Chronic polioencephalitis with cerebral atrophy in infantile X-linked hypogammaglobulineaemia.
The development of a chronic polioencephalitis is reported in a patient with infantile X-linked hypogammaglobulinaemia (IXH Bruton type agammaglobulinaemia). In early childhood, the patient had multiple episodes of purulent inflammation involving the meninges and respiratory tract. He was given continuous administration of gammaglobulin and intermittent treatment with antibiotics, and survived for 21 years. The neuropathological lesion, which revealed severe cerebral atrophy, is described.
D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications / Q000235:genetics* / Q000473:pathology; D001284:Atrophy; D001921:Brain / Q000473:pathology*; D002908:Chronic Disease; D004660:Encephalitis / Q000150:complications* / Q000473:pathology; D005260:Female; D006801:Humans; D008221:Lymphoid Tissue / Q000473:pathology*; D008297:Male; D014960:X Chromosome