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Table 3 Classic vs. Mild Forms of A-T

From: Ataxia telangiectasia: a review

 

Classic Form

Mild Form

Neurological

Manifestations

Neurological deficits are typically observed during the toddler years resulting in wheelchair dependency around the age of 10.

Individuals have more mild neurological deficits in childhood with slower age-related neurodegeneration. The predominant neurological symptoms or symptoms to present first may be myoclonus, dystonia, choreoathetosis or tremor with ataxia appearing later [175–177]. Oculomotor apraxia may also appear later or not at all [95].

Immunodeficiencies

Roughly two-thirds of people with classic A-T suffer from some type of immunodeficiency and/or lymphopenia.

Immunodeficiencies do occur, but are less common.

Pulmonary Disease

Relatively common.

Less common.

Cancer

Although malignancies in these individuals tend to occur at a younger age and are often lymphoid in nature, cancers in older individuals do occur and include both hematopoietic and non-hematopoietic malignancies.

Malignancies tend to appear later in life and include a higher proportion of non-hematopoietic cancers. The diagnosis of cancer can precede the diagnosis of A-T.