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Movement disorders : official journal of the Movement Disorder Society · 2010
AbstractMultiple system atrophy (MSA) is a Parkinson's Disease (PD)‐like α‐synucleinopathy clinically characterized by dysautonomia, parkinsonism, cerebellar ataxia, and pyramidal signs in any combination. We aimed to determine whether the clinical presentation of MSA as well as diagnostic and therapeutic strategies differ across Europe and Israel. In 19 European MSA Study Group centres all consecutive patients with a clinical diagnosis of MSA were recruited from 2001 to 2005. A standardized minimal data set was obtained from all patients. Four‐hundred thirty‐seven MSA patients from 19 centres in 10 countries were included. Mean age at onset was 57.8 years; mean disease duration at inclusion was 5.8 years. According to the consensus criteria 68% were classified as parkinsonian type (MSA‐P) and 32% as cerebellar type (MSA‐C) (probable MSA: 72%, possible MSA: 28%). Symptomatic dysautonomia was present in almost all patients, and urinary dysfunction (83%) more common than symptomatic orthostatic hypotension (75%). Cerebellar ataxia was present in 64%, and parkinsonism in 87%, of all cases. No significant differences in the clinical presentation were observed between the participating countries. In contrast, diagnostic work up and therapeutic strategies were heterogeneous. Less than a third of patients with documented orthostatic hypotension or neurogenic bladder disturbance were receiving treatment. This largest clinical series of MSA patients reported so far shows that the disease presents uniformly across Europe. The observed differences in diagnostic and therapeutic management including lack of therapy for dysautonomia emphasize the need for future guidelines in these areas. © 2010 Movement Disorder Society
Movement disorders : official journal of the Movement Disorder Society · 2002
AbstractWe analyzed parkinsonian features in multiple system atrophy (MSA) compared with age‐ and disease duration‐matched Parkinson's disease (PD) patients, and assessed the applicability of the Unified Parkinson's Disease Rating Scale (UPDRS) ‐III motor scale as a means of rating their severity. Cross‐sectional analysis of parkinsonism was done using UPDRS‐III, International Cerebellar Atatia Rating Scale, and disability scales (Hoehn and Yahr [H&A], Schwab and England, Katz and Lawton) in 50 unselected MSA patients and in 50 matched PD patients. At symptom onset, falls occurred 10 times more frequently in MSA, whereas limb tremor was 10 times more common in PD. At first visit (10.2 months), hemiparkinsonism and pill‐rolling rest tremor were less common in MSA. Hypomimia, atypical rest, postural or action tremor, as well as postural instability were more frequent in MSA. At study examination (62.4 months), parkinsonian signs in MSA patients were more frequently symmetrical and associated with axial rigidity, antecollis and postural instability. A levodopa response of >50% was seen in <10% of MSA patients. Modified H&Y stages (3.2 ± 1.3 vs. 2.2 ± 0.78) and UPDRS‐III scores (48.14 ± 19.5 vs. 31.74 ± 12.9) were significantly (P = 0.0001) higher in MSA. The internal consistency of the UPDRS‐III was fair in MSA patients (Cronbach's α >0.90), and correlated well with marked dependency on the Schwab and England and Katz and Lawton scales. Factor structure analysis of UPDRS‐III in MSA showed five clinically distinct subscores accounting for 74% of the variance, differing from PD by the dependency of the face–speech and limb bradykinesia items and independence of the postural–action tremor from the rest tremor items. There was a significant correlation (R2 = 0.70, P = 0.001) between ICARS ataxia and UPDRS‐III scores in MSA patients. Results confirm a distinct profile of parkinsonism in MSA and greater severity and disability compared with PD. It also indicates that the UPDRS‐III provides a useful severity measure of parkinsonism in MSA, albeit contaminated by additional cerebellar dysfunction. © 2002 Movement Disorder Society
Sleep medicine · 2005
Source PubMed · Recherche par auteur (homonymes possibles, vérifier l'affiliation).
Movement disorders : official journal of the Movement Disorder Society · 2005 · Case Reports
Krim E, Vital A, Macia F, Yekhlef F, et al.
Movement disorders : official journal of the Movement Disorder Society · 2002 · Journal Article
Tison F, Yekhlef F, Chrysostome V, Balestre E, et al.
Movement disorders : official journal of the Movement Disorder Society · 2010 · Journal Article
Köllensperger M, Geser F, Ndayisaba JP, Boesch S, et al.
Sleep medicine · 2005 · Journal Article
Ghorayeb I, Yekhlef F, Bioulac B, Tison F