Acute Disseminated Encephalomyelitis: A Gray Distinction

Abstract Background Acute disseminated encephalomyelitis (ADEM) is an immune-mediated, inflammatory acquired demyelinating syndrome predominantly affecting the white matter of the central nervous system. Methods We describe the case of a 3 year old boy, whose clinical presentation was suspicious for...

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Veröffentlicht in:Pediatric neurology 2017-03, Vol.68, p.64-67
Hauptverfasser: Abu-Libdeh, Amal, MBBS, Goodkin, Howard P., MD, PhD, Ramirez-Montealegre, Denia, MD, PhD, Brenton, J. Nicholas, MD
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container_end_page 67
container_issue
container_start_page 64
container_title Pediatric neurology
container_volume 68
creator Abu-Libdeh, Amal, MBBS
Goodkin, Howard P., MD, PhD
Ramirez-Montealegre, Denia, MD, PhD
Brenton, J. Nicholas, MD
description Abstract Background Acute disseminated encephalomyelitis (ADEM) is an immune-mediated, inflammatory acquired demyelinating syndrome predominantly affecting the white matter of the central nervous system. Methods We describe the case of a 3 year old boy, whose clinical presentation was suspicious for ADEM but initial imaging showed abnormalities confined to the deep gray matter (without evidence of white matter involvement) in the brain. His clinical course was fluctuating and repeat imaging obtained one week after presentation demonstrated interval development of characteristic white matter lesions. Results Treatment with adjunctive intravenous immunoglobulin (IVIg) and high-dose corticosteroids resulted in significant clinical improvement. Conclusions This case of ADEM is novel in demonstrating the initial findings of isolated deep gray matter involvement with delayed appearance of white matter abnormalities, suggesting that repeat imaging is indicated in cases that are clinically suspicious for ADEM but lack characteristic imaging findings
doi_str_mv 10.1016/j.pediatrneurol.2016.12.006
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Nicholas, MD</creatorcontrib><title>Acute Disseminated Encephalomyelitis: A Gray Distinction</title><title>Pediatric neurology</title><addtitle>Pediatr Neurol</addtitle><description>Abstract Background Acute disseminated encephalomyelitis (ADEM) is an immune-mediated, inflammatory acquired demyelinating syndrome predominantly affecting the white matter of the central nervous system. Methods We describe the case of a 3 year old boy, whose clinical presentation was suspicious for ADEM but initial imaging showed abnormalities confined to the deep gray matter (without evidence of white matter involvement) in the brain. His clinical course was fluctuating and repeat imaging obtained one week after presentation demonstrated interval development of characteristic white matter lesions. Results Treatment with adjunctive intravenous immunoglobulin (IVIg) and high-dose corticosteroids resulted in significant clinical improvement. 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His clinical course was fluctuating and repeat imaging obtained one week after presentation demonstrated interval development of characteristic white matter lesions. Results Treatment with adjunctive intravenous immunoglobulin (IVIg) and high-dose corticosteroids resulted in significant clinical improvement. Conclusions This case of ADEM is novel in demonstrating the initial findings of isolated deep gray matter involvement with delayed appearance of white matter abnormalities, suggesting that repeat imaging is indicated in cases that are clinically suspicious for ADEM but lack characteristic imaging findings</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>28173990</pmid><doi>10.1016/j.pediatrneurol.2016.12.006</doi><tpages>4</tpages></addata></record>
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subjects acute disseminated encephalomyelitis
ADEM
autoimmune
Brain - diagnostic imaging
Brain - drug effects
Cervical Cord - diagnostic imaging
Cervical Cord - drug effects
Child, Preschool
Diagnosis, Differential
Disease Progression
encephalitis
Encephalomyelitis, Acute Disseminated - diagnostic imaging
Encephalomyelitis, Acute Disseminated - physiopathology
Encephalomyelitis, Acute Disseminated - therapy
gray matter
Gray Matter - diagnostic imaging
Gray Matter - drug effects
Humans
Male
Neurology
Pediatrics
title Acute Disseminated Encephalomyelitis: A Gray Distinction
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