Clinical characteristics of neuro-Behcet’s disease in Japan: a multicenter retrospective analysis
Open Access
- 21 September 2011
- journal article
- research article
- Published by Oxford University Press (OUP) in Modern Rheumatology
- Vol. 22 (3), 405-413
- https://doi.org/10.1007/s10165-011-0533-5
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 patients were collected; 76 with acute NBD, 35 with CP NBD, and 33 with non-NBD. High-intensity 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 those with CP NBD. 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 of CP NBD versus the recovery phase of acute NBD 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 brainstem atrophy on MRI are useful for the diagnosis of NBD.Keywords
This publication has 22 references indexed in Scilit:
- CSF levels of cytokines in neuro-Behçet's diseaseClinical Neurology and Neurosurgery, 2009
- Neuro-Behçet's disease: epidemiology, clinical characteristics, and managementThe Lancet Neurology, 2009
- Interleukin-6 in neuro-Behçet’s disease: Association with disease subsets and long-term outcomeCytokine, 2008
- Successful treatment of refractory neuro-Behcet's disease with infliximab: a case report to show its efficacy by magnetic resonance imaging, transcranial magnetic stimulation and cytokine profileAnnals Of The Rheumatic Diseases, 2006
- Headache in Behçet's SyndromeHeadache: The Journal of Head and Face Pain, 2005
- Intrathecal activation of the IL-17/IL-8 axis in opticospinal multiple sclerosisBrain, 2005
- Beh??et??s syndrome and the nervous systemCurrent Opinion in Neurology, 2004
- Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patientsBrain, 1999
- Cerebrospinal Fluid Interleukin-6 in Progressive Neuro-Behçet's SyndromeClinical Immunology and Immunopathology, 1997
- Cytokine accumulations in CSF of multiple sclerosis patientsNeurology, 1990