ataxia-telangiectasia

noun

atax·​ia-tel·​an·​gi·​ec·​ta·​sia ə-ˈtak-sē-ə-ˌte-ˌlan-jē-ˌek-ˈtā-zh(ē-)ə 

-tē-,

-tə-,

(ˌ)ā-

: an inherited systemic disorder marked especially by progressive pathological changes in the nervous system resulting in loss of motor coordination and by increased susceptibility to cancer especially of lymphoid tissue

Word History

First Known Use

1957, in the meaning defined above

Medical Definition

ataxia-telangiectasia

noun

: an inherited, systemic disorder that is marked especially by progressive pathological changes in the nervous system resulting in loss of motor coordination, telangiectasias especially on the eyes, ears, and cheeks, and immunodeficiency leading to increased susceptibility to cancer especially of lymphoid tissue

Many children with ataxia-telangiectasia die from respiratory failure or cancer by their teens or early 20s.

—Bill Minutaglio, The Dallas Morning News

Defects in such a multifunctional protein might explain many of the clinical manifestations seen in patients with ataxia–telangiectasia.

—Michael Kastan, The New England Journal of Medicine

Note: Ataxia-telangiectasia is inherited as a recessive autosomal trait requiring that both parents pass on a copy of the defective gene on a chromosome other than a sex chromosome.