Parenchymal neuro-Behcet's disease. Clinical and paraclinical characteristics, a report of 40 cases

Describe the clinical and paraclinical characteristics of parenchymal neuro-Behcet's disease. This is a prospective, descriptive study, concerning 40 patients with parenchymal neuro-Behcet's disease. The patients were followed during 3 years, benefited a thorough physical examination and paraclinical made of inflammatory laboratory tests, infectious serology, serum/CSF autoimmunity assessment, brain/spine MRI and evoked potentials. We also evaluated the frequency of HLA-B51. We identified 22 men and 18 women. The average age was 32 years. The beginning was poly-symptomatic in 65% cases. Twenty-eight patients (70%) reported a decrease in visual acuity, 40% (16 cases) associated with uveitis, 33 cases (82.5%) complained of headache and 11 cases (27.5%) with dizziness. Inaugural signs consisted of motor disorders (50%) and balance disorders (40%). The inflammatory serum markers were positive in 75% and oligoclonal bands present in CSF were found in 7 patients. Infracentimetric demyelinating lesions in MRI study were located in the brainstem (52.5%), the subcortical white matter (40%), the periventricular region (42.5%), cerebellum (32.5%), basal ganglia (30%), internal capsule (25%) and corpus callosum (12.5%). The HLA-B51 was found in 53% of cases. Behçet's disease mainly affects young male. The neurological complications are highly polymorphic, involving severe vital or functional prognosis.