicating Ramsay Hunt syndrome in an immunocompetent patient:case report and review of the literature[J].J Neurovirol,2017,23:922-928.
[7]Phowthongkum P,Phantumchinda K,Jutivorakool K,et al.Basal ganglia and brainstem encephalitis,optic neuritis,and radiculomyelitis in Epstein-Barr virus infection[J].J Infect,2007,54:e141-144.
[8]Karlsson WK,Harboe ZB,Roed C,et al.Early trigeminal nerve involvement in Listeria monocytogenes rhombencephalitis:case series and systematic review[J].J Neurol,2017,264:1875-1884.
[9]Reynauda L,Graf M,Borgia G,et al.A rare case of brainstem encephalitis by Listerria monocytogenes with isolated mesencephalic localization[J].Diagn Microbiol Infec Dis,2007,58:121-123.
[10]Yuki N,Shahrizaila N.How do we identify infectious agents that trigger Guillain Barre syndrome,Fisher syndrome and Bickerstaff brainstem encephalitis[J].J Neurol Sci,2011,302:1-5.
[11]Yuki N,Taki T,Takahashi M,et al.Molecular mimicry between GQlb ganglioside and Iipopolysaccharides of Campylobacter jejuni isolated from patients with Fisher’s syndrome[J].Ann Neurol,1994,36:79l-793.
[12]Yuki N,Hartung HP.Guillain-Barrésyndrome[J].N Engl J Med,2012,366:2294-2304.
[13]Kimoto K,Koga M,Odaka M,et al.Relationship of bacterial strains to clinical syndromes of Campylobacter associated neuropathies[J].Neurology,2006,67:1837-1843.
[14]Ito M,Kuwabara S,Odaka M,et al.Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum:clinical analysis of 581 cases[J].J Neurol,2008,255:674-682.
[15]Hughes RAC,Swan AV,van Doorn PA.Corticosteroids for Guillain-barrésyndrome[J].Cochrane Database Syst Rev,2010,17:CD001446.
[16]Roos RP,Soliven B,Goldenberg F,et al.An elderly patient with Bickerstaff brainstem encephalitis and transient episodes of brainstem dysfunction[J].Arch Neurol,2008,65:821-824.
[17]Kuwabaras S.Fisher syndrome and Bickerstaff brainstem encephalitis[J].Rinsho Shinkeiqaku,2014,54:1056-1057.
[18]Kuitwaard K,van Koningsveld R,Ruts L,et al.Recurrent GuillainBarrésyndrome[J].J Neurol Neurosurg Psychiatry,2009,80:56-59.
[19]Mossberg N,Nordin M,Movitz C,et al.The recurrent GuillainBarrésyndrome:a long-term population-based study[J].Acta Neurol Scand,2012,126:154-161.
[2]Bickerstaff ER.Brain-stem encephalitis:further observations on a grave syndrome with benign prognosis[J].Br Med J,1957,1:1384-1387.
[3]Odaka M,Yuki N,Yamada M,et al.Bickerstaff's brainstem encephalitis:clinical features of 62 cases and a subgroup associated with Guillain-barrésyndrome[J].Brain,2003,126:2279-2290.
[4]Wakerley BR,Uncini A,Yuki N,et al.Guillain-Barréand Miller Fisher Syndromes--new diagnostic classification[J].Nat Rev Neurol,2014,10:537-544.
[5]Garg M,Kulkarni S,Udwadia Hegde A.Herpes simplex encephalitis with thalamic,brainstem and cerebellar involvement[J].Neuroradiol J,2018,31:190-192.
[6]Ricigliano VAG,Saraceno L,Cavalli M,et al.Slowly progressing varicella zoster brainstem encephalitis complicating Ramsay Hunt syndrome in an immunocompetent patient:case report and review of the literature[J].J Neurovirol,2017,23:922-928.
[7]Phowthongkum P,Phantumchinda K,Jutivorakool K,et al.Basal ganglia and brainstem encephalitis,optic neuritis,and radiculomyelitis in Epstein-Barr virus infection[J].J Infect,2007,54:e141-144.
[8]Karlsson WK,Harboe ZB,Roed C,et al.Early trigeminal nerve involvement in Listeria monocytogenes rhombencephalitis:case series and systematic review[J].J Neurol,2017,264:1875-1884.
[9]Reynauda L,Graf M,Borgia G,et al.A rare case of brainstem encephalitis by Listerria monocytogenes with isolated mesencephalic localization[J].Diagn Microbiol Infec Dis,2007,58:121-123.
[10]Yuki N,Shahrizaila N.How do we identify infectious agents that trigger Guillain Barre syndrome,Fisher syndrome and Bickerstaff brainstem encephalitis[J].J Neurol Sci,2011,302:1-5.
[11]Yuki N,Taki T,Takahashi M,et al.Molecular mimicry between GQlb ganglioside and Iipopolysaccharides of Campylobacter jejuni isolated from patients with Fisher’s syndrome[J].Ann Neurol,1994,36:79l-793.
[12]Yuki N,Hartung HP.Guillain-Barrésyndrome[J].N Engl J Med,2012,366:2294-2304.
[13]Kimoto K,Koga M,Odaka M,et al.Relationship of bacterial strains to clinical syndromes of Campylobacter associated neuropathies[J].Neurology,2006,67:1837-1843.
[14]Ito M,Kuwabara S,Odaka M,et al.Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum:clinical analysis of 581 cases[J].J Neurol,2008,255:674-682.
[15]Hughes RAC,Swan AV,van Doorn PA.Corticosteroids for Guillain-barrésyndrome[J].Cochrane Database Syst Rev,2010,17:CD001446.
[16]Roos RP,Soliven B,Goldenberg F,et al.An elderly patient with Bickerstaff brainstem encephalitis and transient episodes of brainstem dysfunction[J].Arch Neurol,2008,65:821-824.
[17]Kuwabaras S.Fisher syndrome and Bickerstaff brainstem encephalitis[J].Rinsho Shinkeiqaku,2014,54:1056-1057.
[18]Kuitwaard K,van Koningsveld R,Ruts L,et al.Recurrent GuillainBarrésyndrome[J].J Neurol Neurosurg Psychiatry,2009,80:56-59.
[19]Mossberg N,Nordin M,Movitz C,et al.The recurrent GuillainBarrésyndrome:a long-term population-based study[J].Acta Neurol Scand,2012,126:154-161.