ATAXIA TELANGIECTASIA WITH CEREBELLAR TUMOR

Two patients with ataxia telangiectasia were studied. There is absence of γA immunoglobulin, poor delayed hypersensitivity skin reactions, and abnormal rejection of skin grafts. The susceptibility to sinopulmonary infections is probably related to the abnormal cellular and circulating antibody responses which these patients demonstrate. Experimental evidence indicates that the fundamental immunologic defect in this disease lies in thymus gland. The etiology of the neurologic abnormalities is unknown. The first instance of a cerebellar tumor to develop in this syndrome is reported.