Magnetic resonance imaging in a patient with X-linked agammaglobulinemia and chronic meningoencephalitis

Magnetic resonance imaging in a patient with X-linked agammaglobulinemia and chronic meningoencephalitis

Author Sole, D. Google Scholar
Radvani, J. Google Scholar
Leser, P. G. Google Scholar
Naspitz, C. K. Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Abstract The case of a boy with congenital agammaglobulinemia is reported. In spite of regular immunoglobulin replacement therapy (fresh plasma transfusion from family donors - 20 ml/kg/month), he developed chronic meningoencephalitis (ME). Besides characteristic clinical signs of ME, he also presented at cerebrospinal fluid analysis pleocytosis with lymphocyte predominance and class II cytomorphology, and delta and theta waves in the EEG. Computerized tomography showed dilation of the ventricles and marked cortical fissures (sulci). Magnetic resonance imaging showed a disease affecting white and gray matter. After diagnosis of ME, replacement therapy with Sandoglobulin(R) (700 mg/kg every 2 weeks) was started. His condition gradually worsened, and coma and death occurred after a follow-up of 18 months. The etiological agent could not be identified.
Keywords CONGENITAL AGAMMAGLOBULINEMIA
CONGENITAL IMMUNODEFICIENCY
HUMORAL IMMUNODEFICIENCY
CHRONIC MENINGOENCEPHALITIS
MAGNETIC RESONANCE
Language English
Date 1992-01-01
Published in Journal Of Investigational Allergology & Clinical Immunology. Barcelona: J R Prous Sa, v. 2, n. 1, p. 43-45, 1992.
ISSN 1018-9068 (Sherpa/Romeo, impact factor)
Publisher J R Prous Sa
Extent 43-45
Access rights Closed access
Type Article
Web of Science ID WOS:A1992HM07000008
URI http://repositorio.unifesp.br/11600/44439

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