Clinical Characteristics Of Neuro-Behcet's Disease: A Retrospective Analysis

Objective:To investigate the clinical characteristics of neurological involvement in Behcet's disease.Methods:We retrospectively reviewed the clinical,laboratory,imaging data,therapy and prognosis of a cohort of BD patients who were in patients at Peking Union Medical College Hospital between January 2000 and June 2016.SPSS 21.0 software was used for statistical analysis.Results:Data on a total of 1009 inpatients with BD during that period of whom 62 patients(6.1%,37 male and 25 female)had neurological involvement.Their age at on set of BD was(29.0±10.8)years old and age at neurological onset was(33.9±11.7)years old.Most of patients suffered neurological symptoms after other initial systemic symptoms of BD in 3 months(range from 0-50).In 10 cases(16.1%)neurological onset was concomitant with the onset of BD.59 patients had central nervous system involvement.42 cases(67.7%)with parenchymal involvement(pNBD),mainly involving brainstem and hemisphere.13 cases suffered multiple lesions.17 cases with non-parenchymal involvement were all presented as cerebral venous sinus thrombosis(CVST).The most frequent locations of CVST were superior sagital sinus and transverse sinus.3 cases had peripheral nervous system involvement.The most frequent neurological signs of pNBD were pyramidal,headache and behavioural change.In non-pNBD,headache,blurred vision and papilledema were common.CSF examination was performed in 57 cases.For pNBD,80%(32/40)patients had normal pressure and 55%(22/40)had elevated protein levels.(9/20)showed lymphocytic predominance and(2/20)showed neutrophilic predominance.For non-pNBD,14 out of 15 patients(93.3%)were found elevated pressure with normal cells and only 3 patients had elevated protein.76.2%pNBD's Cranial MR showed the lesions were in the midline structures,such as brainstem,lateral ventricles and semi-oval center.Typically,the lesions were in hypersignal in T2.All pNBD patients recevied corticosteroids(?mg/Kg/d),23 out of 42 cases(54.8%)received the pulse dose.CTX is the most commonly used immunosuppressant and 10 cases received more than one immunosuppressants(such as MTX?AZA).6 cases received biological agents(4 Infliximab,1Tocilizumab,linterferon-a2a)and 28 cases received intrathecal injection therapy.Non-pNBD patients received corticosteroids,immunosuppressants as well as anticoagulant and dehydration therapy.With a median follow-up of 36 months(0.3 to 156 months),23 cases in pNBD achieved clinical improvements,8 patients relapsed and 3 patients died.2 non-pNBD patients relapsed and no death was reported in non-pNBD cases.Conclusions:The neurological involvement in BD occurs more frequently in male patients.Parenchymal involvement is more common and its most frequent location is brainstem.The mortality is higher.CVST,as the most common non-parenchymal NBD,often has a good prognosis.Early identification and active treatment are vital for NBD.