Fulminant form of acute disseminated encephalomyelitis in a child treated with mild hypothermia.

Abstract

We describe the case of a 3-year-old boy diagnosed with the fulminant form of acute disseminated encephalomyelitis (ADEM). He developed general fatigue, fever, drowsiness and difficulty in walking. He had extensive multiple high-intensity lesions in the white matter of the cerebrum and cerebellum, which are typical findings of ADEM. He became comatose and developed decerebrate rigidity with severe brain edema despite high-dose methylprednisolone therapy, and then was subjected to mild hypothermia therapy, and given i.v. immunoglobulin. The patient recovered remarkably with the sequela of only mild action tremor. The patient was considered to have acute hemorrhagic leukoencephalitis (AHLE), an extremely severe form of ADEM, in terms of the rapidly deteriorating clinical course and neuroimaging features. It was speculated that AHLE and ADEM might be a continuous disease spectrum. It is considered that the severe brain edema associated with ADEM or AHLE is a suitable indication for mild hypothermia therapy.

• Authors: Kazushi Ichikawa, Hirotaka Motoi, Yoshitaka Oyama, Yoshihiro Watanabe, Saoko Takeshita
• Journal: Pediatrics international : official journal of the Japan Pediatric Society (Pediatr Int) Vol. 55 Issue 6 Pg. e149-51 (Dec 2013) ISSN: 1442-200X [Electronic] Australia
• PMID: 24330300 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2013 The Authors. Pediatrics International © 2013 Japan Pediatric Society.

Topics

• Child, Preschool
• Encephalomyelitis, Acute Disseminated (therapy)
• Humans
• Hypothermia, Induced (methods)
• Male
• Severity of Illness Index