Magnetic resonance imaging in a patient with X-linked agammaglobulinemia and chronic meningoencephalitis.
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 dilatation 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 (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.
Solé
D
D
Department of Pediatrics, Escola Paulista de Medicina, São Paulo, Brazil.
Radvani
J
J
Leser
P G
PG
Naspitz
C K
CK
D000361:Agammaglobulinemia / Q000150:complications* / Q000151:congenital / Q000235:genetics; D001921:Brain / Q000473:pathology; D002675:Child, Preschool; D002908:Chronic Disease; D008040:Genetic Linkage; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008590:Meningoencephalitis / Q000150:complications* / Q000175:diagnosis* / Q000209:etiology; D014057:Tomography, X-Ray Computed; D014960:X Chromosome
D016756:Immunoglobulins, Intravenous