One hundred-eighteen spinocerebellar ataxia type 2 patients from 35 distinct families personally observed between 1973 and 2016 were retrospectively reviewed. The time point of data collection was 1 January 2017. Thirty-one patients were confined to wheelchair. The median time to wheelchair was 21 years (95% CI, 17.5-24.6). Thirty-seven patients were deceased. The median time to death was 25 years (95% CI, 22.9-27.1). CAG repeat number and ataxia score at first visit influenced the time to wheelchair and death.
Predictors of survival in spinocerebellar ataxia type 2 population from Southern Italy / Antenora, A; Bruzzese, D; Lieto, M; Roca, A; Florio, Mt; Peluso, S; Saccà, F; De Michele, G; Santorelli, Fm; Filla, A.. - In: NEUROLOGICAL SCIENCES. - ISSN 1590-1874. - 39:11(2018), pp. 1857-1860. [10.1007/s10072-018-3504-1]
Predictors of survival in spinocerebellar ataxia type 2 population from Southern Italy.
Antenora A;Bruzzese D;Lieto M;Roca A;Peluso S;Saccà F;De Michele G;Filla A.
2018
Abstract
One hundred-eighteen spinocerebellar ataxia type 2 patients from 35 distinct families personally observed between 1973 and 2016 were retrospectively reviewed. The time point of data collection was 1 January 2017. Thirty-one patients were confined to wheelchair. The median time to wheelchair was 21 years (95% CI, 17.5-24.6). Thirty-seven patients were deceased. The median time to death was 25 years (95% CI, 22.9-27.1). CAG repeat number and ataxia score at first visit influenced the time to wheelchair and death.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
