Unique presentation of acute neuro-Behçet’s disease involving a cytotoxic oedema core surrounded by vasogenic oedema

Abstract

ABSTRACT A 53-year-old woman with recurrent stomatitis, genital ulcers, and folliculitis was admitted to Tokyo Medical University Hospital after experiencing visual disturbances for the past 2 weeks and a nonthrobbing headache for the past 3 days. She had also developed numbness in her left extremities. An ophthalmological examination revealed inflammatory changes in the eye. Cerebrospinal fluid analysis showed increased cell counts, protein, and interleukin-6 levels. Brain magnetic resonance imaging revealed multiple high signal intensities on T2-weighted/fluid-attenuated inversion recovery images of the pons and occipital and parietal lobes. The T2-weighted/fluid-attenuated inversion recovery high-signal-intensity lesion in the pons was hyperintense on diffusion-weighted imaging and hypointense on apparent diffusion coefficient mapping, suggesting cytotoxic oedema. Another high-signal-intensity lesion on T2-weighted/fluid-attenuated inversion recovery was isointense to hyperintense on diffusion-weighted imaging and hyperintense on apparent diffusion coefficient, indicating vasogenic oedema. The vasogenic oedema in the left occipital lobe contained a small core that was hyperintense on diffusion-weighted imaging and hypointense on apparent diffusion coefficient, suggesting cytotoxic oedema. The patient was diagnosed with acute neuro-Behçet’s disease and responded well to high-dose glucocorticoid and colchicine treatment. The present report emphasises that patients with acute neuro-Behçet’s disease may present with cytotoxic oedema in the pons and cerebral spheres. Further reports of similar cases would contribute to a better understanding of the role of cytotoxic oedema in the pathophysiology of neuro-Behçet’s disease and help elucidate the mechanisms underlying a unique presentation characterised by a central cytotoxic oedema core within vasogenic oedema.