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Conference Paper: The impact of Nusinersen treatment on scoliosis progression in patients with spinal muscular atrophy
| Title | The impact of Nusinersen treatment on scoliosis progression in patients with spinal muscular atrophy |
|---|---|
| Authors | |
| Issue Date | 2022 |
| Publisher | Scoliosis Research Society. |
| Citation | 29th International Meeting on Advanced Spine Techniques (IMAST) (Hybrid), Miami, Florida, USA, 6-9 April 2022. In 29th IMAST: International Meeting on Advanced Spine Techniques, 6-9 Apr 2022, Miami, Florida, USA: Final Program, p. 38-38 How to Cite? |
| Abstract | Objectives: Nusinersen treatment has improved motor function, ambulatory status and carers' satisfaction for spinal muscular atrophy patients. However its effect on scoliosis progression is unknown. The aim of this study was to examine the degree of scoliosis progression in SMA patients on Nusinersen.
Methods: 24 SMA patients with a minimum of 6 month follow up after commencement of Nusinersen treatment were recruited. Demographics, motor function, patient and carers' satisfaction were reviewed. Only patients with erect whole spine radiographs before and after treatment were included in analysis.
Results: 21 patients (Type 1=7, Type 2=7, Type III=7) were included in the analysis. The mean follow up was 29, 22 and 17 months respectively. Motor function was stable or improved in 18 patients. Ambulatory status also improved, most notably 57% of Type I patients became sitters (from 0%). Self or parent-reported improvements including stability and muscle strength were seen in all (100%) patients. However, scoliosis progression continued with a mean Cobb progression of 4.8, 13.8 and 4.0 degree per year for type I, II and III patients respectively. Sub-analysis of Type I and II patients showed that progression was most rapid (16.5 and 18.4 degree per year respectively) between ages 5-10 years. This rate is similar to previously reported by Wikingarrde et al. (5-12 degree per year for Type II, and 2.9-15 degree per year for Type III).
Conclusion: Nusinersen treatment does not reduce the rate of scoliosis progression in SMA patients, and close monitoring with expectant surgical management is still required.
Keywords: Spinal Muscular Atrophy, Scoliosis, Nusinersen, Motor function, Motor neuron disease |
| Description | Session 1. Whitecloud Award Nominees; Paper #6 |
| Persistent Identifier | http://hdl.handle.net/10722/316887 |
| DC Field | Value | Language |
|---|---|---|
| dc.contributor.author | Kwan, KYH | - |
| dc.contributor.author | Ip, HHN | - |
| dc.contributor.author | Chan, S | - |
| dc.date.accessioned | 2022-09-16T07:25:04Z | - |
| dc.date.available | 2022-09-16T07:25:04Z | - |
| dc.date.issued | 2022 | - |
| dc.identifier.citation | 29th International Meeting on Advanced Spine Techniques (IMAST) (Hybrid), Miami, Florida, USA, 6-9 April 2022. In 29th IMAST: International Meeting on Advanced Spine Techniques, 6-9 Apr 2022, Miami, Florida, USA: Final Program, p. 38-38 | - |
| dc.identifier.uri | http://hdl.handle.net/10722/316887 | - |
| dc.description | Session 1. Whitecloud Award Nominees; Paper #6 | - |
| dc.description.abstract | Objectives: Nusinersen treatment has improved motor function, ambulatory status and carers' satisfaction for spinal muscular atrophy patients. However its effect on scoliosis progression is unknown. The aim of this study was to examine the degree of scoliosis progression in SMA patients on Nusinersen. Methods: 24 SMA patients with a minimum of 6 month follow up after commencement of Nusinersen treatment were recruited. Demographics, motor function, patient and carers' satisfaction were reviewed. Only patients with erect whole spine radiographs before and after treatment were included in analysis. Results: 21 patients (Type 1=7, Type 2=7, Type III=7) were included in the analysis. The mean follow up was 29, 22 and 17 months respectively. Motor function was stable or improved in 18 patients. Ambulatory status also improved, most notably 57% of Type I patients became sitters (from 0%). Self or parent-reported improvements including stability and muscle strength were seen in all (100%) patients. However, scoliosis progression continued with a mean Cobb progression of 4.8, 13.8 and 4.0 degree per year for type I, II and III patients respectively. Sub-analysis of Type I and II patients showed that progression was most rapid (16.5 and 18.4 degree per year respectively) between ages 5-10 years. This rate is similar to previously reported by Wikingarrde et al. (5-12 degree per year for Type II, and 2.9-15 degree per year for Type III). Conclusion: Nusinersen treatment does not reduce the rate of scoliosis progression in SMA patients, and close monitoring with expectant surgical management is still required. Keywords: Spinal Muscular Atrophy, Scoliosis, Nusinersen, Motor function, Motor neuron disease | - |
| dc.language | eng | - |
| dc.publisher | Scoliosis Research Society. | - |
| dc.relation.ispartof | 29th IMAST: International Meeting on Advanced Spine Techniques, 6-9 April, 2022, Miami, Florida, USA: Final Program | - |
| dc.title | The impact of Nusinersen treatment on scoliosis progression in patients with spinal muscular atrophy | - |
| dc.type | Conference_Paper | - |
| dc.identifier.email | Kwan, KYH: kyhkwan@hku.hk | - |
| dc.identifier.email | Chan, S: sophehs@hku.hk | - |
| dc.identifier.authority | Kwan, KYH=rp02014 | - |
| dc.identifier.authority | Chan, S=rp02210 | - |
| dc.identifier.hkuros | 336627 | - |
| dc.identifier.spage | 38 | - |
| dc.identifier.epage | 38 | - |
| dc.publisher.place | United States | - |