{"id":2348,"date":"2015-03-02T15:36:57","date_gmt":"2015-03-02T15:36:57","guid":{"rendered":"https:\/\/medicalcriteria.com\/web\/neumsa\/"},"modified":"2026-04-06T19:00:39","modified_gmt":"2026-04-06T19:00:39","slug":"neumsa","status":"publish","type":"post","link":"https:\/\/medicalcriteria.com\/web\/neumsa\/","title":{"rendered":"Diagnostic Criteria for Multiple-System Atrophy (MSA)"},"content":{"rendered":"<div class=\"99c380e4b4a7b96c35d7ddf7dcb434e8\" data-index=\"1\" style=\"float: none; margin:0px 0 0px 0; text-align:center;\">\n<script async src=\"https:\/\/pagead2.googlesyndication.com\/pagead\/js\/adsbygoogle.js\"><\/script>\r\n<!-- MC 2019- Horizontal -->\r\n<ins class=\"adsbygoogle\"\r\n     style=\"display:block\"\r\n     data-ad-client=\"ca-pub-0127150553352455\"\r\n     data-ad-slot=\"3806776041\"\r\n     data-ad-format=\"auto\"\r\n     data-full-width-responsive=\"true\"><\/ins>\r\n<script>\r\n     (adsbygoogle = window.adsbygoogle || []).push({});\r\n<\/script>\n<\/div>\n<p>Multiple system atrophy (MSA) is a sporadic and fatal alpha-synuclein-linked oligodendrogliopathy manifesting with progressive autonomic failure, poorly levodopa-responsive parkinsonism, and cerebellar ataxia, in any combination.<br \/>\n<!--more--><br \/>\n<strong>Diagnostic Criteria for Multiple-System Atrophy (MSA)<\/strong><br \/>\n<strong>Definite MSA<\/strong><br \/>\nNeuropathological findings during postmortem examination must include the following:<\/p>\n<ul>\n<li>Widespread and abundant cerebral alpha-synuclein\u2013positive GCIs<\/li>\n<li>Neurodegenerative changes in striatonigral or olivopontocerebellar region<\/li>\n<\/ul>\n<p><strong>Probable MSA<\/strong><br \/>\nSporadic, progressive disease in adults (onset after 30 yr of age) characterized by autonomic failure, including urinary incontinence\u00a0(with erectile dysfunction in men), or an orthostatic decrease in blood pressure by at least 30 mm Hg systolic or 15 mm Hg\u00a0diastolic within 3 min of standing, plus one of the following:<\/p>\n<ul>\n<li>Parkinsonism (slowness of movements, rigidity, and tendency to fall) with poor response to levodopa (parkinsonian subtype\u00a0[MSA-P])<\/li>\n<li>A cerebellar syndrome (wide-based gait, uncoordinated limb movements, action tremor, and nystagmus) (cerebellar subtype\u00a0[MSA-C])<\/li>\n<\/ul>\n<p><strong>Possible MSA<\/strong><br \/>\nA sporadic, progressive, adult-onset disease characterized by the following:<br \/>\n* Parkinsonism (slowness of movements, rigidity, and tendency to fall) or a cerebellar syndrome (wide-based gait, uncoordinated limb\u00a0movements, action tremor, and nystagmus)<br \/>\n* At least one feature suggesting autonomic dysfunction (otherwise unexplained urinary urgency or frequency, incomplete bladder\u00a0emptying, erectile dysfunction in men, or a substantial orthostatic blood-pressure decline that does not meet the level required\u00a0for probable MSA)<br \/>\n* At least one of the following additional features:<\/p>\n<ul>\n<li>Possible MSA-P or MSA-C: Babinski sign with hyperreflexia, stridor<\/li>\n<li>Possible MSA-P: rapidly progressive parkinsonism; poor response to levodopa; recurrent falls within 3 yr after the onset of motor\u00a0symptoms; cerebellar features (wide-based gait; cerebellar dysarthria; uncoordinated limb movements; or spontaneous,\u00a0gaze-evoked, or positional downbeat nystagmus); recurrent choking within 5 yr after the onset of motor symptoms; atrophy\u00a0on MRI of the putamen, middle cerebellar peduncle, pons, or cerebellum; hypometabolism on FDG-PET in the putamen,\u00a0brain stem, or cerebellum<\/li>\n<li>Possible MSA-C: parkinsonism; atrophy on MRI of the putamen, middle cerebellar peduncle, or pons; hypometabolism on FDGPET\u00a0in the putamen; presynaptic nigrostriatal dopaminergic denervation on SPECT or PET<\/li>\n<\/ul>\n<p>Features supporting the diagnosis of MSA (red flags) and features not supporting the diagnosis<\/p>\n<ul>\n<li>Supporting: head\u2013neck dystonia; disproportionate antecollis; bent spine (forward, lateral, or both); contractures of the hands or feet; inspiratory\u00a0sighs; severe dysphonia; severe dysarthria; new or increased snoring; cold hands and feet; emotional incontinence\u00a0(pathologic laughter or crying); jerky, irregular, or postural or action tremor<\/li>\n<li>Not supporting: classic \u201cpill-rolling\u201d rest tremor, clinically significant neuropathy, hallucinations not induced by drugs, onset after 75 yr\u00a0of age, family history of ataxia or parkinsonism, dementia (in accordance with DSM-IV criteria), white-matter lesions suggesting\u00a0multiple sclerosis<\/li>\n<\/ul>\n<p>*FDG-PET [18F]-fluorodeoxyglucose\u2013positron-emission tomography; GCI glial cytoplasmic inclusion; and SPECT single-photon-emission\u00a0computed tomography.<\/p>\n<p>&nbsp;<\/p>\n<p><strong>References:<\/strong><\/p>\n<ol>\n<li>Fanciulli A, Wenning GK. Multiple-system atrophy. N Engl J Med. 2015 Jan 15;372(3):249-63\u00a0<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/25587949\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Wenning GK, Krismer F. Multiple system atrophy. Handb Clin Neurol. 2013;117:229-41.\u00a0<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/24095129\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ, Wood NW, Colosimo C, D\u00fcrr A, Fowler CJ, Kaufmann H, Klockgether T, Lees A, Poewe W, Quinn N, Revesz T, Robertson D, Sandroni P, Seppi K, Vidailhet M.\u00a0Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008 Aug 26;71(9):670-6.\u00a0<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/18725592\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Krismer F, Fanciulli A, Meissner WG, Coon EA, Wenning GK. Multiple system atrophy: advances in pathophysiology, diagnosis, and treatment. Lancet Neurol. 2024 Dec;23(12):1252-1266. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/39577925\/\" target=\"_blank\" rel=\"noopener\">[Medline]<\/a><\/li>\n<\/ol>\n<p>&nbsp;<\/p>\n<p>Created Apr 14, 2015.<br \/>\nUp-date Apr 6, 2026.<\/p>\n\n<div style=\"font-size: 0px; height: 0px; line-height: 0px; margin: 0; padding: 0; clear: both;\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Sorry, this entry is only available in Espa\u00f1ol.<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"no","_lmt_disable":"no","_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"footnotes":""},"categories":[2],"tags":[818],"class_list":["post-2348","post","type-post","status-publish","format-standard","hentry","category-neurology","tag-diagnostic-criteria-for-multiple-system-atrophy-msa"],"modified_by":"Guillermo Firman","_links":{"self":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2348","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/comments?post=2348"}],"version-history":[{"count":5,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2348\/revisions"}],"predecessor-version":[{"id":10729,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2348\/revisions\/10729"}],"wp:attachment":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/media?parent=2348"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/categories?post=2348"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/tags?post=2348"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}