Long-term nusinersen treatment across a wide spectrum of spinal muscular atrophy severity: a real-world experience

被引:0
作者
Anna Łusakowska
Adrianna Wójcik
Anna Frączek
Karolina Aragon-Gawińska
Anna Potulska-Chromik
Paweł Baranowski
Ryszard Nowak
Grzegorz Rosiak
Krzysztof Milczarek
Dariusz Konecki
Zuzanna Gierlak-Wójcicka
Małgorzata Burlewicz
Anna Kostera-Pruszczyk
机构
[1] Medical University of Warsaw,Department of Neurology
[2] ERN EURO-NMD,Department of Neurology and Stroke
[3] Ludwik Rydygier Specialist Hospital,Department of Econometrics, Faculty of Economics and Sociology
[4] University of Łódź,Department of Radiology
[5] Medical University of Warsaw,undefined
来源
Orphanet Journal of Rare Diseases | / 18卷
关键词
Patient global impression – improvement; Computed tomography–guided lumbar puncture; Functional tests; Nusinersen; Scoliosis; gene; Spinal muscular atrophy;
D O I
暂无
中图分类号
学科分类号
摘要
引用
收藏
相关论文
共 215 条
[1]  
Brzustowicz LM(1990)Genetic mapping of chronic childhood-onset spinal muscular atrophy to chromosome 5q11.2-13.3 Nature 344 540-4
[2]  
Lehner T(1990)Mapping of acute (type I) spinal muscular atrophy to chromosome 5q12-q14. The french spinal muscular atrophy investigators Lancet 336 271-3
[3]  
Castilla LH(1995)Identification and characterization of a spinal muscular atrophy-determining gene Cell 80 155-65
[4]  
Penchaszadeh GK(1997)Correlation between severity and SMN protein level in spinal muscular atrophy Nat Genet 16 265-9
[5]  
Wilhelmsen KC(2000)Molecular basis of spinal muscular atrophy: the SMN gene Neurologia 15 393-400
[6]  
Daniels R(1999)A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy Proc Natl Acad Sci U S A 96 6307-11
[7]  
Melki J(2018)Correlation between SMA type and SMN2 copy number revisited: an analysis of 625 unrelated spanish patients and a compilation of 2834 reported cases Neuromuscul Disord 28 208-15
[8]  
Sheth P(2012)Pan-ethnic carrier screening and prenatal diagnosis for spinal muscular atrophy: clinical laboratory analysis of > 72,400 specimens Eur J Hum Genet 20 27-32
[9]  
Abdelhak S(2017)Emerging therapies and challenges in spinal muscular atrophy Ann Neurol 81 355-68
[10]  
Burlet P(2018)Muscle strength and motor function throughout life in a cross-sectional cohort of 180 patients with spinal muscular atrophy types 1c-4 Eur J Neurol 25 512-8