Managing intrathecal administration of nusinersen in adolescents and adults with 5q-spinal muscular atrophy and previous spinal surgery
ABSTRACT Background: Spinal muscular atrophy (SMA) is a neurodegenerative disease of lower motor neurons associated with frequent occurrence of spinal deformity. Nusinersen is an antisense oligonucleotide that increases SMN protein level and is administrated by frequent intrathecal lumbar injection...
| मुख्य लेखकों: | , , , , , , , , |
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| स्वरूप: | लेख |
| भाषा: | English |
| प्रकाशित: |
Academia Brasileira de Neurologia (ABNEURO)
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| श्रृंखला: | Arquivos de Neuro-Psiquiatria |
| विषय: | |
| ऑनलाइन पहुंच: | http://www.scielo.br/pdf/anp/v79n2/1678-4227-anp-79-02-0127.pdf |
| _version_ | 1830508760370511872 |
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| author | Rodrigo de Holanda Mendonça Hermann dos Santos Fernandes Rafael Barbéro Schimmelpfeng Pinto Ciro Matsui Júnior Graziela Jorge Polido André Macedo Serafim da Silva Luis Fernando Grossklauss Umbertina Conti Reed Edmar Zanoteli |
| author_facet | Rodrigo de Holanda Mendonça Hermann dos Santos Fernandes Rafael Barbéro Schimmelpfeng Pinto Ciro Matsui Júnior Graziela Jorge Polido André Macedo Serafim da Silva Luis Fernando Grossklauss Umbertina Conti Reed Edmar Zanoteli |
| author_sort | Rodrigo de Holanda Mendonça |
| collection | DOAJ |
| description | ABSTRACT Background: Spinal muscular atrophy (SMA) is a neurodegenerative disease of lower motor neurons associated with frequent occurrence of spinal deformity. Nusinersen is an antisense oligonucleotide that increases SMN protein level and is administrated by frequent intrathecal lumbar injections. Thus, spinal deformities and previous spinal surgery are important challenges for drug delivery in SMA. Objective: To report imaging methods used for Nusinersen injection in SMA patients. Methods: Nusinersen injection procedures in SMA types 2 and 3 patients who had previous spinal surgery were analyzed retrospectively to describe the imaging and puncture procedures, as well as the occurrence of complications. Results: Nine SMA patients (14 to 50 years old) underwent 57 lumbar punctures for nusinersen injection. Six patients had no interlaminar space available; in five of them, a transforaminal approach was used, and another one underwent a surgery to open a posterior bone window for the injections. Transforaminal puncture was performed using CT scan in three cases and fluoroscopy in the other two, with a similar success rate. One patient in the transforaminal group had post-procedure radiculitis, and another one had vagal reaction (hypotension). In three cases, with preserved interlaminar space, injections were performed by posterior interlaminar puncture, and only one adverse event was reported (post-puncture headache). Conclusion: In SMA patients with previous spinal surgery, the use of imaging-guided intervention is necessary for administering intrathecal nusinersen. Transforaminal technique is indicated in patients for whom the interlaminar space is not available, and injections should always be guided by either CT or fluoroscopy. |
| first_indexed | 2024-12-22T01:22:37Z |
| format | Article |
| id | doaj.art-72543c1c2b0d4e3b8f1cb3d5d24f0b45 |
| institution | Directory Open Access Journal |
| issn | 1678-4227 |
| language | English |
| last_indexed | 2024-12-22T01:22:37Z |
| publisher | Academia Brasileira de Neurologia (ABNEURO) |
| record_format | Article |
| series | Arquivos de Neuro-Psiquiatria |
| spelling | doaj.art-72543c1c2b0d4e3b8f1cb3d5d24f0b452022-12-21T18:43:41ZengAcademia Brasileira de Neurologia (ABNEURO)Arquivos de Neuro-Psiquiatria1678-422710.1590/0004-282x-anp-2020-0200Managing intrathecal administration of nusinersen in adolescents and adults with 5q-spinal muscular atrophy and previous spinal surgeryRodrigo de Holanda MendonçaHermann dos Santos FernandesRafael Barbéro Schimmelpfeng PintoCiro Matsui JúniorGraziela Jorge PolidoAndré Macedo Serafim da SilvaLuis Fernando GrossklaussUmbertina Conti ReedEdmar ZanoteliABSTRACT Background: Spinal muscular atrophy (SMA) is a neurodegenerative disease of lower motor neurons associated with frequent occurrence of spinal deformity. Nusinersen is an antisense oligonucleotide that increases SMN protein level and is administrated by frequent intrathecal lumbar injections. Thus, spinal deformities and previous spinal surgery are important challenges for drug delivery in SMA. Objective: To report imaging methods used for Nusinersen injection in SMA patients. Methods: Nusinersen injection procedures in SMA types 2 and 3 patients who had previous spinal surgery were analyzed retrospectively to describe the imaging and puncture procedures, as well as the occurrence of complications. Results: Nine SMA patients (14 to 50 years old) underwent 57 lumbar punctures for nusinersen injection. Six patients had no interlaminar space available; in five of them, a transforaminal approach was used, and another one underwent a surgery to open a posterior bone window for the injections. Transforaminal puncture was performed using CT scan in three cases and fluoroscopy in the other two, with a similar success rate. One patient in the transforaminal group had post-procedure radiculitis, and another one had vagal reaction (hypotension). In three cases, with preserved interlaminar space, injections were performed by posterior interlaminar puncture, and only one adverse event was reported (post-puncture headache). Conclusion: In SMA patients with previous spinal surgery, the use of imaging-guided intervention is necessary for administering intrathecal nusinersen. Transforaminal technique is indicated in patients for whom the interlaminar space is not available, and injections should always be guided by either CT or fluoroscopy.http://www.scielo.br/pdf/anp/v79n2/1678-4227-anp-79-02-0127.pdfMuscular Atrophy, SpinalAntisense OligonucleotideSpinal PunctureSurvival of Motor Neuron 1 ProteinMotor Neuron Disease |
| spellingShingle | Rodrigo de Holanda Mendonça Hermann dos Santos Fernandes Rafael Barbéro Schimmelpfeng Pinto Ciro Matsui Júnior Graziela Jorge Polido André Macedo Serafim da Silva Luis Fernando Grossklauss Umbertina Conti Reed Edmar Zanoteli Managing intrathecal administration of nusinersen in adolescents and adults with 5q-spinal muscular atrophy and previous spinal surgery Arquivos de Neuro-Psiquiatria Muscular Atrophy, Spinal Antisense Oligonucleotide Spinal Puncture Survival of Motor Neuron 1 Protein Motor Neuron Disease |
| title | Managing intrathecal administration of nusinersen in adolescents and adults with 5q-spinal muscular atrophy and previous spinal surgery |
| title_full | Managing intrathecal administration of nusinersen in adolescents and adults with 5q-spinal muscular atrophy and previous spinal surgery |
| title_fullStr | Managing intrathecal administration of nusinersen in adolescents and adults with 5q-spinal muscular atrophy and previous spinal surgery |
| title_full_unstemmed | Managing intrathecal administration of nusinersen in adolescents and adults with 5q-spinal muscular atrophy and previous spinal surgery |
| title_short | Managing intrathecal administration of nusinersen in adolescents and adults with 5q-spinal muscular atrophy and previous spinal surgery |
| title_sort | managing intrathecal administration of nusinersen in adolescents and adults with 5q spinal muscular atrophy and previous spinal surgery |
| topic | Muscular Atrophy, Spinal Antisense Oligonucleotide Spinal Puncture Survival of Motor Neuron 1 Protein Motor Neuron Disease |
| url | http://www.scielo.br/pdf/anp/v79n2/1678-4227-anp-79-02-0127.pdf |
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