Estimation of the age at onset in spinocerebellar ataxia type 2 Cuban patients by survival analysis.

Previous studies have investigated the close association that exists between CAG repeat number and the age at onset in SCA2 = spinocerebellar ataxia type 2. These studies have focused on affected individuals. To further characterize this association and estimate the risk of a carrier developing SCA2 at a particular age as a function of a specific CAG repeat size, we have analyzed a large group of 924 individuals, including 394 presymptomatic and 530 affected individuals with a CAG repeat length of 32-79 units.

Antigliadin antibodies in Cuban patients with spinocerebellar ataxia type 2.

Objective: To evaluate the significance of antigliadin antibodies (AGA) levels for spinocerebellar ataxia type 2.

Methods: We determined AGA levels in 64 patients with spinocerebellar ataxia type 2 and in 65 healthy matched controls. The clinical assessment was carried out using the International Cooperative Ataxia Rating Scale and CAG repeat number was assessed by PCR.