Vol.22 No.3

Original Article

Clinical characteristics of neuro-Behçet’s disease in Japan: a multicenter retrospective analysis

Authors

Shunsei Hirohata1 , Hirotoshi Kikuchi2 , Tetsuji Sawada3 , Hiroko Nagafuchi4 , Masataka Kuwana5 , Mitsuhiro Takeno6 , Yoshiaki Ishigatsubo6

  • Department of Rheumatology and Infectious Diseases, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
  • Department of Microbiology and Immunology, Teikyo University School of Medicine, Itabashi, Tokyo, Japan
  • Department of Internal Medicine 3, Tokyo Medical University, Shinjuku, Tokyo, Japan
  • Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Kanagawa, Japan
  • Department of Internal Medicine, Keio University School of Medicine, Shinjuku, Tokyo, Japan
  • Department of Internal Medicine and Clinical Immunology, Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa, Japan
Received:

5 July 2011

Accepted:

1 September 2011

Published online:

21 September 2011

Full Text

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Abstract

To delineate the clinical characteristics of neuro-Behçet’s disease (NBD), a multicenter retrospective survey was performed in BD patients who had presented any neurological manifestations between 1988 and 2008. The diagnosis of acute NBD, chronic progressive (CP) NBD, and non-NBD was confirmed by retrospective review of clinical records. Data on a total of 144 patientswere collected; 76with acute NBD, 35 with CP NBD, and 33 with non-NBD. Highintensity lesions on T2-weighted magnetic resonance imaging (MRI) were found in 60.5% of the patients with acute NBD, 54.2% with CP NBD, and 42.4% with non-NBD, whereas brainstem atrophy was observed in 7.5% with acute NBD, 71.4% with CP NBD, and 9.0% with non-NBD. The cerebrospinal fluid (CSF) cell count was prominently elevated in patients with acute NBD, but was normal in about 15% of thosewithCPNBD. The sensitivity and specificity of the CSF cell count for the diagnosis of acute NBD versus non-NBD were 97.4 and 97.0%, respectively (cut-off 6.2/mm3). The sensitivity and specificity of CSF interleukin (IL)-6 for the diagnosis ofCPNBDversus the recovery phase of acuteNBD were 86.7 and 94.7%, respectively (cut-off 16.55 pg/ml). The results indicate that elevation of the CSF cell count and CSF IL-6 and the presence of brainstematrophy onMRI are useful for the diagnosis of NBD.

Key words

Behçet’s disease - Central nervous system - Cerebrospinal fluid - Diagnostic criteria - Magnetic resonance imaging