Combination disease-modifying treatment in spinal muscular atrophy: A proposed classification.


Journal

Annals of clinical and translational neurology
ISSN: 2328-9503
Titre abrégé: Ann Clin Transl Neurol
Pays: United States
ID NLM: 101623278

Informations de publication

Date de publication:
11 2023
Historique:
revised: 17 07 2023
received: 26 04 2023
accepted: 18 07 2023
medline: 16 11 2023
pubmed: 11 9 2023
entrez: 11 9 2023
Statut: ppublish

Résumé

We sought to devise a rational, systematic approach for defining/grouping survival motor neuron-targeted disease-modifying treatment (DMT) scenarios. The proposed classification is primarily based on a two-part differentiation: initial DMT, and persistence/discontinuation of subsequent DMT(s). Treatment categories were identified: monotherapy add-on, transient add-on, combination with onasemnogene abeparvovec, bridging to onasemnogene abeparvovec, and switching to onasemnogene abeparvovec. We validated this approach by applying the classification to the 443 patients currently in the RESTORE registry and explored the demographics of these different groups of patients. This work forms the basis to explore the safety and efficacy profile of the different combinations of DMT in SMA.

Identifiants

pubmed: 37691296
doi: 10.1002/acn3.51889
pmc: PMC10646995
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

2155-2160

Informations de copyright

© 2023 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.

Références

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Ann Clin Transl Neurol. 2022 Mar;9(3):339-350
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Nat Rev Dis Primers. 2022 Aug 4;8(1):52
pubmed: 35927425

Auteurs

Crystal M Proud (CM)

Children's Hospital of The King's Daughters, Norfolk, Virginia, USA.

Eugenio Mercuri (E)

Department of Paediatric Neurology and Nemo Clinical Centre, Catholic University, Rome, Italy.

Richard S Finkel (RS)

Center for Experimental Neurotherapeutics, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.

Janbernd Kirschner (J)

Department of Neuropediatrics and Muscle Disorders, Medical Center University of Freiburg, Faculty of Medicine, Freiburg, Germany.

Darryl C De Vivo (DC)

Departments of Neurology and Pediatrics, Columbia University Irving Medical Center, New York, New York, USA.

Francesco Muntoni (F)

The Dubowitz Neuromuscular Centre, University College London, Great Ormond Street Institute of Child Health & Great Ormond Street Hospital, London, UK.
National Institute of Health Research, Great Ormond Street Hospital Biomedical Research Centre, London, UK.

Kayoko Saito (K)

Institute of Medical Genetics, Tokyo Women's Medical University, Tokyo, Japan.

Eduardo F Tizzano (EF)

Department of Clinical and Molecular Genetics, Hospital Vall d'Hebron, Barcelona, Spain.

Isabelle Desguerre (I)

Hôpital Necker Enfants Malades, APHP, Paris, France.

Susana Quijano-Roy (S)

Garches Neuromuscular Reference Center (GNMH), APHP Raymond Poincare University Hospital (UVSQ Paris Saclay), Garches, France.

Kamal Benguerba (K)

Novartis Gene Therapies Switzerland GmbH, Rotkreuz, Switzerland.

Dheeraj Raju (D)

Novartis Gene Therapies, Inc, Bannockburn, Illinois, USA.

Eric Faulkner (E)

Novartis Gene Therapies, Inc, Bannockburn, Illinois, USA.
Institute for Precision and Individualized Therapy, Eshelman School of Pharmacy, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
Genomics, Biotech and Emerging Medical Technology Institute, National Association of Managed Care Physicians, Richmond, Virginia, USA.

Laurent Servais (L)

Department of Paediatrics, MDUK Oxford Neuromuscular Centre & NIHR Oxford Biomedical Research Centre, University of Oxford, Oxford, UK.
Department of Paediatrics, Neuromuscular Reference Center, University and University Hospital of Liège, Liège, Belgium.

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