Neuro-Behçet disease, neuro-Sweet disease, and dementia

Kinya Hisanaga

Departments of Neurology and Clinical Research Center, NHO Miyagi National Hospital

Neuro-Behçet and neuro-Sweet diseases are multisystem inflammatory conditions characterized by muco-cutaneous and neuropsychiatric symptoms. Dementia is one of the neuropsychiatric symptoms, particularly in the chronic progressive subtype of neuro-Behçet disease. In both diseases the dysregulation of cytokines, following the onset of oral muco-cutaneous bacterial infection, may trigger an abnormal chemotaxis of neutrophils leading to ectopic encephalitis and meningitis, termed as neuro-neutrophilic diseases. Compared with controls, the frequencies of human leukocyte antigen (HLA)-B51 and -A26 in neuro-Behçet disease, along with HLA-B54 and -Cw1 in neuro-Sweet disease, are significantly higher. To date, various immunosuppressant drugs have been used as therapeutic agents. Overall, neuro-neutrophilic diseases constitute a type of dementia that is considered treatable.

Address correspondence to Dr. Kinya Hisanaga, Departments of Neurology and Clinical Research Center, NHO Miyagi National Hospital (100 Kassenhara, Takase, Yamamoto, Watari, Miyagi 989-2202, Japan)