Neurological involvement in Behçet's disease: evaluation of 67 patients

Background: Neurological manifestations of Behçet disease (BD) are polymorphic and serious. The purpose of this study was to analyze the clinical patterns and outcome of neurological involvement in BD.

Methods: The medical records of patients with neurological manifestations of BD were reviewed retrospectively. All patients fulfilled the International Study Group Criteria for the Diagnosis of BD. Patients with headache and normal findings (neurological examination, cerebrospinal fluid, computed tomography scan, magnetic resonance imaging) were excluded.

Results: Sixty-seven patients had clinical evidence of neurological involvement. There were 53 men and 14 women. A male/female sex ratio was 3.78. The average age of onset of neurological involvement was 31.5. The meningoparenchymal (MP) central nervous system involvement was found in 83.58%. The most common findings were pyramidal signs. Eleven patients (16.41%) without parenchymal central nervous system involvement were noted. In this group, there were six cases of intracranial thrombosis, one case of intracranial aneurysm and four cases of idiopathic intracranial hypertension. Other clinical features were reported: extrapyramidal signs and isolated spinal cord involvement. The course of disease was favorable in 70% of cases, and unfavorable in 30%.

Conclusions: Clinical patterns of neurological involvement in BD are various and particularly serious in the MP group.