Fulminant acute disseminated encephalomyelitis in children |
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Authors: | Yukako Yae Go Kawano Takaoki Yokochi Toru Imagi Yukihiro Akita Keizo Ohbu Toyojiro Matsuishi |
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Institution: | 1. Department of Pediatrics, St Mary’s Hospital, 422 Tsubukuhonmachi, Kurume, Fukuoka 830-8543, Japan;2. Research Centre for Children and Research Centre for Rett Syndrome, St Mary’s Hospital, 422 Tsubukuhonmachi, Kurume, Fukuoka 830-8543, Japan |
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Abstract: | Acute disseminated encephalomyelitis (ADEM) is a typically monophasic inflammatory demyelinating disease of the central nervous system with a favorable outcome. However, 2% of ADEM involves acute hemorrhagic leukoencephalitis (AHLE), which is a fulminant and hyperacute variant of ADEM with a poor outcome and high mortality. There are limited case reports of fulminant ADEM including AHLE in children. Herein, we report two pediatric cases of fulminant ADEM. Both cases had a rapid deterioration of consciousness, repetitive seizures, and brain edema on neuroimaging, in addition to atypical neuroradiological findings on magnetic resonance imaging (MRI), a reversible splenial lesion in case 1, and bilateral frontal and occipital cortical lesions in case 2. Both cases were treated with early high-dose methyl-prednisolone and immunoglobulin, while therapeutic hypothermia was also initiated in case 2 after the patient exhibited a decerebrate posture and irregular breathing pattern. Both cases had a favorable outcome. Further case reports on pediatric fulminant ADEM are required to clarify the various clinical types, and to examine the efficacy of various treatment modalities for fulminant ADEM and AHLE in children. |
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Keywords: | Acute disseminated encephalomyelitis (ADEM) Fulminant ADEM Acute hemorrhagic leukoencephalitis (AHLE) High-dose methyl-prednisolone (mPSL) Therapeutic hypothermia |
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