Pattern of pareses in 5q-spinal muscular atrophy
| Autor: | Zeljko Uzelac, Beate Schwäble, Johannes Dorst, Angela Rosenbohm, Kurt Wollinsky, Claudia D. Wurster, Janna S. Steinbreier, Albert C. Ludolph |
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| Jazyk: | angličtina |
| Rok vydání: | 2024 |
| Předmět: | |
| Zdroj: | Therapeutic Advances in Neurological Disorders, Vol 17 (2024) |
| Druh dokumentu: | article |
| ISSN: | 1756-2864 17562864 |
| DOI: | 10.1177/17562864241263420 |
| Popis: | Background: 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. Methods: Between January 2021, and June 2021, 66 SMA patients (59.1% male, aged 33.6 ± 15.2 years) were included in the study. Most patients had SMA type II ( n = 28) or SMA type III ( n = 28), 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. Results: In both, upper and lower limbs muscle weakness was less pronounced in distal (upper limbs: MRC median 3.0 (interquartile range 1.5–3.5); lower limbs: 1.5 (0.5–3.0)) compared to proximal muscle groups (upper limbs: 2.0 (1.5–2.6); p |
| Databáze: | Directory of Open Access Journals |
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