Hypertrophic Olivary Degeneration Secondary to Neuro-Behçet’s Disease

Excerpt

Behçet’s disease (BD) is a multisystem inflammatory disease of unknown etiology, characterized by intense inflammatory perivasculitis [1]. It mainly affects the skin and mucous membranes, leading to recurrent oral and genital ulcers, skin lesions, and uveitis [1‐3]. Neurological involvement in BD is rare and occurs most often in the set of systemic disease [1‐5]. Several neurological syndromes may ensue, but meningoencephalitis is the most common, representing 75 % of central nervous system lesions [1]. Rhombencephalitis is usually the main clinical picture, but there is also inflammatory damage to the thalamus, basal ganglia, cerebellum and cerebral cortex, a combination known as brainstem-plus sign [6]. …