2002
DOI: 10.1086/338718
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Pediatric Acute Hemorrhagic Leukoencephalitis: Report of a Surviving Patient and Review

Abstract: Acute hemorrhagic leukoencephalitis (AHLE) is a rare, fulminant CNS demyelinating condition usually diagnosed at autopsy. We report the clinical, laboratory, radiographic, and pathologic features of the first nonfatal case of pediatric AHLE confirmed by brain biopsy. Pathologic diagnosis of this condition may be critical to exclude more-common processes and to expedite the decision to administer high-dose corticosteroid therapy, which is potentially lifesaving.

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Cited by 43 publications
(42 citation statements)
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“…ADEM accounts for the majority of cases of parainfectious inflammatory encephalopathies, and since Hurst first described AHLE, fewer than 100 cases have been reported in the medical literature [3,37,56].…”
Section: Acute Parainfectious Inflammatory Encephalopathies ■ Patholomentioning
confidence: 99%
“…ADEM accounts for the majority of cases of parainfectious inflammatory encephalopathies, and since Hurst first described AHLE, fewer than 100 cases have been reported in the medical literature [3,37,56].…”
Section: Acute Parainfectious Inflammatory Encephalopathies ■ Patholomentioning
confidence: 99%
“…It typically presents as a monophasic illness with headache, followed by rapid progression to confusion, somnolence and eventually coma and death, frequently preceded by a nonspecific viral illness [3]. The mortality reported in the literature is as high as 70% [4, 5, 6]. The aetiology of the disease is unknown.…”
Section: Introductionmentioning
confidence: 99%
“…Elle est caractérisée sur le plan neuropathologique par une vascularite nécrosante des [29]. Depuis la description initiale par Weston Hurst en 1941, moins d'une centaine de cas ont été publiés dans la littérature [30][31][32]. L'AHLE est généralement déclenchée par une infection des voies respiratoires basses.…”
Section: Formes Cliniques Particulières Leucoencéphalite Aiguë Hémorrunclassified
“…Le traitement repose sur la corticothérapie systémique fortes doses, éventuelle-ment en association avec les IgG polyvalentes (IgIV) ou les échanges plasmatiques (EP). Certaines observations soulignent l'intérêt d'un traitement précoce agressif associant corticoïdes (CT), immunosuppresseurs et éventuellement décompression chirurgicale [31,[34][35][36].…”
Section: Formes Cliniques Particulières Leucoencéphalite Aiguë Hémorrunclassified