[Neurological involvement in Behçet's disease. 154 cases from a cohort of 925 patients and review of the literature]

Abstract

Introduction: Neurological involvement is well described in Behçet's Disease (BD), with variable prevalence of 5.3 to 30p.cent. The purpose of this retrospective study was to analyze the clinical patterns of neuro-Behçet (NB) and to compare them with different clinical features of 925 BD registered in the same period and the literature.

Methods: All patients of NB fulfilled the International Study Group Criteria for the diagnosis of BD. 93 patients with headache, without other neurological symptoms were excluded. The findings were supported by cerebrospinal fluid, computed tomography scan, magnetic resonance imaging and angiography.

Results: NB was present in 16.64p.cent of BD. A sex ratio male/female: 4.31. The mean age of patients with NB was 31.76 years. The average age of onset of NB was 29.83. The mean duration of the NB disease was 3.81 years. The findings were categorised in 2 main types: parenchymal and non-parenchymal involvement. Some patients had features of both types (mixed patterns). 94 patients (61.03p.cent) presented with parenchymal central nervous system (CNS) involvement. The most common findings were pyramidal signs, cranial nerve palsies, pseudobulbar syndrome and cerebellar signs. 27 patients (17.53p.cent) without parenchymal CNS which were divided into: intracranial hypertension in 24 patients (15.58p.cent) presenting headache, vomiting and bilateral papilloedema; cerebro-arterial involvement in 3 cases (one of them had cerebrovascular aneurysms). Mixed patterns were observed in 9 cases. Erythema nodosum and vascular involvement were more frequent in intracranial hypertension than in BD. Other clinical features were reported: pure meningeal pattern in 15 cases, pure peripheral nervous system involvement in 4 cases, isolated cranial nerves in 8 cases, chorea in one patient and pseudotumor of cervical medulla in other patient. 6 cases of juvenile BD and one case of familial Behçet were observed. 133 patients were treated (86.36p.cent). Mean duration of treatment was 7.46 month. We used corticosteroids, immunosuppressive agents (cyclophosphamid, azathioprine, chlorambucil) and anticoagulant in intracranial hypertension. The course of disease was good in only 54.13p.cent of cases, and was bad in 18.79p.cent.

Conclusion: NB occurred frequently in men and is more serious especially in parenchymal CNS involvement: 7 deaths (6 from parenchymal CNS).