Intrathecal administration of nusinersen for spinal muscular atrophy: report of three cases with severe spinal deformity
Abstract Background Spinal muscular atrophy (SMA) is a genetic disease characterized by degeneration of the spinal cord, resulting in progressive muscle atrophy. Recently, nusinersen has been approved for treating SMA, which should be administered intrathecally. Case presentation Patient 1 was a 36-...
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| Format: | Article |
| Language: | English |
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SpringerOpen
2020-04-01
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| Series: | JA Clinical Reports |
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| Online Access: | http://link.springer.com/article/10.1186/s40981-020-00334-7 |
| _version_ | 1818353138269159424 |
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| author | Sayo Nakao Shinichi Yamada Katsuya Tsuda Taishi Yokomizo Teruyuki Sato Shuichi Tanoue Teruyuki Hiraki |
| author_facet | Sayo Nakao Shinichi Yamada Katsuya Tsuda Taishi Yokomizo Teruyuki Sato Shuichi Tanoue Teruyuki Hiraki |
| author_sort | Sayo Nakao |
| collection | DOAJ |
| description | Abstract Background Spinal muscular atrophy (SMA) is a genetic disease characterized by degeneration of the spinal cord, resulting in progressive muscle atrophy. Recently, nusinersen has been approved for treating SMA, which should be administered intrathecally. Case presentation Patient 1 was a 36-year-old woman with SMA type 2. Patients 2 and 3 were 10- and 17-year-old girls with SMA type 1. In patients 1 and 2, the needle was inserted into the spinal column, but outflow of cerebrospinal fluid was unable to be confirmed. CT revealed that the dural sac terminated at the L5 level in patients 1 and 3 and at the L5/S1 level in patient 2. Conclusions Patients with SMA often present with high-grade scoliosis, making intrathecal administration difficult. In addition, the dural sac may terminate at a level higher than normal. To ensure intrathecal administration, the level of dural sac termination must be confirmed by CT before puncture. |
| first_indexed | 2024-12-13T19:04:46Z |
| format | Article |
| id | doaj.art-295e84374d6248ad85291bff27e57e4d |
| institution | Directory Open Access Journal |
| issn | 2363-9024 |
| language | English |
| last_indexed | 2024-12-13T19:04:46Z |
| publishDate | 2020-04-01 |
| publisher | SpringerOpen |
| record_format | Article |
| series | JA Clinical Reports |
| spelling | doaj.art-295e84374d6248ad85291bff27e57e4d2022-12-21T23:34:34ZengSpringerOpenJA Clinical Reports2363-90242020-04-01611410.1186/s40981-020-00334-7Intrathecal administration of nusinersen for spinal muscular atrophy: report of three cases with severe spinal deformitySayo Nakao0Shinichi Yamada1Katsuya Tsuda2Taishi Yokomizo3Teruyuki Sato4Shuichi Tanoue5Teruyuki Hiraki6Department of Anesthesiology, Kurume University School of MedicineDepartment of Anesthesiology, Kurume University School of MedicineDepartment of Anesthesiology, Kurume University School of MedicineDepartment of Anesthesiology, Kurume University School of MedicineDepartment of Anesthesiology, Kurume University School of MedicineDepartment of Radiology, Kurume University School of MedicineDepartment of Anesthesiology, Kurume University School of MedicineAbstract Background Spinal muscular atrophy (SMA) is a genetic disease characterized by degeneration of the spinal cord, resulting in progressive muscle atrophy. Recently, nusinersen has been approved for treating SMA, which should be administered intrathecally. Case presentation Patient 1 was a 36-year-old woman with SMA type 2. Patients 2 and 3 were 10- and 17-year-old girls with SMA type 1. In patients 1 and 2, the needle was inserted into the spinal column, but outflow of cerebrospinal fluid was unable to be confirmed. CT revealed that the dural sac terminated at the L5 level in patients 1 and 3 and at the L5/S1 level in patient 2. Conclusions Patients with SMA often present with high-grade scoliosis, making intrathecal administration difficult. In addition, the dural sac may terminate at a level higher than normal. To ensure intrathecal administration, the level of dural sac termination must be confirmed by CT before puncture.http://link.springer.com/article/10.1186/s40981-020-00334-7Spinal muscular atrophyIntrathecal administrationNusinersenSevere scoliosis |
| spellingShingle | Sayo Nakao Shinichi Yamada Katsuya Tsuda Taishi Yokomizo Teruyuki Sato Shuichi Tanoue Teruyuki Hiraki Intrathecal administration of nusinersen for spinal muscular atrophy: report of three cases with severe spinal deformity JA Clinical Reports Spinal muscular atrophy Intrathecal administration Nusinersen Severe scoliosis |
| title | Intrathecal administration of nusinersen for spinal muscular atrophy: report of three cases with severe spinal deformity |
| title_full | Intrathecal administration of nusinersen for spinal muscular atrophy: report of three cases with severe spinal deformity |
| title_fullStr | Intrathecal administration of nusinersen for spinal muscular atrophy: report of three cases with severe spinal deformity |
| title_full_unstemmed | Intrathecal administration of nusinersen for spinal muscular atrophy: report of three cases with severe spinal deformity |
| title_short | Intrathecal administration of nusinersen for spinal muscular atrophy: report of three cases with severe spinal deformity |
| title_sort | intrathecal administration of nusinersen for spinal muscular atrophy report of three cases with severe spinal deformity |
| topic | Spinal muscular atrophy Intrathecal administration Nusinersen Severe scoliosis |
| url | http://link.springer.com/article/10.1186/s40981-020-00334-7 |
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