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Published in: Neurological Sciences 10/2019

01-10-2019 | Original Article

Longitudinal study of a cohort of MSA-C patients in South Italy: survival and clinical features

Authors: Maria Lieto, Alessandro Roca, Dario Bruzzese, Antonella Antenora, Girolamo Alfieri, Francesco Saccà, Marta Bellofatto, Leonilda Bilo, Stefano Barbato, Giuseppe De Michele, Alessandro Filla

Published in: Neurological Sciences | Issue 10/2019

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Abstract

Sixty-six patients with possible or probable MSA (multiple system atrophy) cerebellar type, personally observed between 2006 and 2018 were retrospectively reviewed. The time point of data collection was January 1, 2019. Forty-nine patients lost independent walking after a median time of 5 years (95% C. I. 4–6). Thirty-two patients were confined to wheelchair after a median time of 7 years (95% C. I. 7–8). Twenty-seven patients were deceased after a median time of 9 years (95% C. I. 8–10). A later onset predicted an earlier loss of independent walking (HR 1.07; 95% C.I. 1.03–1.11; p = 0.001). Higher UMSARS score predicted shorter time to loss of independent walking (HR 1.04; 95% C.I. 1.02–1.06; p = 0.001) and to wheelchair (HR 1.03; 95% C.I. 1.01–1.06; p = 0.021). No predictor of time to death was found.
Literature
1.
go back to reference Schrag A, Ben-Shlomo Y, Quinn NP (1999) Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross- sectional study. Lancet 354:1771–1775CrossRefPubMed Schrag A, Ben-Shlomo Y, Quinn NP (1999) Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross- sectional study. Lancet 354:1771–1775CrossRefPubMed
3.
go back to reference Kollensperger M, Geser F, Ndayisaba JP, Boesch S, Seppi K, Ostergaard K, Dupont E, Cardozo A, Tolosa E, Abele M, Klockgether T, Yekhlef F, Tison F, Daniels C, Deuschl G, Coelho M, Sampaio C, Bozi M, Quinn N, Schrag A, Mathias CJ, Fowler C, Nilsson CF, Widner H, Schimke N, Oertel W, Del Sorbo F, Albanese A, Pellecchia MT, Barone P, Djaldetti R, Colosimo C, Meco G, Gonzalez-Mandly A, Berciano J, Gurevich T, Giladi N, Galitzky M, Rascol O, Kamm C, Gasser T, Siebert U, Poewe W, Wenning GK, EMSA-SG (2010) Presentation, diagnosis, and management of multiple system atrophy in Europe: final analysis of the European multiple system atrophy registry. Mov Disord 25:2604–2612. https://doi.org/10.1002/mds.23192 CrossRefPubMed Kollensperger M, Geser F, Ndayisaba JP, Boesch S, Seppi K, Ostergaard K, Dupont E, Cardozo A, Tolosa E, Abele M, Klockgether T, Yekhlef F, Tison F, Daniels C, Deuschl G, Coelho M, Sampaio C, Bozi M, Quinn N, Schrag A, Mathias CJ, Fowler C, Nilsson CF, Widner H, Schimke N, Oertel W, Del Sorbo F, Albanese A, Pellecchia MT, Barone P, Djaldetti R, Colosimo C, Meco G, Gonzalez-Mandly A, Berciano J, Gurevich T, Giladi N, Galitzky M, Rascol O, Kamm C, Gasser T, Siebert U, Poewe W, Wenning GK, EMSA-SG (2010) Presentation, diagnosis, and management of multiple system atrophy in Europe: final analysis of the European multiple system atrophy registry. Mov Disord 25:2604–2612. https://​doi.​org/​10.​1002/​mds.​23192 CrossRefPubMed
4.
go back to reference Watanabe H, Saito Y, Terao S, Ando T, Kachi T, Mukai E, Aiba I, Abe Y, Tamakoshi A, Doyu M, Hirayama M, Sobue G (2002) Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients. Brain 125:1070–1083CrossRefPubMed Watanabe H, Saito Y, Terao S, Ando T, Kachi T, Mukai E, Aiba I, Abe Y, Tamakoshi A, Doyu M, Hirayama M, Sobue G (2002) Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients. Brain 125:1070–1083CrossRefPubMed
6.
go back to reference Wenning GK, Geser F, Krismer F, Seppi K, Duerr S, Boesch S, Köllensperger M, Goebel G, Pfeiffer KP, Barone P, Pellecchia MT, Quinn NP, Koukouni V, Fowler CJ, Schrag A, Mathias CJ, Giladi N, Gurevich T, Dupont E, Ostergaard K, Nilsson CF, Widner H, Oertel W, Eggert KM, Albanese A, del Sorbo F, Tolosa E, Cardozo A, Deuschl G, Hellriegel H, Klockgether T, Dodel R, Sampaio C, Coelho M, Djaldetti R, Melamed E, Gasser T, Kamm C, Meco G, Colosimo C, Rascol O, Meissner WG, Tison F, Poewe W, European Multiple System Atrophy Study Group (2013) The natural history of multiple system atrophy: a prospective European cohort study. Lancet Neurol 12:264–274. https://doi.org/10.1016/S1474-4422(12)70327-7 CrossRefPubMedCentralPubMed Wenning GK, Geser F, Krismer F, Seppi K, Duerr S, Boesch S, Köllensperger M, Goebel G, Pfeiffer KP, Barone P, Pellecchia MT, Quinn NP, Koukouni V, Fowler CJ, Schrag A, Mathias CJ, Giladi N, Gurevich T, Dupont E, Ostergaard K, Nilsson CF, Widner H, Oertel W, Eggert KM, Albanese A, del Sorbo F, Tolosa E, Cardozo A, Deuschl G, Hellriegel H, Klockgether T, Dodel R, Sampaio C, Coelho M, Djaldetti R, Melamed E, Gasser T, Kamm C, Meco G, Colosimo C, Rascol O, Meissner WG, Tison F, Poewe W, European Multiple System Atrophy Study Group (2013) The natural history of multiple system atrophy: a prospective European cohort study. Lancet Neurol 12:264–274. https://​doi.​org/​10.​1016/​S1474-4422(12)70327-7 CrossRefPubMedCentralPubMed
8.
go back to reference Schulz JB, Klockgether T, Petersen D, Jauch M, Müller-Schauenburg W, Spieker S, Voigt K, Dichgans J (1994) Multiple system atrophy: natural history, MRI morphology and dopamine receptor imaging with 123IBZM-SPECT. J Neurol Neurosurg Psychiatry 57(9):1047–1056CrossRefPubMedCentralPubMed Schulz JB, Klockgether T, Petersen D, Jauch M, Müller-Schauenburg W, Spieker S, Voigt K, Dichgans J (1994) Multiple system atrophy: natural history, MRI morphology and dopamine receptor imaging with 123IBZM-SPECT. J Neurol Neurosurg Psychiatry 57(9):1047–1056CrossRefPubMedCentralPubMed
10.
go back to reference Tada M, Onodera O, Tada M, Ozawa T, Piao YS, Kakita A, Takahashi H, Nishizawa M (2007) Early development of autonomic dysfunction may predict poor prognosis in patients with multiple system atrophy. Arch Neurol 64(2):256–260CrossRefPubMed Tada M, Onodera O, Tada M, Ozawa T, Piao YS, Kakita A, Takahashi H, Nishizawa M (2007) Early development of autonomic dysfunction may predict poor prognosis in patients with multiple system atrophy. Arch Neurol 64(2):256–260CrossRefPubMed
11.
go back to reference Ben-Shlomo Y, Wenning GK, Tison F, Quinn NP (1997) Survival of patients with pathologically proven multiple system atrophy: a meta-analysis. Neurology 48:384–393CrossRefPubMed Ben-Shlomo Y, Wenning GK, Tison F, Quinn NP (1997) Survival of patients with pathologically proven multiple system atrophy: a meta-analysis. Neurology 48:384–393CrossRefPubMed
16.
go back to reference Campanella G, Filla A, De Falco F, Mansi D, Durivage A, Barbeau A (1980) Friedreich’s ataxia in south of Italy: a clinical and biochemical survey of 23 patients. Can J Neurol Sci 7:351–357CrossRefPubMed Campanella G, Filla A, De Falco F, Mansi D, Durivage A, Barbeau A (1980) Friedreich’s ataxia in south of Italy: a clinical and biochemical survey of 23 patients. Can J Neurol Sci 7:351–357CrossRefPubMed
17.
go back to reference Wenning GK, Tison F, Seppi K, Sampaio C, Diem A, Yekhlef F, Ghorayeb I, Ory F, Galitzky M, Scaravilli T, Bozi M, Colosimo C, Gilman S, Shults CW, Quinn NP, Rascol O, Poewe W (2004) Multiple System Atrophy Study Group. Development and validation of the Unified Multiple System Atrophy Rating Scale (UMSARS). Mov Disord 19(12):1391–1402CrossRefPubMed Wenning GK, Tison F, Seppi K, Sampaio C, Diem A, Yekhlef F, Ghorayeb I, Ory F, Galitzky M, Scaravilli T, Bozi M, Colosimo C, Gilman S, Shults CW, Quinn NP, Rascol O, Poewe W (2004) Multiple System Atrophy Study Group. Development and validation of the Unified Multiple System Atrophy Rating Scale (UMSARS). Mov Disord 19(12):1391–1402CrossRefPubMed
Metadata
Title
Longitudinal study of a cohort of MSA-C patients in South Italy: survival and clinical features
Authors
Maria Lieto
Alessandro Roca
Dario Bruzzese
Antonella Antenora
Girolamo Alfieri
Francesco Saccà
Marta Bellofatto
Leonilda Bilo
Stefano Barbato
Giuseppe De Michele
Alessandro Filla
Publication date
01-10-2019
Publisher
Springer International Publishing
Published in
Neurological Sciences / Issue 10/2019
Print ISSN: 1590-1874
Electronic ISSN: 1590-3478
DOI
https://doi.org/10.1007/s10072-019-03948-7

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