Neurological Involvement in Behçet’s Disease

Abstract

Behçet's disease is a systemic vasculitis that manifests in outbreaks and can affect the skin, mucosa, joints, vessels, eyes, and nervous and gastrointestinal systems. Neurological involvement is present in 5% of patients with this disease. It is classified into parenchymatous, which comprises the cerebral form, and nonparenchymal, related to cerebral venous thrombosis and intracranial hypertension. CASE REPORTA 40-year-old woman, former drug user, hospitalized with convulsive crisis, fever and severe holocranial headache, was diagnosed with bacterial meningitis, liquor with gram-positive coconuts. She was treated with ceftriaxone and vancomycin. During hospital evolution, she presented hemiparesis of the left hemibody without etiological definition. She was discharged from hospital with outpatient follow-up with a neurologist, who requested a brain nuclear magnetic resonance, which showed poorly delimited hypodense areas, involving the bridge, midbrain and nucleocapsular regions, expanding them. In view of the suspected examination of central nervous system vasculitis, she was referred for emergency hospitalization and pulse therapy with methylprednisolone 1 g for 5 days was started. It evolved with significant recovery of the strength of the left hemibody, being redirected to follow-up with outpatient neurologist. Due to delay in outpatient consultations, after 8 months, the patient presented hemiplegia of the right hemibody, paralysis of the VI cranial nerve to the right, central facial paralysis, dysarthria, dysphagia and ataxia. During the systems review, recurrent oral and genital ulcers were observed. Pulse therapy was initiated with methylprednisolone 1 g for 5 days and cyclophosphamide 1 g, due to the diagnosis of Behçet's disease with central nervous system involvement. After pulse therapy, she presented significant improvement of the neurological picture, with partial recovery of strength and partial improvement of dysphagia. She maintained cyclophosphamide with monthly infusions. After the sixth dose, a new cranial magnetic resonance imaging revealed great improvement in brain stem injury. CONCLUSIONWe conclude that effective patient care, based on physical examination and clinical history, is extremely important. Behçet's disease presents a varied form of manifestations, including the neurological form, which is related to high morbidity and mortality rates.