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Journal of Pediatric Neurology 2021; 19(04): 270-275
DOI: 10.1055/s-0040-1716824
Case Report

Fulminant Acute Disseminated Encephalomyelitis: A Remarkable Outcome with Cyclophosphamide

Hadas Meirson
1   Pediatric Neurology Institute, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
,
Shelly I. Shiran
2   Pediatric Radiology Unit, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
,
Michal Raz
3   Department of Pathology, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
,
Jonathan Roth
4   Department of Pediatric Neurosurgery, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
,
Aviva Fattal-Valevski
1   Pediatric Neurology Institute, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
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Abstract

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system which occurs predominantly in the pediatric population. Acute treatment is high-dose intravenous glucocorticoids. Alternative treatment is usually intravenous immune globulin and/or plasma exchange. Fulminant ADEM is rare in children. Only a few cases of cyclophosphamide use in refractory ADEM have been reported. Here, we report a case of a 12-year-old girl with fulminant ADEM who was comatose and improved dramatically after cyclophosphamide administration. Cyclophosphamide treatment should be considered as a therapy in children with fulminant ADEM nonresponsive to standard therapies.

Keywords

acute disseminated encephalomyelitis - intravenous immune globulin - cyclophosphamide


Publication History

Received: 12 June 2020

Accepted: 09 August 2020

Article published online:
21 September 2020

© 2020. Thieme. All rights reserved.

Georg Thieme Verlag KG
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