Recurrent Neurological Episodes for 10 Years Preceding Skin Lesions in Neuro-Sweet Disease

An absence of skin lesions at the neurological onset may obscure the diagnosis of neuro-Sweet disease (NSD). We herein report a 32-year-old man with NSD in whom neurological symptoms preceded the development of skin lesions by 10 years. The patient exhibited four distinct neurological episodes: meningoencephalitis, scattered brain lesions, ocular flutter, and isolated seizures. Acute relapses responded to corticosteroid therapy, and the patient was successfully maintained on corticosteroid and dapsone combination therapy. NSD should be considered in the differential diagnosis of patients with recurrent neurological manifestations, especially with both meningeal and brain parenchymal involvement, even if no skin lesions are observed.