Prediction of survival with long-term disease progression in most common spinocerebellar ataxia
Movement Disorders Jun 26, 2019
Diallo A, et al. - In this investigation, researchers sought to quantify the effect of disease progression measured by the Scale for the Assessment and Rating of Ataxia on survival, and to determine profiles of disease progression and survival. From the EUROSCA prospective cohort study, 462 spinocerebellar ataxia patients were selected. They analyzed patients suffering from spinocerebellar ataxia type 1, spinocerebellar ataxia type 2, spinocerebellar ataxia type 3, and spinocerebellar ataxia type 6, and who had at least two measurements of Scale for the Assessment and Rating of Ataxia score. To analyze disease progression and survival, joint model was used. For patients with spinocerebellar ataxia type 1, 3 subgroups of disease progression and survival were identified: “severe,” “intermediate,” and “moderate.” Compared with patients in the other groups, those in the severe group were more severely affected at baseline with higher Scale for the Assessment and Rating of Ataxia scores and frequency of nonataxia signs. An association was found between rapid ataxia progression and poor survival of the most common spinocerebellar ataxia. These current outcomes have implications for designing future spinocerebellar ataxia interventional studies.
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