Cerebral venous sinus thrombosis in Beh‡et's disease: a retrospective case-control study

The objective of the study is to investigate the clinical features of Beh double dagger et's disease (BD) complicated with cerebral venous sinus thrombosis (CVST). We retrospectively analyzed the clinical features of age- and gender-matched BD patients with and without CVST. Of the 840 BD patients enrolled, 21 (2.5%, 11 female, mean age 30.3 +/- 9.5) were diagnosed as CVST. Eighteen patients (85.7%) developed neurologic manifestations after BD onset, with an average interval of 47.6 months (SD 36.1). Onset of neurologic symptoms was mostly progressive (89.5%), with headache (95.2%) being the most common symptom. Intracranial hypertension occurred in 17 cases (89.5%). Thrombosis was detected mostly in transverse (71.4%) and superior sagittal (66.7%) sinuses, with more frequently (66.7%) dual or multiple sinuses involved. In addition, extra cranial thrombosis was presented in 11 cases. Cerebral infarction was presented in four cases. Compared with BD patients without CVST, the prevalence of extra cranial vascular involvement (52.4 versus 20.6%; P = 0.005) and BDCAF 2006 score (3.7 +/- 1.4 versus 1.8 +/- 1.3; P = 1.7863 x 10(-7)) was higher in BD patients with CVST. During a median 7-month follow-up (range 0.3-88.8 months), 18 patients (85.7%) achieved remission following glucocorticoid, immunosuppressant therapy, anticoagulants, and dehydration, although 3 patients experienced (14.3%) flare. No death was reported. CVST is rare complication of BD and predominately progressively developed in active BD and after BD onset. It typically features intracranial hypertension syndrome and is frequently complicated with extra cranial thrombosis; thus, systemic thrombus screening is highly recommended. Promptly, diagnosis and aggressive treatment are keys to improve the prognosis.