Chronic encephalitis in X-linked agammaglobulinaemia.
Clinicopathological and immunological findings are reported in a boy with X linked agammaglobulinaemia (XLA), who after multiple episodes of purulent infections in various organs developed encephalitis associated with excemata and non suppurative arthritis. Inspite of gammaglobulin administration he developed progressive spastic tetraparesis, convulsions, mental deterioration and blindness and died in a decerebrate state at the age of 8 years. Necropsy revealed a general diminuition of the lymphoid tissue. The atrophic brain showed multiple cystic destructions and chronic, but still active polioencephalitis with prominent inflammatory involvement of basal ganglia and brain stem. Symmetric Wernicke-like lesions were seen in inferior corpora quadrigemina. The aetiology of this particular type of chronic progressive encephalitis in XLA is unknown but some kind of viral infection is to be considered.
D000361:Agammaglobulinemia / Q000235:genetics* / Q000473:pathology; D001284:Atrophy; D001921:Brain / Q000473:pathology; D002648:Child; D002908:Chronic Disease; D004660:Encephalitis / Q000235:genetics* / Q000473:pathology; D005260:Female; D008040:Genetic Linkage; D006801:Humans; D007111:Immunity, Cellular; D008297:Male; D014960:X Chromosome