Bernard Brais
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Investigator
Professor, Neurology and Human Genetics, McGill University
Neurologist and Director of the Rare Neurological Diseases Group, Montreal Neurological Institute and Hospital
NMD4C Involvement: Pillar 1: Preclinical Science, Pillar 2: Clinical Research, Theme 5: Open Science
Email BernardBiography
Bernard Brais, MDCM, MPhil, PhD, FRCP(C) is Full Professor of Neurology and Human Genetics at McGill University in Montreal. He is a trained neuromuscular neurologist, PhD in laboratory human genetics and historian of medicine. He is the director of the Montreal Neurological Institute’s Rare Neurological Diseases Group since 2011, and he is the director of McGill’s Adult Neurogenetics Fellowship.
His specialized practice is centered on the diagnosis and rehabilitation of rare often undiagnosed neurogenetic diseases, in particular: myopathies, ataxias, sensory neuropathies and leukoencephalopathies. Dr. Brais’ laboratory for the past 20 years has largely focussed on identifying new genes and mutations that cause hereditary diseases that are more prevalent in the French Canadian population of Quebec due to founder effects. He played a key role in the first identification of the genes responsible for: Oculopharyngeal Muscular Dystrophy (PABPN1, 1998), Hereditary sensory and autonomic neuropathy type II (HSN2/WINK1, 2004), Limb Girdle Muscular dystrophy LGMD2L-anoctamin 5 (ANO5, 2010), Posterior column ataxia and retinitis pigmentosa (FLVCR1, 2010), 0PolR3-related leukodystrophy (POLR3A and POLR3B, 2011), and congenital myopathy with fibre type disproportion (ZAK, 2017). Since 2007, he has been an international leader on collaborative research on Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS).
Recent Publications
Smith, IC, Sampaio, ML, Melkus, G, Meier-Ross, K, Chakraborty, S, Stotts, C et al.. Plasma-derived protein and imaging biomarkers distinguish disease severity in oculopharyngeal muscular dystrophy. J Neuromuscul Dis. 2024. 22143602241304990 PMID:39973404
Pellerin, D, Iruzubieta, P, Xu, IRL, Danzi, MC, Cortese, A, Synofzik, M et al.. Recent Advances in the Genetics of Ataxias: An Update on Novel Autosomal Dominant Repeat Expansions. Curr Neurol Neurosci Rep. 2025.25 (1)16 PMID:39820740
Livanos, I, Votsi, C, Michailidou, K, Pellerin, D, Brais, B, Zuchner, S et al.. The FGF14 GAA repeat expansion is a major cause of ataxia in the Cypriot population. Brain Commun. 2025.7 (1)fcae479 PMID:39801711
Côté, C, Brais, B, Batcho, CS, Brisson, JD, Youssof, S, Sogbossi, ES et al.. Measurement Properties of the Dysphagiameter for the Assessment of Dysphagia in Oculopharyngeal Muscular Dystrophy. Dysphagia. 2024. PMID:39708080
Türkdoğan, D, Smolina, N, Tekgül, Ş, Gül, T, Yeşilyurt, A, Houlden, H et al.. The First Case of Autosomal Recessive Cerebellar Ataxia with Prominent Paroxysmal Non-kinesigenic Dyskinesia Caused by a Truncating FGF14 Variant in a Turkish Patient. Mov Disord. 2025.40 (2)370-375 PMID:39704271
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