title: Successful treatment for sympathetic storms in a patient with neuro-Behçet's disease.
abstract: Sympathetic storms (SyS) are characterized by hyperactivity of autonomic functions, resulting in episodes of hyperthermia, hypertension, tachycardia, and hyperhidrosis. We show here a patient with neuro-Behçet's disease (NBD) complicated by SyS. Although SyS is well known to occur with brain tumors, trauma, and hydrocephalus, this is the first report to show that SyS is a manifestation of central nervous system involvement in a patient with NBD. High concentrations of norepinephrine (NE) and IL-8 in cerebrospinal fluid reflected the activity of SyS. The patient's symptoms showed almost complete improvement after treatment with corticosteroids and intravenous cyclophosphamide. Also, the concentrations of NE and IL-8 were decreased to normal levels. An awareness of the potential for SyS and adequate immunosuppressant therapy are of importance when dealing with patients with NBD.
authors.affiliation: Institute of Rheumatology, Tokyo Women's Medical University, 10-22 Kawada-cho, Shinjuku-Ku, Tokyo 162-0054, Japan.
authors.forename: Takahisa
authors.initials: T
authors.lastname: Gono
authors.forename: Megumi
authors.initials: M
authors.lastname: Murata
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Kawaguchi
authors.forename: Daisuke
authors.initials: D
authors.lastname: Wakasugi
authors.forename: Makoto
authors.initials: M
authors.lastname: Soejima
authors.forename: Hisashi
authors.initials: H
authors.lastname: Yamanaka
authors.forename: Masako
authors.initials: M
authors.lastname: Hara
mesh_terms: D000284:Administration, Oral; D000328:Adult; D001342:Autonomic Nervous System Diseases / Q000134:cerebrospinal fluid / Q000188:drug therapy* / Q000209:etiology; D001528:Behcet Syndrome / Q000134:cerebrospinal fluid / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007275:Injections, Intravenous; D016209:Interleukin-8 / Q000134:cerebrospinal fluid; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use*; D009638:Norepinephrine / Q000134:cerebrospinal fluid; D012074:Remission Induction
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D016209:Interleukin-8; D003520:Cyclophosphamide; D009638:Norepinephrine; D008775:Methylprednisolone