inherited disease characterized by onset in early childhood of progressive cerebellar ataxia, oculocutaneous telangiectasis, and severe sinopulmonary infections.
Rare hereditary disease characterized by extreme sensitivity to ionizing radiation or radiomimetic drugs because of a defect in DNA repair. AT heterozygosity is estimated to occur in more than 2% of the U.S. population; heterozygotes exhibit increased radiation sensitivity and are at increased risk for several types of cancer. The normal version of the gene that is defective in AT appears to activate the p53-dependent response to DNA damage.
Also applicable to/known as
Ataxia-telangiectasia [Louis-Bar syndrome]
Corticostriatal-spinal degeneration
ICD-9-CM Volume 2 Index Entries That Refer To 334.8
2009 ICD-9-CM Diagnosis Code 334.8
334.8
