title: Fulminant encephalopathy with marked brain edema and bilateral thalamic lesions.
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.
authors: Department of Pediatrics, Nagoya City University Graduate School of Medicine, Nagoya, Japan.
authors: Naoki
authors: N
authors: Ando
authors: Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan.
authors: Akihisa
authors: A
authors: Okumura
authors: Department of Pediatrics, Nagoya City University Graduate School of Medicine, Nagoya, Japan.
authors: Satoru
authors: S
authors: Kobayashi
authors: Department of Pediatrics, Nagoya City University Graduate School of Medicine, Nagoya, Japan.
authors: Yutaka
authors: Y
authors: Negishi
authors: Department of Pediatrics, Nagoya City University Graduate School of Medicine, Nagoya, Japan.
authors: Ayako
authors: A
authors: Hattori
authors: Department of Pediatrics, Nagoya City University Graduate School of Medicine, Nagoya, Japan.
authors: Tohru
authors: T
authors: Okanishi
authors: Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan.
authors: Shinpei
authors: S
authors: Abe
authors: Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan.
authors: Mitsuru
authors: M
authors: Ikeno
authors: Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan.
authors: Ayuko
authors: A
authors: Igarashi
authors: Department of Pediatrics, Nagoya City University Graduate School of Medicine, Nagoya, Japan.
authors: Shinji
authors: S
authors: Saitoh
authors: Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan.
authors: Toshiaki
authors: T
authors: Shimizu
mesh_terms: D000208:Acute Disease; D001929:Brain Edema / Q000175:diagnosis*; D002334:Carnitine O-Palmitoyltransferase / Q000235:genetics; D002648:Child; D004660:Encephalitis / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D015850:Interleukin-6 / Q000134:cerebrospinal fluid; D008297:Male; D013787:Thalamic Nuclei / Q000473:pathology*
chemical_list: D015850:Interleukin-6; D002334:Carnitine O-Palmitoyltransferase