Cerebral venous thrombosis in Behçet's disease compared to those associated with other etiologies

Department of Neurology, Istanbul Medical School, Capa, 34293 Istanbul, Turkey.
Journal of Neurology (Impact Factor: 3.38). 03/2009; 256(7):1134-42. DOI: 10.1007/s00415-009-5088-4
Source: PubMed


Cerebral venous thrombosis (CVT) is caused by various etiologies. In Mediterranean and Middle Eastern countries, Behçet's disease (BD) is one of the leading causes of CVT. We aimed to evaluate any differences in CVT patients with and without BD. All registered patients with CVT were evaluated retrospectively. Clinical, neuroradiological findings and follow-up data were compared between patients with BD and patients with other etiologies. There were 36 patients with CVT and BD, and 32 patients with CVT related to other etiological causes. BD patients were younger (median age at onset 26 vs. 39 years; P < 0.001), and there was a male preponderance (28 males, 8 females) as compared to the non-BD group (10 males, 22 females; P < 0.001). Onset was frequently acute in the non-BD group, and it was subacute or chronic in the BD group. Hemi/quadriparesis, aphasia and seizures were significantly more common (P < 0.001) in the non-BD group. In the BD group 94% of the patients presented with symptoms of isolated intracranial hypertension (P < 0.001). Venous infarcts were observed in 63% of the patients with other causes and in 6% of the patients with BD (P < 0.001). At admission 97% of the patients in the BD group and 41% of the patients in the non-BD group had a modified Rankin score of 0-2. Outcome was good in all of the patients with BD and in 91% of patients with other causes. Clinical recurrences were seen in six patients with BD and in one patient without BD. CVT associated with BD has a subacute onset, mostly presents with signs of isolated intracranial hypertension and venous infarction rarely develops; these features distinguish CVT due to BD from those with other causes.

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    • "Patient with seizures before the diagnosis of NBD had only severe headache. Good prognosis was reported for BD patients with CVT like our patients [18]. The antiepileptic treatment was stopped in two patients while we decided to terminate the treatment in other two patients. "
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    ABSTRACT: Behcet disease (BD) is a chronic relapsing inflammatory disorder. Neuro BD (NBD) is seen in approximately 5% of all patients. The aim of this study is to investigate the frequency, type and prognosis of epileptic seizures in different forms of NBD. All files of 42 patients with NBD were evaluated between 2006 and 2012, retrospectively. The demographic data, the presentation of NBD, clinical findings including seizures, EEG and neuroimaging findings were reviewed. The mean age of patients was 35.02±8.43 years. Thirty (71.4%) patients were male; the remaining 12 of them were female. Twenty-four patients had brainstem lesions; 16 patients had cerebral venous thrombosis. Spinal cord involvement was seen in two patients. Seven patients had epileptic seizures (six partial onset seizures with or without secondary generalization). Six of them had cerebral sinus thrombosis (CVT). Four patients had a seizure as the first symptom of the thrombosis. One patient had late onset seizure due to chronic venous infarct. The other patient with seizure had brainstem involvement. The remaining was diagnosed as epilepsy before the determination of NBD. CVT seen in BD seems to be the main risk factor for epileptic seizures in patients with NBD. The prognosis is usually good especially in patients with CVT. Epileptic seizures in patients with brainstem involvement may be an indicator for poor prognosis. Superior sagittal thrombosis or cortical infarct would be predictor of seizures occurrence because of the high ratio in patients with seizures. Copyright © 2015 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.
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    • "Massive subarachnoid hemorrhage might occur in the course of CVT [18]. In one of the 32 cases with CVT caused by other etiologies than Behçet's disease, recurrent lobar hematomas were a remarkable clinical presentation [19]. SDH is an unusual complication of CVT. "
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    ABSTRACT: Spinal subdural hematoma (SDH) is a rare condition and can be caused by several factors. Concomitant cranial and spinal SDH is even much less common. We present a 77-year-old male patient with lower back pain, paraparesis, and urinary retention following a sudden onset headache. Imaging revealed concomitant cranial and spinal SDH related to cerebral venous thrombosis (CVT) associated with hemorrhagic venous infarct. Laboratory examinations were consistent with polycythemia vera. There was no history of trauma and previous cranial surgery. Brain angiography did not reveal any evidence of arteriovenous fistula or vascular malformation. Since lower back pain occurred shortly after the headache and there was no other reasonable explanation for spinal hemorrhage, we suppose that the mechanism of spinal SDH is the migration of blood from the intracranial compartment. Therefore, this is the first report of concomitant spinal SDH and cerebral hemorrhage associated with CVT in a patient with myeloproliferative disease.
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