Analysis on clinical characteristics of chronic progressive parenchymal neuro-Behçet's syndrome

Abstract

Objective To summarize the clinical manifestations of chronic progressive parenchymal neuro-Behçet's syndrome (CPPNBS). Methods The diagnosis and treatment of one patient with CPPNBS was reviewed and analyzed. Taking neuro Behçet's syndrome, neuro Behçet's disease AND chronic progressive (in English and Chinese) as search terms, retrieve in databases such as PubMed, China National Knowledge Infrastructure (CNKI) and Wanfang Data, in order to collect clinical studies and case reports of CPPNBS from January 1, 1980 to December 1, 2018, summarizing the sociodemographic, clinical, laboratory and imaging features, treatment and prognosis of CPPNBS. Results A total of 14 English articles with 122 cases were enrolled after searching. Combined with our case, there were 123 cases. CPPNBS was common in male, and the peak onset age ranged 45-47 years old, with a chronic disease course. The common clinical manifestations were ataxia, pseudobulbar palsy and cognitive impairment. Elevated level of interleukin-6 (IL-6) in cerebrospinal fluid (CSF) was the specific laboratory index. Brain stem atrophy in MRI was the specific imaging feature. Glucocorticoids and immunosuppressive agents were the main treatment option. The prognosis was poor. Conclusions CPPNBS is a rare subtype of Behçet's syndrome (BS), and could be easily missed or misdiagnosed. The clinical manifestations are not specific. CSF and MRI examinations are important for the diagnosis. The prognosis of this disease is poor. The rates of disability and mortality are high.

DOI: 10.3969/j.issn.1672-6731.2019.06.011