OBJECTIVE: To investigate the related factors of international cooperative ataxia rating scale (ICARS) and scale for the assessment and rating of ataxia scores (SARA) in patients with spinocerebellar ataxia type 3/Machado-Joseph disease. METHODS: A total of 126 SCA3/MJD patients were assessed by ICARS and SARA. The relation between ICARS or SARA scores and age of onset, disease duration and CAG repeat size was analyzed. RESULTS: Either the total ICARS or the total SARA score was positively related with the disease duration(r=0.586,P<0.05;r=0.643,P<0.05). Simple linear regression equations were: Y(1)(total ICARS score)=13.072+2.388 X(2)(disease duration)(F=68.874,P<0.05); Y(2)(total SARA score)=4.403+ 0.961 X(2)(disease duration)(F=87.254, P<0.05). Either age adjusted the total ICARS score or age adjusted the total SARA score was positively related with CAG repeat size(r=0.328, P<0.05; r=0.335, P<0.05). Both the ICARS subscores and the SARA subscores were positively related with the disease duration(r=0.257-0.589, P<0.05; r=0.432-0.623, P<0.05). Both age adjusted ICARS subscores and age adjusted SARA subscores were positively related with CAG repeat size(r=0.263-0.403, P<0.05; r=0.189-0.366, P<0.05). Analysis of variance showed that the total ICARS score and the total SARA score increased with the disease stage. CONCLUSION: ICARS and SARA are both reliable and effective scales in assessing the severity of ataxia in patients with SCA3/MJD, and researchers can choose the most suitable scale according to specific requirement.
OBJECTIVE: To investigate the related factors of international cooperative ataxia rating scale (ICARS) and scale for the assessment and rating of ataxia scores (SARA) in patients with spinocerebellar ataxia type 3/Machado-Joseph disease. METHODS: A total of 126 SCA3/MJDpatients were assessed by ICARS and SARA. The relation between ICARS or SARA scores and age of onset, disease duration and CAG repeat size was analyzed. RESULTS: Either the total ICARS or the total SARA score was positively related with the disease duration(r=0.586,P<0.05;r=0.643,P<0.05). Simple linear regression equations were: Y(1)(total ICARS score)=13.072+2.388 X(2)(disease duration)(F=68.874,P<0.05); Y(2)(total SARA score)=4.403+ 0.961 X(2)(disease duration)(F=87.254, P<0.05). Either age adjusted the total ICARS score or age adjusted the total SARA score was positively related with CAG repeat size(r=0.328, P<0.05; r=0.335, P<0.05). Both the ICARS subscores and the SARA subscores were positively related with the disease duration(r=0.257-0.589, P<0.05; r=0.432-0.623, P<0.05). Both age adjusted ICARS subscores and age adjusted SARA subscores were positively related with CAG repeat size(r=0.263-0.403, P<0.05; r=0.189-0.366, P<0.05). Analysis of variance showed that the total ICARS score and the total SARA score increased with the disease stage. CONCLUSION: ICARS and SARA are both reliable and effective scales in assessing the severity of ataxia in patients with SCA3/MJD, and researchers can choose the most suitable scale according to specific requirement.
Authors: Mercedes Prudencio; Hector Garcia-Moreno; Karen R Jansen-West; Rana Hanna Al-Shaikh; Tania F Gendron; Michael G Heckman; Matthew R Spiegel; Yari Carlomagno; Lillian M Daughrity; Yuping Song; Judith A Dunmore; Natalie Byron; Björn Oskarsson; Katharine A Nicholson; Nathan P Staff; Sorina Gorcenco; Andreas Puschmann; João Lemos; Cristina Januário; Mark S LeDoux; Joseph H Friedman; James Polke; Robin Labrum; Vikram Shakkottai; Hayley S McLoughlin; Henry L Paulson; Takuya Konno; Osamu Onodera; Takeshi Ikeuchi; Mari Tada; Akiyoshi Kakita; John D Fryer; Christin Karremo; Inês Gomes; John N Caviness; Mark R Pittelkow; Jan Aasly; Ronald F Pfeiffer; Venka Veerappan; Eric R Eggenberger; William D Freeman; Josephine F Huang; Ryan J Uitti; Klaas J Wierenga; Iris V Marin Collazo; Philip W Tipton; Jay A van Gerpen; Marka van Blitterswijk; Guojun Bu; Zbigniew K Wszolek; Paola Giunti; Leonard Petrucelli Journal: Sci Transl Med Date: 2020-10-21 Impact factor: 17.956