Abstract
Multiple system atrophy (MSA) is a neurodegenerative disease with two motor phenotypes: parkinsonian (MSA-P) and cerebellar (MSA-C). To elucidate whether in addition to the motor abnormalities there are other significant differences between these phenotypes, we performed a retrospective review of 100 patients (61 males, 39 females) with a diagnosis of possible (12 %), or probable (88 %) MSA. Four patients eventually had post-mortem confirmation (i.e., definite MSA). Sixty percent were classified as having MSA-P and 40 % as MSA-C. MSA-C and MSA-P patients had similar male prevalence (60 %), age of onset (56 ± 9 years), and frequency of OH (69 %). Brain MRI abnormalities were more frequent in MSA-C patients (p < 0.001). Mean survival was 8 ± 3 years for MSA-C and 9 ± 4 years for MSA-P patients (p = 0.22). Disease onset before 55 years predicted longer survival in both phenotypes. Initial autonomic involvement did not influence survival. We conclude that patients with both motor phenotypes have mostly similar survivals and demographic distributions. The differences here identified could help counseling of patients with MSA.
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Acknowledgments
National Institutes for Health (NIH) Rare Diseases Clinical Research Network Grant (U54 S065736 to LJNK, JM and HK) and the Dysautonomia Foundation, Inc. (to DR, JAP, NG, and HK). The Rare Autonomic Disorders Consortium is a part of the National Institutes of Health (NIH) Rare Diseases Clinical Research Network (RDCRN), supported through collaboration between the NIH Office of Rare Diseases Research (ORDR) at the National Center for Advancing Translational Science (NCATS), and the NINDS. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.
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Roncevic, D., Palma, JA., Martinez, J. et al. Cerebellar and parkinsonian phenotypes in multiple system atrophy: similarities, differences and survival. J Neural Transm 121, 507–512 (2014). https://doi.org/10.1007/s00702-013-1133-7
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DOI: https://doi.org/10.1007/s00702-013-1133-7