Comparative Study On Bickerstaff’s Brainstem Encephalitis And Miller Fisher Syndrome

Objective:The aims of our study were to (1) clarify the clinical profiles, outcomes and nosological relationship of Bickerstaff s brainstem encephalitis and Miller Fisher syndrome by studying their clinical features, imaging and laboratory characteristics (2) investigate the effectiveness of immunomodulating treatment on Bickerstaff’s brainstem encephalitis and Miller Fisher syndrome (3) observe whether the therapeutic response of immunotherapy differs between the two.Method:We reviewed medical records of32BBE and67MFS patients diagnosed by strict criteria at the second Xiangya Hospital and Xiangya Hospital in Changsha, China between2003and2013. We collected data on clinical profiles and outcomes of BBE and MFS. The recovery time was analyzed by the Kaplan-Meier method. Multivariate logistic regression analysis was performed to analyze risk factors for worse outcomes in BBE patients.Result:1. In. our study, we found that BBE and MFS shared many similarities:upper respiratory infection was the most frequent preceding symptom; ophthalmoplegia and ataxia were the common clinical features. There was no significant difference between the two groups in the proportion of patients presenting with headache, internal ophthalmoplegia, bulbar palsy, facial palsy, superficial sense impairment. CSF albuminocytological dissociation, and abnormalities on nerve electrophysiology and brain MRI were present in both BBE and MFS, suggesting both central and peripheral nervous system involvement. Compared with MFS, our BBE patients had more evidence of CNS involvement because almost all had impaired consciousness, hyperreflexia, Babinski’s sign, and abnormal MRI and EEG findings.2. The Kaplan-Meier curves for the periods between the onset of impaired consciousness, ophthalmoplegia and ataxia and the start of alleviation in these symptoms show that (1) BBE patients were more likely to have faster alleviation of impaired consciousness when treated with combination of IVIg and steroids than control (p<0.05), whereas those between the IVIg group and the control group and those between the steroid group and the control group did not significantly differ in impaired consciousness, ophthalmoplegia and ataxia(p>0.05).(2) There was no significant differences among IVIg, PE, steroids, the combinations of IVIg with steroids and control group in the start of the alleviation and the disappearance of ophthalmoplegia and ataxia in MFS patients (p>0.05).(3) There was no difference between each therapeutic response of immunotherapy in BBE an MFS patients.3. Both outcomes generally are good, by1year after disease onset21(66%) of the32BBE patients and66(97%) of the67MFS patients show complete remission with no residual symptoms. In addition,9BBE patients died. The univariable analysis results show as following: the short-term prognostic differences of those patients who having fever, with disturbance of consciousness and assisted ventilation have statistically significant (p<0.05). The multivariate logistic regression analysis results showed assisted ventilation was the independent risk factor influencing the short-term prognosis of patients with BBE (p<0.05).Conclusions:1. Because of the similarities in the clinical presentations of BBE and MFS, both have CSF albuminocytological dissociation, both with variable central and peripheral nervous system involvement, we could make conclusive that BBE and MFS are not two distinct conditions, but form a single autoimmune continuous spectrum.2. IVIg combination with steroids woud be recommended being used in BBE patients since data have suggested a slightly hastening in impaired consciousness recovery.3. The prognosis of the patients who having fever, being unconsciousness, or being dyspnoea needing assisted respiration at the onset of disease is poorer than those didn’t. Assisted ventilation was the independent risk factor influencing the short-term prognosis of patients with BBE.