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We aimed to develop and validate a novel rating scale for multiple system atrophy (Unified Multiple System Atrophy Rating Scale - UMSARS). The scale comprises the following components: Part I, historical, 12 items; Part II, motor examination, 14 items; Part III, autonomic examination; and Part IV, global disability scale. For validation purposes, 40 MSA patients were assessed in four centers by 4 raters per center (2 senior and 2 junior raters). The raters applied the UMSARS, as well as a range of other scales, including the Unified Parkinson's Disease Rating Scale (UPDRS) and the International Cooperative Ataxia Rating Scale (ICARS). Internal consistency was high for both UMSARS-1 (Crohnbach's alpha = 0.84) and UMSARS-II (Crohnbach's alpha = 0.90) sections. The interrater reliability of most of the UMSARS-I and -II items as well as of total UMSARS-I and -II subscores was substantial (k (w) = 0.6-0.8) to excellent (k (w) > 0.8). UMSARS-II correlated well with UPDRS-III and ICARS (rs > 0.8). Depending on the degree of the patient's disability, completion of the entire UMSARS took 30 to 45 minutes. Based on our findings, the UMSARS appears to be a multidimensional, reliable, and valid scale for semiquantitative clinical assessments of MSA patients.

Objectives : (1) To evaluate prevalence and risk factors of sleep disorders, sexual dysfunction and autonomic failure in idiopathic cervical dystonia (CD) and blepharospasm (BL). (2) To analyze the impact of botulinum toxin (BoNT) treatment on comorbidity in focal dystonia (FD). Background : The approach to movement disorder change, and non-motor comorbidity was shown to considerably impair quality of life in degenerative diseases. In contrast, there are no data on the prevalence of sleep disorders, sexual dysfunction or autonomic failure in non-degenerative movement disorders such as CD and BL, although depression and pain are well-known complications in FD. Methods : Standardized interviews, clinical examinations, autonomic testing, and self-rating forms in 221 patients with idiopathic FD (111 CD, 110 BL). Results : Impaired sleep quality was found in 45% of CD and in 46% of BL patients. Of 80 CD patients tested, 46% had autonomic failure. Sexual dysfunction became apparent in 35% of 82 patients. Depression was found in 25% of CD and 30% of BL patients. Conclusions : Beyond pain and depression, patients with FD frequently suffer from non-motor symptoms. Interestingly, results in CD and BL patients are similar, pointing to intrinsic mechanisms of sleep and sexual disturbances. In our series, BoNT treatment ameliorated non-motor symptoms only marginally, underlining the necessity of a multimodal treatment concept in FD.

Item does not contain fulltext OBJECTIVE: Differentiating Parkinson's disease (PD) from atypical parkinsonian disorders (APD) such as Multiple System Atrophy, parkinsonian type (MSA-p) or Progressive Supranuclear Palsy (PSP-RS) can be challenging. Early signs of postural Instability and gait disability (PIGD) are considered clues that may signal presence of APD. However, it remains unknown which PIGD test - or combination of tests - can best distinguish PD from APD. We evaluated the discriminative value of several widely-used PIGD tests, and aimed to develop a short PIGD evaluation that can discriminate parkinsonian disorders. METHODS: In this multicentre cohort study patients were recruited by 11 European MSA Study sites. Patients were diagnosed using standardized criteria. Postural instability and gait disability was evaluated using interviews and several clinical tests. RESULTS: Nineteen PD, 21 MSA-p and 25PSP-RS patients were recruited. PIGD was more common in APD compared to PD. There was no significant difference in axial symptoms between PSP-RS and MSA-p, except for self-reported falls (more frequent in PSP-RS patients). The test with the greatest discriminative power to distinguish APD from PD was the ability to perform tandem gait (AUC 0.83; 95% CI 71-94; p