RSS-Feed abonnieren
DOI: 10.1055/s-2002-36736
Georg Thieme Verlag Stuttgart · New York
Hypercytokinemia in Hemiconvulsions-Hemiplegia Syndrome Associated with Dual Infection with Varicella Zoster and Epstein-Barr Viruses
Publikationsverlauf
Received: 7 January 2002
Accepted after Revision: 27 May 2002
Publikationsdatum:
21. Januar 2003 (online)
Abstract
Hemiconvulsions-hemiplegia (HH) syndrome is an acquired condition in which hemiplegia develops after a preceding febrile unilateral status epilepticus in a previously healthy child. Although viral encephalitis or vascular diseases may be the underlying etiology, the pathogenesis remains unknown in the majority of cases. We measured both plasma and cerebrospinal fluid cytokine levels in a girl with HH syndrome, and found elevated plasma concentrations of soluble interleukin-2 receptor and tumor necrosis factor-α, and a slightly increased plasma level of interleukin-6. Furthermore, she had a high serum concentration of soluble E-selectin, which is a marker of inflammatory endothelial activation. These findings suggest that proinflammatory cytokine-induced cerebrovascular endothelial injury could play a role in the pathogenesis of HH syndrome.
Key words
Hemiconvulsions-Hemiplegia Syndrome - Cytokine - E-Selectin - Unilateral Hyperperfusion
References
- 1 Fassbender K, Bertsch T, Mielke O, Muhlhauser F, Hennerici M. Adhesion molecules in cerebrovascular diseases. Evidence for an inflammatory endothelial activation in cerebral large- and small-vessel disease. Stroke. 1999; 30 1647-1650
- 2 Forbes K PN, Collie D A, Parker A. CNS involvement of virus-associated hemophagocytic syndrome. AJNR. 2000; 21 1248-1250
- 3 Gastaut H, Poirier F, Payan H. et al . H. H. E. syndrome - hemiconvulsions, hemiplegia, epilepsy. Epilepsia. 1959 - 60; 1 418-447
-
4 Green M.
The nervous system. Green M Pediatric Diagnosis: Interpretation of Symptoms and Signs in Infants, Children, and Adolescents. 5th ed. Philadelphia; W. B. Saunders Company 1992: 126 - 5 Henter J I, Elinder G, Ost A. Diagnostic guidelines for hemophagocytic lymphohistiocytosis: The FHL Study Group of the Histiocyte Society. Semin Oncol. 1991; 18 29-33
- 6 Howells D W, Strobel S, Smith I, Levinsky R J, Hyland K. Central nervous system involvement in the erythrophagocytic disorders of infancy: the role of cerebrospinal fluid neopterins in their differential diagnosis and clinical management. Pediatr Res. 1990; 28 116-119
- 7 Ichiyama T, Nishikawa M, Yoshitomi T, Hayashi T, Furukawa S. Tumor necrosis factor-α, interleukin-1β, and interleukin-6 in cerebrospinal fluid from children with prolonged febrile seizures. Comparison with acute encephalitis/encephalopathy. Neurology. 1998; 50 407-411
- 8 Komp D M, McNamara J, Buckley P. Elevated soluble interleukin-2 receptor in childhood hemophagocytic syndromes. Blood. 1989; 73 2128
- 9 Launes J, Hokkanen L, Nikkinen P, Liewendahl K, Salonen O, Siren J, Iivanainen M. Hyperfixation of 99 mTc-HMPAO and hypofixation of 123I-iomazenil in acute herpes encephalitis. Neuroreport. 1995; 6 1203-1206
- 10 Munakata M, Kato R, Yokoyama H. et al . Combined therapy with hypothermia and anticytokine agents in influenza A encephalopathy. Brain Dev. 2000; 22 373-377
- 11 Roger J, Dravet C, Bureau M. Unilateral seizures: hemiconvulsions-hemiplegia syndrome (HH) and hemiconvulsions-hemiplegia-epilepsy syndrome (HHE). Electroencephalogr Clin Neurophysiol. 1982; 35 (Suppl) 211-221
M. D. Hiroyuki Wakamoto
Department of Pediatrics · Ehime Prefecture Niihama Hospital
3-1-1 Hongo
Niihama City, Ehime 792-0042
Japan
eMail: wakamot@mail2.netwave.or.jp