Progressive neurodegenerative syndrome in a patient with X-linked agammaglobulinemia receiving intravenous immunoglobulin therapy.
Cogn Behav Neurol
; 27(3): 155-9, 2014 Sep.
Article
em En
| MEDLINE
| ID: mdl-25237746
ABSTRACT
A progressive encephalopathy of unknown etiology has been described in patients with primary immunodeficiency disorders. In this report, we characterize the clinical features of this progressive neurodegenerative dementing disorder in a young man with Bruton agammaglobulinemia, through neuropsychological tests and a video sequence. The clinical course of the encephalopathy seems rather uniform Cognition, especially frontal lobe function, is affected in the early stages, and some patients develop movement disorders. The syndrome causes severe cognitive and physical disability, and can eventually be fatal. The autoimmunity results from dysregulated immune responses, but the underlying mechanism has not yet been fully explained.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Encéfalo
/
Imunoglobulinas Intravenosas
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Transtornos Cognitivos
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Agamaglobulinemia
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Doenças Genéticas Ligadas ao Cromossomo X
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Transtornos dos Movimentos
Tipo de estudo:
Diagnostic_studies
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Etiology_studies
Limite:
Adult
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Child, preschool
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Humans
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Male
Idioma:
En
Ano de publicação:
2014
Tipo de documento:
Article