Bernard Brais

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
Scaravilli, A, Mari, G, Gabusi, I, Battocchio, M, Bosticardo, S, Schiavi, S et al.. An Investigation of Corticospinal Tract Microstructural Integrity in ARSACS Using a Profilometry MRI Analysis: Results From the PROSPAX Study. Eur J Neurol. 2025.32 (4)e70128 PMID:40241303
Chelban, V, Pellerin, D, Vijiaratnam, N, Lee, H, Goh, YY, Brown, L et al.. Intronic FGF14 GAA repeat expansions impact progression and survival in multiple system atrophy. Brain. 2025. PMID:40239008
Genís, D, Alemany, B, Pellerin, D, Brais, B, Dicaire, MJ, Volpini, V et al.. Late-onset vestibulocerebellar ataxia: clinical and genetic studies in a long follow-up series of 50 patients. J Neurol. 2025.272 (3)235 PMID:40024931
Chen, S, Ashton, C, Sakalla, R, Clement, G, Planel, S, Bonnet, C et al.. Involvement of the Superior Cerebellar Peduncles in GAA-FGF14 Ataxia. Neurol Genet. 2025.11 (2)e200253 PMID:39996128
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
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