Cerebellar and parkinsonian phenotypes in multiple system atrophy: similarities, differences and survival
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 10...
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| Veröffentlicht in: | Journal of Neural Transmission 2014-05, Vol.121 (5), p.507-512 |
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| creator | Roncevic, Dusan Palma, Jose-Alberto Martinez, Jose Goulding, Niamh Norcliffe-Kaufmann, Lucy Kaufmann, Horacio |
| description | 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 (
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| doi_str_mv | 10.1007/s00702-013-1133-7 |
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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.</description><identifier>ISSN: 0300-9564</identifier><identifier>EISSN: 1435-1463</identifier><identifier>DOI: 10.1007/s00702-013-1133-7</identifier><identifier>PMID: 24337696</identifier><identifier>CODEN: JNTRF3</identifier><language>eng</language><publisher>Vienna: Springer Vienna</publisher><subject>Age of Onset ; Anti-Dyskinesia Agents - therapeutic use ; Brain - pathology ; Disease Progression ; Female ; Humans ; Levodopa - therapeutic use ; Magnetic Resonance Imaging ; Male ; Medicine ; Medicine & Public Health ; Middle Aged ; Motor Activity - drug effects ; Multiple System Atrophy - diagnosis ; Multiple System Atrophy - drug therapy ; Multiple System Atrophy - epidemiology ; Multiple System Atrophy - pathology ; Neurology ; Neurology and Preclinical Neurological Studies - Original Article ; Neurosciences ; Phenotype ; Prevalence ; Prognosis ; Psychiatry ; Retrospective Studies ; Sex Factors ; Survival Analysis ; Treatment Outcome</subject><ispartof>Journal of Neural Transmission, 2014-05, Vol.121 (5), p.507-512</ispartof><rights>Springer-Verlag Wien 2013</rights><rights>Springer-Verlag Wien 2014</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-p267t-446a10decc5cda1c3cbdf9f2d26caf23da60f311868cad8d4ee3d8b2a197b40a3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s00702-013-1133-7$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s00702-013-1133-7$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>230,314,780,784,885,27924,27925,41488,42557,51319</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/24337696$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Roncevic, Dusan</creatorcontrib><creatorcontrib>Palma, Jose-Alberto</creatorcontrib><creatorcontrib>Martinez, Jose</creatorcontrib><creatorcontrib>Goulding, Niamh</creatorcontrib><creatorcontrib>Norcliffe-Kaufmann, Lucy</creatorcontrib><creatorcontrib>Kaufmann, Horacio</creatorcontrib><title>Cerebellar and parkinsonian phenotypes in multiple system atrophy: similarities, differences and survival</title><title>Journal of Neural Transmission</title><addtitle>J Neural Transm</addtitle><addtitle>J Neural Transm (Vienna)</addtitle><description>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.</description><subject>Age of Onset</subject><subject>Anti-Dyskinesia Agents - therapeutic use</subject><subject>Brain - pathology</subject><subject>Disease Progression</subject><subject>Female</subject><subject>Humans</subject><subject>Levodopa - therapeutic use</subject><subject>Magnetic Resonance Imaging</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Middle Aged</subject><subject>Motor Activity - drug effects</subject><subject>Multiple System Atrophy - diagnosis</subject><subject>Multiple System Atrophy - drug therapy</subject><subject>Multiple System Atrophy - epidemiology</subject><subject>Multiple System Atrophy - pathology</subject><subject>Neurology</subject><subject>Neurology and Preclinical Neurological Studies - Original Article</subject><subject>Neurosciences</subject><subject>Phenotype</subject><subject>Prevalence</subject><subject>Prognosis</subject><subject>Psychiatry</subject><subject>Retrospective Studies</subject><subject>Sex Factors</subject><subject>Survival Analysis</subject><subject>Treatment Outcome</subject><issn>0300-9564</issn><issn>1435-1463</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2014</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpdkcFu1DAQhi0EotvCA3BBlrhwIDDj8ToJByS0ooBUiQucLcd2ui6JE-xkpX17vNqCCpeZw3zzaeyfsRcIbxGgfpdLAVEBUoVIVNWP2AYlbSuUih6zDRBA1W6VvGCXOd8BAGLdPGUXQhLVqlUbFnY--c4Pg0ncRMdnk36GmKcYTOTz3sdpOc4-8xD5uA5LmAfP8zEvfuRmSdO8P77nOYyh7Icl-PyGu9D3xRlt2ToZ85oO4WCGZ-xJb4bsn9_3K_bj-tP33Zfq5tvnr7uPN9UsVL1UUiqD4Ly1W-sMWrKd69teOKGs6QU5o6AnxEY11rjGSe_JNZ0w2NadBENX7MPZO6_d6J31cUlm0HMKo0lHPZmg_53EsNe300FLJAnQFsHre0Gafq0-L3oM2Z6-KPppzRq3Qha2Jizoq__Qu2lNsTyvUNgIAtXIQr18eNHfU_6kUABxBnIZxVufHmhAn6LW56h1iVqfotY1_QatJZ1s</recordid><startdate>20140501</startdate><enddate>20140501</enddate><creator>Roncevic, Dusan</creator><creator>Palma, Jose-Alberto</creator><creator>Martinez, Jose</creator><creator>Goulding, Niamh</creator><creator>Norcliffe-Kaufmann, Lucy</creator><creator>Kaufmann, Horacio</creator><general>Springer Vienna</general><general>Springer Nature B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>K9.</scope><scope>7TK</scope><scope>5PM</scope></search><sort><creationdate>20140501</creationdate><title>Cerebellar and parkinsonian phenotypes in multiple system atrophy: similarities, differences and survival</title><author>Roncevic, Dusan ; Palma, Jose-Alberto ; Martinez, Jose ; Goulding, Niamh ; Norcliffe-Kaufmann, Lucy ; Kaufmann, Horacio</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p267t-446a10decc5cda1c3cbdf9f2d26caf23da60f311868cad8d4ee3d8b2a197b40a3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2014</creationdate><topic>Age of Onset</topic><topic>Anti-Dyskinesia Agents - therapeutic use</topic><topic>Brain - pathology</topic><topic>Disease Progression</topic><topic>Female</topic><topic>Humans</topic><topic>Levodopa - therapeutic use</topic><topic>Magnetic Resonance Imaging</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Middle Aged</topic><topic>Motor Activity - drug effects</topic><topic>Multiple System Atrophy - diagnosis</topic><topic>Multiple System Atrophy - drug therapy</topic><topic>Multiple System Atrophy - epidemiology</topic><topic>Multiple System Atrophy - pathology</topic><topic>Neurology</topic><topic>Neurology and Preclinical Neurological Studies - Original Article</topic><topic>Neurosciences</topic><topic>Phenotype</topic><topic>Prevalence</topic><topic>Prognosis</topic><topic>Psychiatry</topic><topic>Retrospective Studies</topic><topic>Sex Factors</topic><topic>Survival Analysis</topic><topic>Treatment Outcome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Roncevic, Dusan</creatorcontrib><creatorcontrib>Palma, Jose-Alberto</creatorcontrib><creatorcontrib>Martinez, Jose</creatorcontrib><creatorcontrib>Goulding, Niamh</creatorcontrib><creatorcontrib>Norcliffe-Kaufmann, Lucy</creatorcontrib><creatorcontrib>Kaufmann, Horacio</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Neurosciences Abstracts</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Journal of Neural Transmission</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Roncevic, Dusan</au><au>Palma, Jose-Alberto</au><au>Martinez, Jose</au><au>Goulding, Niamh</au><au>Norcliffe-Kaufmann, Lucy</au><au>Kaufmann, Horacio</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Cerebellar and parkinsonian phenotypes in multiple system atrophy: similarities, differences and survival</atitle><jtitle>Journal of Neural Transmission</jtitle><stitle>J Neural Transm</stitle><addtitle>J Neural Transm (Vienna)</addtitle><date>2014-05-01</date><risdate>2014</risdate><volume>121</volume><issue>5</issue><spage>507</spage><epage>512</epage><pages>507-512</pages><issn>0300-9564</issn><eissn>1435-1463</eissn><coden>JNTRF3</coden><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.</abstract><cop>Vienna</cop><pub>Springer Vienna</pub><pmid>24337696</pmid><doi>10.1007/s00702-013-1133-7</doi><tpages>6</tpages></addata></record> |
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| subjects | Age of Onset Anti-Dyskinesia Agents - therapeutic use Brain - pathology Disease Progression Female Humans Levodopa - therapeutic use Magnetic Resonance Imaging Male Medicine Medicine & Public Health Middle Aged Motor Activity - drug effects Multiple System Atrophy - diagnosis Multiple System Atrophy - drug therapy Multiple System Atrophy - epidemiology Multiple System Atrophy - pathology Neurology Neurology and Preclinical Neurological Studies - Original Article Neurosciences Phenotype Prevalence Prognosis Psychiatry Retrospective Studies Sex Factors Survival Analysis Treatment Outcome |
| title | Cerebellar and parkinsonian phenotypes in multiple system atrophy: similarities, differences and survival |
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