Rare brain biopsy findings in a first ADEM-like event of pediatric MS: histopathologic, neuroradiologic and clinical features.
J Neural Transm (Vienna)
; 118(9): 1311-7, 2011 Sep.
Article
em En
| MEDLINE
| ID: mdl-21547392
ABSTRACT
Pediatric MS tends to present more often with an acute onset and a polysymptomatic form of the disease, possibly with encephalopathy and large tumefactive lesions similar to those observed in some cases of acute disseminated encephalomyelitis (ADEM), which makes it more difficult to differentiate between an explosive and severe onset of MS vs. ADEM. An ADEM-like first demyelinating event can be the first attack of pediatric MS, but international consensus definitions require two or more non-ADEM demyelinating events for diagnosis of MS. In our patient KIDMUS MRI criteria for MS (Mikaeloff et al. J Pediatr 144246-252, 2004a; Mikaeloff et al. Brain 1271942-1947, 2004b) were negative at first attack, but Barkhof criteria for lesion dissemination in space in adults (Barkhof et al. 1202059-2069, 1997), Callen modified MS-criteria and Callen MS-ADEM criteria for children (Callen et al. Neurology 72961-967, 2009a; Callen et al. Neurology 72968-973, 2009b) were positive suggesting pediatric MS. As the clinical course was devastating with non-responsiveness upon high-dose immune modulatory therapy and due to the absence of an alternative diagnosis other than demyelinating disease brain biopsy was performed. Brain biopsy studies or autopsy case reports of fulminant pediatric MS patients are extremely rare. Histopathology revealed an inflammatory demyelinating CNS process with confluent demyelination, indicating the likelihood of a relapsing disease course compatible with an acute to subacute demyelinating inflammatory disease. This pattern was corresponding to the early active multiple sclerosis subtype I of Lucchinetti et al. (Ann Neurol 47(6)707-717, 2000).
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Base de dados:
MEDLINE
Assunto principal:
Biópsia
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Encéfalo
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Encefalomielite Aguda Disseminada
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Esclerose Múltipla
Tipo de estudo:
Diagnostic_studies
Limite:
Adolescent
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Female
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Humans
Idioma:
En
Ano de publicação:
2011
Tipo de documento:
Article