Multisystem Atrophy and Progressive Supranuclear Palsy Assessment

Prior to the start of the trial, items were selected through expert consensus as part of a broad clinical description of both MSA and PSP. The dimensions included (i) functional disability (activities of daily living), (ii) mental function (cognition, mood & behavior); (iii) extra-pyramidal motor disability (rigidity, bradykinesia), (iv) tremor, (v) oculomotor function, (vi) cerebellar signs, (vii) pyramidal signs, (viii) dysautonomia, (ix) bulbar/pseudobulbar symptoms, (x) myoclonus, and (xi) dystonia. Items were selected from the following scales available at that time, the UPDRS (all items from Mental, ADL and Motor examination sections) [13] , the PSP-RS (six items from the mental section) [17] , three items from the International Cooperative Ataxia Rating Scale (ICARS) [21] (link), the global ataxia score of the Expanded Disability Status Scale (EDSS ) [22] (link), and four items evaluating orthostatic signs and three for urinary signs from the Autonomic Symptom Profile [23] (link) adapted to interview record instead of self-rating. Additional items were included to assess oculomotor signs, dystonia, myoclonus, pyramidal signs, sitting down and strength of cough.
A preliminary version of 109 items was evaluated in a pilot study to check each item and category wording. Redundant or inappropriate items were eliminated to obtain the first version comprising 85 items to be tested. Severity levels of items ranged from 0 (“normal”) to a maximum of 6 (very severe), with a majority of items (65) scored on a 5-point scale (0–4) (Supporting information S1). Four sections were interview based with patient and/or caregiver (Mental, Activities of Daily Living-ADL, orthostatic and urinary signs), eleven were assessed through examination. Time to complete the scale was 30–45 minutes depending on clinical state of patient. Throughout the study, the scale was completed in all centres using an English version.