Journal article

Nusinersen for SMA: Expanded access programme

MA Farrar, HL Teoh, KA Carey, A Cairns, R Forbes, K Herbert, S Holland, KJ Jones, MP Menezes, M Morrison, K Munro, D Villano, R Webster, IR Woodcock, EM Yiu, H Sampaio, MM Ryan

Journal of Neurology Neurosurgery and Psychiatry | BMJ PUBLISHING GROUP | Published : 2018

Abstract

Background Spinal muscular atrophy (SMA) is a devastating motor neuron disorder causing progressive muscle weakness and respiratory insufficiency. We present the initial Australian experiences implementing the expanded access programme (EAP) to enable preapproval access to nusinersen, the first disease-modifying therapy, for SMA type 1. Methods An Australian multicentre, open-label EAP for nusinersen enrolled patients with infantile-onset SMA type 1 from November 2016 to September 2017. Standard-of-care medical therapy and treatment with intrathecal nusinersen were provided to all patients. Clinical and diagnostic characteristics, molecular genetics, treatment administered, and functional mo..

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University of Melbourne Researchers