ADAMANTIADIS-BEHCET SYNDROME AND CNS: CASE-REPORT

Abstract

A 44-year-old woman, was admitted to our clinic with a 3-year history of slow cognitive decline, gait unsteadiness, dysarthria and behavioral changes. From her history, she had been operated for left breast cancer five years ago. Moreover, she has experienced episodes with recurrent oral ulcers and one episode with genital ulcers. On examination, she had left-sided hemiparesis with ataxia, pseudo bulbar speech, chorea in the arms and face, spastic-ataxic gait and urinary incontinence. She scored 81/100 on the Addenbrooke’s Cognitive Examination. Further investigation revealed hyper-intense lesions in T2-weighted and FLAIR images, located in the periventricular region, the brainstem, the cerebellar peduncles and the internal capsule bilaterally. The electroencephalogram showed an encephalopathetic pattern. The patient was found positive for the HLA-B51 allele. Extensive laboratory testing ruled out Neuro-Behcet mimics and the patient was diagnosed with neuro-Behcet, based on current criteria.