Neuro-Behçet's Disease Presented with Progressive Disinhibition

Abstract

We report a man who presented with progressive disinhibition and through clinicoradiologic correlation using magnetic resonance imaging (MRI), aim to investigate the pathomechanism of disinhibition in neuro-Behçet's disease (NBD). A 46-year-old man presented with progressive disinhibition and apathy for 4 months. One month after his visit, additionally, he developed left partial third nerve palsy. His brain MRI showed lesions in the ventral caudate nucleus as well as left midbrain and thalamus. Taking his recurrent oral ulcers, uveitis, and erythema nodosum into consideration, he was diagnosed with NBD. We found that progressive disinhibition could be one of presenting symptoms in BD and might be associated with the caudate nucleus. This finding suggests that involvement of the basal ganglia in BD prior to the involvement of the brainstem could result in unique clinical features such as behavioral changes without extrapyramidal signs. Although neuropsychiatric manifestations have been reported in patients with NBD, those presenting with disinhibition has rarely been described [3,4]. Furthermore, correlation of the neuroanatomy with behavioral changes has barely been documented in literature. In a male patient who presented with progressive disinhibition, we investigated the pathomechanism of disinhibition in NBD through clinicoradiologic correlation using magnetic resonance imaging (MRI). CASE REPORTA 48-year-old man with diabetes was admitted to the department of neurology due to progressive behavioral changes.Several years ago, he experienced reddish skin in one of his legs after receiving an operation for varicose veins. Two years ago, he suffered from uveitis in his right eye and was administered with steroids. His family history was unremarkable.He was noted to have recurrent oral ulcers without genital ulcers. He was a calm and timid man who managed a pharmacy,