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

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/mm(3)). 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.

Neuro-Behçet’s Disease: An Unusual Cause of Headache

“…Although no consensus definition for NBD exists, the diagnosis is generally made in a BD patient with headache or neurological symptoms in the presence of compatible abnormalities on neuroimaging. 2,6,7 Approximately 5-15 % of those with BD subsequently develop NBD, 6,8 but 45 % of those with NBD will have moderate to severe neurological disability at 10-year follow-up. 2 It remains unclear if the prompt recognition and specific treatment of NBD would improve long-term prognosis, 6,8 but the diagnostic approach to headache and use of neuroimaging in BD can be categorized by the types of headaches experienced by BD patients and associated clinical features (Fig.…”

“…8 In contrast, venous sinus thrombosis is the most common presentation of the second form of NBD, which is considered non-parenchymal or vascular NBD. 2,[6][7][8][9] Nearly all present with headache and papilledema, and less commonly with pyramidal signs. 8 Therefore, headache in the absence of other signs and symptoms would be an unusual presentation for NBD.…”

“…Up to 7 % are related to ocular inflammation, 6,10 while a smaller percentage may be due to trigeminal neuralgia or malignancy. [6][7][8]10 Finally, when neuroimaging is justified, MRI is the preferred diagnostic modality for NBD, with MRVadditionally recommended if venous thrombosis appears likely. 6,8,12,13 In one retrospective study of 200 patients with neurological findings consistent with NBD, 31 % of CT scans were found to be abnormal while 70 % of MRI scans were found to be abnormal.…”

“…8 In addition, because CSF analysis often demonstrates a pleocytosis with elevated protein levels in parenchymal NBD, this may be mistaken for infectious meningitis, given the simultaneous presence of meningeal signs and symptoms commonly seen in NBD. 6 Such studies consequently have equivocal diagnostic value in parenchymal NBD, 6,7 though abnormal CSF is considered a poor prognostic marker. 8 Additional markers indicative of poor prognosis include the presence of parenchymal disease, attack recurrence or progression, and residual neurological dysfunction.…”

Cerebral Venous Thrombosis in Behçet's Disease Patients Compared to Other Causes of Cerebral Venous Thrombosis: a Retrospective Study

“…[2,9,[11][12][13][14][15][16][17]20,30,[34][35][36][37][38][39][40] Moreover, warfarin and acetyl salicylic acid have been given to some CVT patients with BD. [10][11][12]17,26,34 Wechsler et al 14 reported that treatment of CVT during acute phase consisted of combined heparin and steroids in 19 patients, steroids alone in three, and heparin alone in three others.…”