Ataxia-telangiectasia is the association of severe combined immunodeficiency (affecting mainly the humoral immune response) with progressive cerebellar ataxia. It is characterized by neurological signs, telangiectasias, increased susceptibility to infections and a higher risk of cancer. The disease is ataxia telangiectasia (MONDO_0008840). Also known as: Louis Bar syndrome, Louis-Bar syndrome, ataxia - telangiectasia, ataxia telangiectasia syndrome, boder-Sedgwick syndrome.