Early symptoms in spinocerebellar ataxia type 1, 2, 3, and 6
L Schöls, C Globas, T Schmitz-Hübsch, D Timmann, S Szymanski, A Dürr, C Dupont, B Melegh, C Mariotti, S Boesch, B van de Warrenburg, A Filla, M Rakowicz, T Klockgether, S Tezenas du Montcel, P Bauer · Aktuelle Neurologie · 2008 · Heft 09
Bibliografische Angaben
Zusammenfassung
Onset of genetically determined neurodegenerative diseases is difficult to specify due to the insidious and slowly progressive nature of these disorders. This is especially true for spinocerebellar ataxia (SCA) because of varying affection of many parts of the nervous system and huge variability of symptoms. We investigated early symptoms in 287 patients with SCA1, SCA2, SCA3 or SCA6 and calculated the influence of CAG repeat length on age of onset depending on (i) the definition of disease onset, (ii) people defining onset and (iii) duration of symptoms. Gait difficulty was the initial symptom in about two thir…
