{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T13:36:44Z","timestamp":1753882604437,"version":"3.41.2"},"reference-count":42,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Ther Adv Neurol Disord"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:sec><jats:title>Background:<\/jats:title><jats:p> This prospective study investigates the pattern of pareses in 5q-associated spinal muscular atrophy (SMA) to identify disease-specific characteristics and potential differences from amyotrophic lateral sclerosis (ALS) and spinobulbar muscular atrophy (SBMA). Detailed knowledge about pareses patterns in SMA facilitates differential diagnosis and supports therapeutic monitoring. <\/jats:p><\/jats:sec><jats:sec><jats:title>Methods:<\/jats:title><jats:p> Between January 2021, and June 2021, 66 SMA patients (59.1% male, aged 33.6\u2009\u00b1\u200915.2\u2009years) were included in the study. Most patients had SMA type II ( n\u2009=\u200928) or SMA type III ( n\u2009=\u200928), seven patients had SMA type I, and three patients had SMA type IV. We analyzed the pattern of pareses using the UK Medical Research Council (MRC) scoring system. <\/jats:p><\/jats:sec><jats:sec><jats:title>Results:<\/jats:title><jats:p> In both, upper and lower limbs muscle weakness was less pronounced in distal (upper limbs: MRC median 3.0 (interquartile range 1.5\u20133.5); lower limbs: 1.5 (0.5\u20133.0)) compared to proximal muscle groups (upper limbs: 2.0 (1.5\u20132.6); p\u2009&lt;\u20090.001; lower limbs: 0.5 (0.5\u20131.5); p\u2009&lt;\u20090.001). Thenar muscles were stronger than other small hand muscles (3.0 (2.0\u20133.5) vs 3.0 (1.5\u20133.5); p\u2009=\u20090.004). Muscles had more strength in upper (2.3 (1.5\u20133.1)) compared to lower limbs (1.1 (0.5\u20132.3); p\u2009&lt;\u20090.001) and in flexors compared to extensors. <\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion:<\/jats:title><jats:p> We identified a specific pattern of muscle paresis in SMA which is different from the pattern of paresis in ALS and SBMA. As a rule of thumb, the pattern of pareses is similar, but not identical to ALS in distal, but different in proximal muscle groups. <\/jats:p><\/jats:sec>","DOI":"10.1177\/17562864241263420","type":"journal-article","created":{"date-parts":[[2024,8,27]],"date-time":"2024-08-27T09:50:52Z","timestamp":1724752252000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":1,"title":["Pattern of pareses in 5q-spinal muscular atrophy"],"prefix":"10.1177","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2479-2025","authenticated-orcid":false,"given":"Zeljko","family":"Uzelac","sequence":"first","affiliation":[{"name":"Department of Neurology, Ulm University, Ulm, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beate","family":"Schw\u00e4ble","sequence":"additional","affiliation":[{"name":"Department of Neurology, Ulm University, Ulm, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0338-5439","authenticated-orcid":false,"given":"Johannes","family":"Dorst","sequence":"additional","affiliation":[{"name":"Department of Neurology, Ulm University, Ulm, Germany"},{"name":"German Center for Neurodegenerative Diseases, Research Site Ulm, Ulm, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9700-0338","authenticated-orcid":false,"given":"Angela","family":"Rosenbohm","sequence":"additional","affiliation":[{"name":"Department of Neurology, Ulm University, Ulm, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kurt","family":"Wollinsky","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, RKU\u2014University and Rehabilitation Clinics, Ulm University, Ulm, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia 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