2006 ICD-9-CM Volume 1 Diagnosis Codes Home > Diseases Of The Nervous System And Sense Organs 320-389 > Hereditary And Degenerative Diseases Of The Central Nervous System 330-338 > Spinocerebellar Disease 334.* > 2006 ICD-9-CM Diagnosis 334.8 Other spinocerebellar diseases View the most recent version of ICD-9-CM 334.8 An autosomal recessive inherited disorder characterized by choreoathetosis beginning in childhood, progressive cerebellar ataxia, telangiectasis of conjunctiva and skin, dysarthria, B- and T-cell immunodeficiency, and sensitivity to ionizing radiation. Affected individuals are prone to recurrent sinobronchopulmonary infections, lymphoreticular neoplasms, and other malignancies. Serum ALPHA-FETOPROTEINS are usually elevated. (Menkes, Textbook of Child Neurology, 5th ed, p688) The gene for this disorder has been mapped to the long arm of chromosome 11 (11q22.3). 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. 334.8 is a specific code that can be used to specify a diagnosis 334.8 contains 15 index entries View the ICD-9-CM Volume 1 334.* hierarchy Alternate Terminology Ataxia-telangiectasia [Louis-Bar syndrome] Corticostriatal-spinal degeneration  Index entries containing 334.8: Ataxia, ataxy, ataxic 781.3 telangiectasia 334.8 Ataxia-telangiectasia 334.8 Azorean disease (of the nervous system) 334.8 Boder-Sedgwick syndrome (ataxia-telangiectasia) 334.8 Degeneration, degenerative corticostriatal-spinal 334.8 Disease, diseased - see also Syndrome Azorean (of the nervous system) 334.8 Machado-Joseph 334.8 spinocerebellar 334.9 specified NEC 334.8 Hemiatrophy 799.89 cerebellar 334.8 Louis-Bar syndrome (ataxia-telangiectasia) 334.8 Machado-Joseph disease 334.8 Syndrome - see also Disease ataxia-telangiectasia 334.8 Boder-Sedgwick (ataxia-telangiectasia) 334.8 Louis-Bar (ataxia-telangiectasia) 334.8 Telangiectasia, telangiectasis (verrucous) 448.9 ataxic (cerebellar) 334.8