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.
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References
Akman-Demir G, Serdaroglu P, Tasçi B (1999) Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. The neuro-Behçet study group. Brain 122(Pt 11):2171–2182
Goh K, Conway E, DaRosso R, Muszynski C, Epstein F (1999) Sympathetic storms in a child with a midbrain glioma: a variant of diencephalic seizures. Pediatr Neurol 21:742–744
Elrod R, Peskind E, DiGiacomo L, Brodkin K, Veith R, Raskind M (1997) Effects of Alzheimer’s disease severity on cerebrospinal fluid norepinephrine concentration. Am J Psychiatry 154:25–30
DeQuattro V, Feng M (2002) The sympathetic nervous system: the muse of primary hypertension. J Hum Hypertens 16(Suppl 1):S64–S69
Weil-Malherbe H, Axelrod J, Tomchick R (1959) Blood–brain barrier for adrenaline. Science 129:1226–1227
al-Dalaan A, al-Sedairy S, al-Balaa S et al (1995) Enhanced interleukin 8 secretion in circulation of patients with Behçet’s disease. J Rheumatol 22:904–907
Gür-Toy G, Lenk N, Yalcin B, Aksaray S, Alli N (2005) Serum interleukin-8 as a serologic marker of activity in Behçet’s disease. Int J Dermatol 44:657–660
Kimura A, Sakurai T, Koumura A et al (2008) Longitudinal analysis of cytokines and chemokines in the cerebrospinal fluid of a patient with neuro-Sweet disease presenting with recurrent encephalomeningitis. Intern Med 47:135–141
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Gono, T., Murata, M., Kawaguchi, Y. et al. Successful treatment for sympathetic storms in a patient with neuro-Behçet’s disease. Clin Rheumatol 28, 357–359 (2009). https://doi.org/10.1007/s10067-008-1070-2
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DOI: https://doi.org/10.1007/s10067-008-1070-2