Background Neurologic involvement in Behçet's disease ocurrs in 5-10% of cases. There are two patterns: parenchymal o non parenchymal involvement. Demyelinating lesions is a rare form of parenchymal afectation.
Objectives Our objective is to describe the clinical manifestations, laboratory test, the location of demyelinating lesions, treatment and evolution of these patients.
Methods A retrospective study was carried out in patients diagnosed of Behçet's disease between January 1990 and december 2013. The diagnosis was based on International criteria for diagnosis of Behçet's disease (International Study Group of Behçet's Disease), Magnetic Resonance Imaging (MRI) of the brain and results of cerebrospinal fluid.
Results 32 patients were diagnosed of Behçet's disease during this period, and 6 (18.7%) of them had demyelinating lesions in MRI. Mean age at diagnosis was 31 (15- 62) years and 4 (66.6%) were women. Clinical manifestations: headache in all, alteration of cranial nerves in 4 (66.6%) -two with bilateral optic neuritis and two with recurrent peripheral facial paralysis-, sensory deficits in 3 (50%), seizures in 2 (33.3%), Lhermitte's sign in 2 (33.3%), urinary incontinence in 3 (50%), ataxia in 1and transient motor deficit in 1. Three patients had low titer of positive ANA. The cerebrospinal fluid analysis showed no pleocytosis or elevated protein in any of them and three showed oligoclonal bands (50%). The MRI with gadolinium showed T2-weighted signal abnormality in the white matter in all of them (supratentorial and infratentorial). One patient had progression of lesions with involvement of the spinal cord and brainstem. Treatment: colchicine in 3, azathioprine in 3, oral corticosteroids in 3 and pulses of metilprednisolone in 5. In evolution, two patients are stable with low doses of corticosteroids; one remains stable without any treatment; one presents relapse despite infliximab and another progresses clinically and radiologically associated with poor adherence to treatment. One remains stable with adalimumab.
Conclusions Demyelinating lesions in Behçet disease are frequent and the clinical course may clinically and radiologically mimic MS. All patients with demyelinating lesions should be required about symptoms of systemic disease and a full autoantibodies investigation should be carried out. Patients should be followed for years, since the possibility of presenting a systemic disease is high (even if the autoantibodies are negative in the first investigation).
Disclosure of Interest : None declared