Neuropediatrics 2014; 45(04): 256-260
DOI: 10.1055/s-0033-1363300
Short Communication
Georg Thieme Verlag KG Stuttgart · New York

Fulminant Encephalopathy with Marked Brain Edema and Bilateral Thalamic Lesions

Naoki Ando
1   Department of Pediatrics, Nagoya City University Graduate School of Medicine, Nagoya, Japan
,
Akihisa Okumura
2   Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
,
Satoru Kobayashi
1   Department of Pediatrics, Nagoya City University Graduate School of Medicine, Nagoya, Japan
,
Yutaka Negishi
1   Department of Pediatrics, Nagoya City University Graduate School of Medicine, Nagoya, Japan
,
Ayako Hattori
1   Department of Pediatrics, Nagoya City University Graduate School of Medicine, Nagoya, Japan
,
Tohru Okanishi
1   Department of Pediatrics, Nagoya City University Graduate School of Medicine, Nagoya, Japan
,
Shinpei Abe
2   Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
,
Mitsuru Ikeno
2   Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
,
Ayuko Igarashi
2   Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
,
Shinji Saitoh
1   Department of Pediatrics, Nagoya City University Graduate School of Medicine, Nagoya, Japan
,
Toshiaki Shimizu
2   Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan
› Author Affiliations
Further Information

Publication History

29 June 2013

09 October 2013

Publication Date:
13 December 2013 (online)

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Abstract

We encountered two children with acute encephalopathy associated with unique clinical manifestations. Both the patients had status epilepticus at onset and neuroimaging studies revealed marked brain edema and bilateral thalamic lesions. Although they were treated with steroids and immunoglobulin, their outcomes were very poor. A thermolabile variant of carnitine palmitoyltransferase II and an elevated interleukin-6 level in cerebrospinal fluid were observed in one patient each. The constellation of clinical and neuroimaging findings in our patients is apparently not consistent with any established subtype of acute encephalopathy/encephalitis.