Enfermedad de Behçet con doble afectación neurológica atípica

Abstract

Neurological involvement in Behçet s disease (BD) occurs in 10 30% of the cases and is usually central, with the peripheral nervous system being rarely involved. It is also unusual for the disorder to present with neurological features at the onset.A 51 year old man with an undefined history of brainstem encephalitis and epileptic seizures. He later developed a cerebellar syndrome and relapsing orogenital ulcers, diagnosed as BD with involvement of the central nervous system. At the present time the patient complained of marked deterioration in gait and progressive difficulty with carrying out everyday tasks using his upper limbs. On neurological examination there was paresia of the right VI cranial nerve, hyporeflexia of the left half of his body, left hypo esthesia (slight for touch and discrimination, but marked for proprioception) and dissymmetry of upper and lower left limbs. Complementary investigations showed there to be sensory polyneuropathy of the left arm and leg. Both central and peripheral nervous system alterations improved on steroid treatment.The probable onset of BD with central nervous system alterations and subsequent appearance of peripheral neuropathy are infrequent findings in BD. The relation of both disorders with vasculitis is reinforced by the good response to immunosuppressive treatment.