{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T19:37:26Z","timestamp":1790537846236,"version":"4.1.0"},"reference-count":57,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2022,12,9]],"date-time":"2022-12-09T00:00:00Z","timestamp":1670544000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004243","name":"Prinses Beatrix Spierfonds","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100004243","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Stichting Spieren voor Spieren and Vriendenloterij"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,12,29]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Hereditary proximal spinal muscular atrophy causes weakness and increased fatigability of repetitive motor functions. The neuromuscular junction is anatomically and functionally abnormal in patients with spinal muscular atrophy. Pharmacological improvement of neuromuscular transmission may therefore represent a promising additional treatment strategy. We conducted a Phase II, monocentre, placebo-controlled, double-blind, cross-over trial with the acetylcholinesterase inhibitor pyridostigmine in treatment-na\u00efve patients with spinal muscular atrophy types 2\u20134. We investigated the safety and efficacy of pyridostigmine on fatigability and motor function. Each participant received pyridostigmine and a placebo for 8 weeks, in random order. Primary outcomes were the repeated nine-hole peg test for fatigability and motor function measure. Secondary outcomes were patient-reported effects, endurance shuttle test combined scores and adverse events. We included 35 patients. For the repeated nine-hole peg test, the mean difference was 0.17\u2005s\/trial (95% confidence interval: \u22121.17\u20131.49; P = 0.8), favouring placebo, and for the motor function measure, 0.74% (95% confidence interval: 0.00\u20131.49; P = 0.05), favouring pyridostigmine. Around 74% of patients reported medium-to-large beneficial effects of pyridostigmine on fatigability, compared with 29.7% in the placebo arm. This was paralleled by a reduced dropout risk of 70% on the endurance shuttle test combined scores (hazard ratio: 0.30; 95% confidence interval: 0.15\u20130.58) under pyridostigmine. Adverse events, mostly mild and self-limiting, occurred more frequently under pyridostigmine. No serious adverse events related to the study medication were observed. Patients with spinal muscular atrophy tolerated pyridostigmine well. There were no significant differences in primary outcomes, but the self-reported reduction of fatigability and improved endurance shuttle test combined score performance suggest that pyridostigmine may be useful as an additional therapy to survival motor neuron-augmenting drugs.<\/jats:p><jats:p>Trial registration number: EudraCT: 2011\u2013004369-34, NCT02941328<\/jats:p>","DOI":"10.1093\/braincomms\/fcac324","type":"journal-article","created":{"date-parts":[[2022,12,9]],"date-time":"2022-12-09T05:14:06Z","timestamp":1670562846000},"source":"Crossref","is-referenced-by-count":26,"title":["Randomized double-blind placebo-controlled crossover trial with pyridostigmine in spinal muscular atrophy types 2\u20134"],"prefix":"10.1093","volume":"5","author":[{"given":"Marloes","family":"Stam","sequence":"first","affiliation":[{"name":"UMC Utrecht Brain Centre, Department of Neurology, University Medical Centre Utrecht, Utrecht University , 3584 CX Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3661-0962","authenticated-orcid":false,"given":"Camiel A","family":"Wijngaarde","sequence":"additional","affiliation":[{"name":"UMC Utrecht Brain Centre, Department of Neurology, University Medical Centre Utrecht, Utrecht University , 3584 CX Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bart","family":"Bartels","sequence":"additional","affiliation":[{"name":"Child Development and Exercise Centre, Wilhelmina\u2019s Children Hospital, University Medical Centre Utrecht, Utrecht University , 3584 EA Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fay-Lynn","family":"Asselman","sequence":"additional","affiliation":[{"name":"UMC Utrecht Brain Centre, Department of Neurology, University Medical Centre Utrecht, Utrecht University , 3584 CX Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louise A M","family":"Otto","sequence":"additional","affiliation":[{"name":"UMC Utrecht Brain Centre, Department of Neurology, University Medical Centre Utrecht, Utrecht University , 3584 CX Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura E","family":"Habets","sequence":"additional","affiliation":[{"name":"Child Development and Exercise Centre, Wilhelmina\u2019s Children Hospital, University Medical Centre Utrecht, Utrecht University , 3584 EA Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruben P A","family":"van Eijk","sequence":"additional","affiliation":[{"name":"UMC Utrecht Brain Centre, Department of Neurology, University Medical Centre Utrecht, Utrecht University , 3584 CX Utrecht , the Netherlands"},{"name":"Biostatistics & Research Support, Julius Centre for Health Sciences and Primary Care, University Medical Centre Utrecht , 3584 CG Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bas M","family":"Middelkoop","sequence":"additional","affiliation":[{"name":"UMC Utrecht Brain Centre, Department of Neurology, University Medical Centre Utrecht, Utrecht University , 3584 CX Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H Stephan","family":"Goedee","sequence":"additional","affiliation":[{"name":"UMC Utrecht Brain Centre, Department of Neurology, University Medical Centre Utrecht, Utrecht University , 3584 CX Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janke F","family":"de Groot","sequence":"additional","affiliation":[{"name":"Child Development and Exercise Centre, Wilhelmina\u2019s Children Hospital, University Medical Centre Utrecht, Utrecht University , 3584 EA Utrecht , the Netherlands"},{"name":"Knowledge Institute for Medical Specialists , Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kit C B","family":"Roes","sequence":"additional","affiliation":[{"name":"Biostatistics & Research Support, Julius Centre for Health Sciences and Primary Care, University Medical Centre Utrecht , 3584 CG Utrecht , the Netherlands"},{"name":"Department of Health Evidence, Section Biostatistics, Radboud University Medical Centre, Radboud University , 6525 EZ Nijmegen , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marja A G C","family":"Schoenmakers","sequence":"additional","affiliation":[{"name":"Child Development and Exercise Centre, Wilhelmina\u2019s Children Hospital, University Medical Centre Utrecht, Utrecht University , 3584 EA Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward E S","family":"Nieuwenhuis","sequence":"additional","affiliation":[{"name":"Department of Paediatric Gastroenterology, Wilhelmina\u2019s Children Hospital, University Medical Centre Utrecht, Utrecht University , 3584 EA Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Inge","family":"Cuppen","sequence":"additional","affiliation":[{"name":"UMC Utrecht Brain Centre, Department of Neurology and Child Neurology, Wilhelmina\u2019s Children Hospital, University Medical Centre Utrecht, Utrecht University , 3584 EA Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leonard H","family":"van den Berg","sequence":"additional","affiliation":[{"name":"UMC Utrecht Brain Centre, Department of Neurology, University Medical Centre Utrecht, Utrecht University , 3584 CX Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0339-5506","authenticated-orcid":false,"given":"Renske I","family":"Wadman","sequence":"additional","affiliation":[{"name":"UMC Utrecht Brain Centre, Department of Neurology, University Medical Centre Utrecht, Utrecht University , 3584 CX Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W Ludo","family":"van der Pol","sequence":"additional","affiliation":[{"name":"UMC Utrecht Brain Centre, Department of Neurology, University Medical Centre Utrecht, Utrecht University , 3584 CX Utrecht , the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,12,9]]},"reference":[{"issue":"1","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/0092-8674(95)90460-3","article-title":"Identification and characterization of a spinal muscular atrophy-determining gene","volume":"80","author":"Lefebvre","year":"1995","journal-title":"Cell"},{"issue":"3","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"512","DOI":"10.1111\/ene.13534","article-title":"Muscle strength and motor function throughout life in a cross-sectional cohort of 180 patients with spinal muscular atrophy types 1c-4","volume":"25","author":"Wadman","year":"2018","journal-title":"Eur J Neurol"},{"issue":"5","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1001\/archneur.1995.00540290108025","article-title":"Natural history in proximal spinal muscular atrophy. Clinical analysis of 445 patients and suggestions for a modification of existing classifications","volume":"52","author":"Zerres","year":"1995","journal-title":"Arch Neurol"},{"issue":"1","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1097\/NEN.0000000000000144","article-title":"Spectrum of neuropathophysiology in spinal muscular atrophy type I","volume":"74","author":"Harding","year":"2015","journal-title":"J Neuropathol Exp Neurol"},{"issue":"1","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1002\/path.4080","article-title":"Synaptic defects in type I spinal muscular atrophy in human development","volume":"229","author":"Martinez-Hernandez","year":"2013","journal-title":"J Pathol"},{"issue":"20","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"2050","DOI":"10.1212\/WNL.0b013e3182749eca","article-title":"Dysfunction of the neuromuscular junction in spinal muscular atrophy types 2 and 3. Research support, non-U.S. Gov\u2019t","volume":"79","author":"Wadman","year":"2012","journal-title":"Neurology"},{"issue":"5","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1016\/S1474-4422(12)70061-3","article-title":"Childhood spinal muscular atrophy: Controversies and challenges. Review","volume":"11","author":"Mercuri","year":"2012","journal-title":"Lancet Neurol"},{"issue":"21","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"e2492","DOI":"10.1212\/WNL.0000000000007527","article-title":"Nusinersen in later-onset spinal muscular atrophy: Long-term results from the phase 1\/2 studies","volume":"92","author":"Darras","year":"2019","journal-title":"Neurology"},{"issue":"18","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"1723","DOI":"10.1056\/NEJMoa1702752","article-title":"Nusinersen versus sham control in infantile-onset spinal muscular atrophy","volume":"377","author":"Finkel","year":"2017","journal-title":"N Engl J Med"},{"issue":"7","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1056\/NEJMoa1710504","article-title":"Nusinersen versus sham control in later-onset spinal muscular atrophy","volume":"378","author":"Mercuri","year":"2018","journal-title":"N Engl J Med"},{"issue":"18","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"1713","DOI":"10.1056\/NEJMoa1706198","article-title":"Single-Dose gene-replacement therapy for spinal muscular atrophy","volume":"377","author":"Mendell","year":"2017","journal-title":"N Engl J Med"},{"issue":"10","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"915","DOI":"10.1056\/NEJMoa2009965","article-title":"Risdiplam in type 1 spinal muscular atrophy","volume":"384","author":"Baranello","year":"2021","journal-title":"N Engl J Med"},{"issue":"17","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"1853","DOI":"10.1007\/s40265-020-01410-z","article-title":"Risdiplam: First approval","volume":"80","author":"Dhillon","year":"2020","journal-title":"Drugs"},{"issue":"11","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"1166","DOI":"10.1136\/jnnp-2020-323822","article-title":"Nusinersen safety and effects on motor function in adult spinal muscular atrophy type 2 and 3","volume":"91","author":"Maggi","year":"2020","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"4","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/S1474-4422(20)30037-5","article-title":"Nusinersen in adults with 5q spinal muscular atrophy: A non-interventional, multicentre, observational cohort study","volume":"19","author":"Hagenacker","year":"2020","journal-title":"Lancet Neurol"},{"issue":"6","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"e845","DOI":"10.1212\/WNL.0000000000011230","article-title":"Correlates of fatigability in patients with spinal muscular atrophy","volume":"96","author":"Bartels","year":"2021","journal-title":"Neurology"},{"issue":"1","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"109","DOI":"10.3233\/JND-180342","article-title":"Perceived fatigue in spinal muscular atrophy: A pilot study","volume":"6","author":"Young S","year":"2019","journal-title":"J Neuromuscul Dis"},{"issue":"16","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"2552","DOI":"10.1093\/hmg\/ddn156","article-title":"Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy","volume":"17","author":"Kariya","year":"2008","journal-title":"Hum Mol Genet"},{"issue":"7","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"949","DOI":"10.1093\/hmg\/ddm367","article-title":"Selective vulnerability of motor neurons and dissociation of pre- and post-synaptic pathology at the neuromuscular junction in mouse models of spinal muscular atrophy","volume":"17","author":"Murray","year":"2008","journal-title":"Hum Mol Genet"},{"issue":"3","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1523\/JNEUROSCI.4434-08.2009","article-title":"Impaired synaptic vesicle release and immaturity of neuromuscular junctions in spinal muscular atrophy mice","volume":"29","author":"Kong","year":"2009","journal-title":"J Neurosci"},{"issue":"7","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"100346","DOI":"10.1016\/j.xcrm.2021.100346","article-title":"Spinal muscular atrophy: From approved therapies to future therapeutic targets for personalized medicine","volume":"2","author":"Chaytow","year":"2021","journal-title":"Cell Rep Med"},{"issue":"6","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"724","DOI":"10.1002\/mus.21319","article-title":"Acetylcholinesterase inhibitors in MG: To be or not to be?","volume":"39","author":"Punga","year":"2009","journal-title":"Muscle Nerve"},{"issue":"7","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"e019932","DOI":"10.1136\/bmjopen-2017-019932","article-title":"Protocol for a phase II, monocentre, double-blind, placebo-controlled, cross-over trial to assess efficacy of pyridostigmine in patients with spinal muscular atrophy types 2-4 (SPACE trial)","volume":"8","author":"Stam","year":"2018","journal-title":"BMJ Open"},{"issue":"1","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1186\/s13023-018-0904-5","article-title":"A continuous repetitive task to detect fatigability in spinal muscular atrophy","volume":"13","author":"Stam","year":"2018","journal-title":"Orphanet J Rare Dis"},{"issue":"7","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1016\/j.nmd.2005.03.004","article-title":"A motor function measure for neuromuscular diseases. Construction and validation study","volume":"15","author":"Berard","year":"2005","journal-title":"Neuromuscul Disord"},{"issue":"14","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"e1988","DOI":"10.1212\/WNL.0000000000010540","article-title":"Muscle strength and motor function in adolescents and adults with spinal muscular atrophy","volume":"95","author":"Wijngaarde","year":"2020","journal-title":"Neurology"},{"issue":"1","key":"2023022702535546700_","first-page":"71","article-title":"Determination of pyridostigmine bromide and its metabolites in biological samples","volume":"9","author":"Zhao","year":"2006","journal-title":"J Pharm Pharm Sci"},{"issue":"2","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1093\/toxsci\/kfh076","article-title":"Interactive effects of paraoxon and pyridostigmine on blood-brain barrier integrity and cholinergic toxicity","volume":"78","author":"Song","year":"2004","journal-title":"Toxicol Sci"},{"issue":"6","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"386","DOI":"10.5014\/ajot.39.6.386","article-title":"Adult norms for the box and block test of manual dexterity","volume":"39","author":"Mathiowetz","year":"1985","journal-title":"Am J Occup Ther"},{"issue":"1","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1007\/s11136-006-9109-9","article-title":"Minimally important change determined by a visual method integrating an anchor-based and a distribution-based approach","volume":"16","author":"de Vet","year":"2007","journal-title":"Qual Life Res"},{"issue":"1","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1186\/s13023-020-1348-2","article-title":"Fatigability in spinal muscular atrophy: Validity and reliability of endurance shuttle tests","volume":"15","author":"Bartels","year":"2020","journal-title":"Orphanet J Rare Dis"},{"issue":"1","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1186\/s12883-019-1244-3","article-title":"Assessment of fatigability in patients with spinal muscular atrophy: Development and content validity of a set of endurance tests","volume":"19","author":"Bartels","year":"2019","journal-title":"BMC Neurol"},{"key":"2023022702535546700_"},{"key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"1","DOI":"10.18637\/jss.v067.i01","article-title":"Fitting linear mixed-effects models using lme4","volume":"67","author":"Bates","year":"2015","journal-title":"J Stat Softw"},{"issue":"3","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1016\/j.ajhg.2009.08.002","article-title":"A positive modifier of spinal muscular atrophy in the SMN2 gene. Case reports research support, N.I.H., extramural research support. Non-U.S. Gov't","volume":"85","author":"Prior","year":"2009","journal-title":"Am J Hum Genet"},{"issue":"7","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"593","DOI":"10.1016\/j.nmd.2015.04.009","article-title":"209th ENMC international workshop: Outcome measures and clinical trial readiness in spinal muscular atrophy 7-9 November 2014, Heemskerk, The Netherlands","volume":"25","author":"Finkel","year":"2015","journal-title":"Neuromuscul Disord"},{"issue":"7","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1016\/S1474-4422(17)30085-6","article-title":"Safety and efficacy of olesoxime in patients with type 2 or non-ambulatory type 3 spinal muscular atrophy: A randomised, double-blind, placebo-controlled phase 2 trial","volume":"16","author":"Bertini","year":"2017","journal-title":"Lancet Neurol"},{"issue":"9","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"1774","DOI":"10.1097\/HJH.0000000000002932","article-title":"Pyridostigmine ameliorates preeclamptic features in pregnant rats by inhibiting tumour necrosis factor-alpha synthetsis and antagonizing tumour necrosis factor-alpha-related effects","volume":"39","author":"Zibrila A","year":"2021","journal-title":"J Hypertens"},{"issue":"B","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"108365","DOI":"10.1016\/j.intimp.2021.108365","article-title":"Acetylcholinesterase inhibition with pyridostigmine attenuates hypertension and neuroinflammation in the paraventricular nucleus in rat model for preeclampsia","volume":"101","author":"Zibrila A","year":"2021","journal-title":"Int Immunopharmacol"},{"key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"1301","DOI":"10.3389\/fimmu.2017.01301","article-title":"Add-on pyridostigmine enhances CD4(+) T-cell recovery in HIV-1-infected immunological non-responders: A proof-of-concept study","volume":"8","author":"Valdes-Ferrer","year":"2017","journal-title":"Front Immunol"},{"issue":"4","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1007\/s13365-019-00756-9","article-title":"The effect of pyridostigmine on small intestinal bacterial overgrowth (SIBO) and plasma inflammatory biomarkers in HIV-associated autonomic neuropathies","volume":"25","author":"Robinson-Papp","year":"2019","journal-title":"J Neurovirol"},{"issue":"10","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"2570","DOI":"10.1111\/j.1572-0241.2008.01871.x","article-title":"Enhanced cholinergic-mediated increase in the pro-inflammatory cytokine IL-6 in irritable bowel syndrome: Role of muscarinic receptors","volume":"103","author":"Dinan","year":"2008","journal-title":"Am J Gastroenterol"},{"issue":"8","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"R697","DOI":"10.1152\/ajpregu.00328.2015","article-title":"Increase in cholinergic modulation with pyridostigmine induces anti-inflammatory cell recruitment soon after acute myocardial infarction in rats","volume":"310","author":"Rocha","year":"2016","journal-title":"Am J Physiol Regul Integr Comp Physiol"},{"issue":"6","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1093\/ajh\/hpz036","article-title":"Chronic treatment with acetylcholinesterase inhibitors attenuates vascular dysfunction in spontaneously hypertensive rats","volume":"32","author":"Lataro","year":"2019","journal-title":"Am J Hypertens"},{"issue":"8","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"R908","DOI":"10.1152\/ajpregu.00102.2013","article-title":"Increase in parasympathetic tone by pyridostigmine prevents ventricular dysfunction during the onset of heart failure","volume":"305","author":"Lataro","year":"2013","journal-title":"Am J Physiol Regul Integr Comp Physiol"},{"key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"757","DOI":"10.1016\/j.ijcard.2016.10.061","article-title":"Impact of exercise training associated to pyridostigmine treatment on autonomic function and inflammatory profile after myocardial infarction in rats","volume":"227","author":"Feriani","year":"2017","journal-title":"Int J Cardiol"},{"issue":"8","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"e104476","DOI":"10.1371\/journal.pone.0104476","article-title":"Pyridostigmine restores cardiac autonomic balance after small myocardial infarction in mice","volume":"9","author":"Durand","year":"2014","journal-title":"PLoS One"},{"issue":"9","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"610","DOI":"10.1111\/1440-1681.12121","article-title":"Cholinergic stimulation with pyridostigmine improves autonomic function in infarcted rats","volume":"40","author":"de La Fuente","year":"2013","journal-title":"Clin Exp Pharmacol Physiol"},{"issue":"25","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"1797","DOI":"10.1056\/NEJM199406233302507","article-title":"Myasthenia gravis","volume":"330","author":"Drachman","year":"1994","journal-title":"N Engl J Med"},{"issue":"14","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"2325","DOI":"10.1093\/hmg\/ddaa116","article-title":"Effect of salbutamol on neuromuscular junction function and structure in a mouse model of DOK7 congenital myasthenia","volume":"29","author":"Webster","year":"2020","journal-title":"Hum Mol Genet"},{"issue":"10","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"863","DOI":"10.1016\/j.nmd.2018.07.013","article-title":"Does albuterol have an effect on neuromuscular junction dysfunction in spinal muscular atrophy?","volume":"28","author":"Pera","year":"2018","journal-title":"Neuromuscul Disord"},{"issue":"12","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"856","DOI":"10.1136\/jmg.2010.080366","article-title":"Salbutamol increases survival motor neuron (SMN) transcript levels in leucocytes of spinal muscular atrophy (SMA) patients: Relevance for clinical trial design","volume":"47","author":"Tiziano","year":"2010","journal-title":"J Med Genet"},{"issue":"5","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1136\/jmedgenet-2018-105482","article-title":"Longitudinal evaluation of SMN levels as biomarker for spinal muscular atrophy: Results of a phase IIb double-blind study of salbutamol","volume":"56","author":"Tiziano","year":"2019","journal-title":"J Med Genet"},{"key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"5858","DOI":"10.1007\/s00415-022-11231-7","article-title":"Amifampridine safety and efficacy in spinal muscular atrophy ambulatory patients: A randomized, placebo-controlled, crossover phase 2 trial","volume":"269","author":"Bonanno","year":"2022","journal-title":"J Neurol"},{"issue":"4","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1002\/mus.26633","article-title":"Nusinersen improves walking distance and reduces fatigue in later-onset spinal muscular atrophy","volume":"60","author":"Montes","year":"2019","journal-title":"Muscle Nerve"},{"issue":"4","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1016\/S1474-4422(20)30068-5","article-title":"Nusinersen in adults with spinal muscular atrophy: New challenges","volume":"19","author":"Mercuri","year":"2020","journal-title":"Lancet Neurol"},{"issue":"4","key":"2023022702535546700_","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1136\/jnnp-2016-314292","article-title":"Association of motor milestones, SMN2 copy and outcome in spinal muscular atrophy types 0-4","volume":"88","author":"Wadman","year":"2017","journal-title":"J Neurol Neurosurg Psychiatry"}],"container-title":["Brain Communications"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/braincomms\/advance-article-pdf\/doi\/10.1093\/braincomms\/fcac324\/47751437\/fcac324.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/braincomms\/article-pdf\/5\/1\/fcac324\/48606509\/fcac324.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/braincomms\/article-pdf\/5\/1\/fcac324\/48606509\/fcac324.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,3,16]],"date-time":"2023-03-16T12:43:20Z","timestamp":1678970600000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/braincomms\/article\/doi\/10.1093\/braincomms\/fcac324\/6884168"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,9]]},"references-count":57,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2022,12,29]]}},"URL":"https:\/\/doi.org\/10.1093\/braincomms\/fcac324","relation":{},"ISSN":["2632-1297"],"issn-type":[{"value":"2632-1297","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,2,1]]},"published":{"date-parts":[[2022,12,9]]},"article-number":"fcac324"}}