Chronic polioencephalitis with cerebral atrophy in infantile X-linked hypogammaglobulineaemia.

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Abstract
The development of a chronic polioencephalitis is reported in a patient with infantile X-linked hypogammaglobulinemia (IXH, Bruton type agammaglobulinemia). 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 yr. The neuropathological lesion, which revealed severe cerebral atrophy, is described.