Fulminant Encephalopathy with Marked Brain Edema and Bilateral Thalamic Lesions

• DOI: 10.1055/s-0033-1363300
• Publication Date: 2013-12-13T23:40:54Z
• Authors: Naoki Ando; Satoru Kobayashi; Yutaka Negishi; Ayako Hattori; Tohru Okanishi; Shinpei Abe; Mitsuru Ikeno; Ayuko Igarashi; Shinji Saitoh; Toshiaki Shimizu; Akihisa Okumura
• Keywords: Male; Carnitine O-Palmitoyltransferase; Interleukin-6; Infant; Brain Edema; 3. Good health; 03 medical and health sciences; 0302 clinical medicine; Thalamic Nuclei; Acute Disease; Encephalitis; Humans; Female; Child

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.