Neuropediatrics 2013; 44(03): 155-158
DOI: 10.1055/s-0032-1327772
Short Communication
Georg Thieme Verlag KG Stuttgart · New York

Human Herpesvirus 6–Associated Encephalopathy in a Child with Dravet Syndrome

Ayako Hiraiwa-Sofue
1   Department of Pediatrics, Hiraiwa Hospital, Nagoya, Japan
2   Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
,
Yoshinori Ito
2   Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
,
Rieko Ohta
2   Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
,
Hiroshi Kimura
3   Department of Virology, Nagoya University Graduate School of Medicine, Nagoya, Japan
,
Akihisa Okumura
4   Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
› Author Affiliations
Further Information

Publication History

18 November 2011

15 August 2012

Publication Date:
03 October 2012 (online)

Abstract

Dravet syndrome presents with generalized and unilateral clonic or clonic–tonic seizures that occur during the first year of life, followed by severe epilepsy. Prolonged seizures are often provoked by fever and usually followed by recovery of the previous condition. We describe the case of a 13-month-old girl with Dravet syndrome who experienced severe neurological sequelae as a result of human herpesvirus 6–associated encephalopathy. Biphasic clinical course was observed, with fever and prolonged seizures at onset and late seizures refractory against antiepileptic agents. Serum concentrations of proinflammatory cytokines and matrix metalloproteinase-9, which have been associated with development of acute encephalopathy, were not markedly increased in this patient, suggesting that these molecules were not the main causes of neuronal damage in this patient. Instead, seizure susceptibility due to SCN1A mutation may have contributed to acute encephalopathy in our patient.

 
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