Prediction of Survival With Long-Term Disease Progression in Most Common Spinocerebellar Ataxia
- Diallo, A.
- Jacobi, H.
- Cook, A.
- Giunti, P.
- Parkinson, M.H.
- Labrum, R.
- Durr, A.
- Brice, A.
- Charles, P.
- Marelli, C.
- Mariotti, C.
- Nanetti, L.
- Panzeri, M.
- Castaldo, A.
- Rakowicz, M.
- Rola, R.
- Sulek, A.
- Schmitz-Hubsch, T.
- Schols, L.
- Hengel, H.
- Baliko, L.
- Melegh, B.
- Filla, A.
- Antenora, A.
- Infante, J.
- Berciano, J.
- Warrenburg, B.P.C. van de
- Timmann, D.
- Boesch, S.
- Nachbauer, W.
- Pandolfo, M.
- Schulz, J.B.
- Bauer, P.
- Jun-Suk, K.
- Klockgether, T.
- Tezenas du Montcel, S.
DOIAbstract
Item does not contain fulltextBACKGROUND: Spinocerebellar ataxias are rare dominantly inherited neurodegenerative diseases that lead to severe disability and premature death. OBJECTIVE: To quantify the impact of disease progression measured by the Scale for the Assessment and Rating of Ataxia on survival, and to identify different profiles of disease progression and survival. METHODS: Four hundred sixty-two spinocerebellar ataxia patients from the EUROSCA prospective cohort study, 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, were analyzed. Outcomes wer...
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