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
Chukwuocha, I, Pellerin, D, Shanmugarajah, P, Tsironis, T, Foster, E, Hoggard, N et al.. Clinical characteristics, cerebellar MR spectroscopy and response to 3,4-diaminopyridine in spinocerebellar ataxia 27B: the Sheffield Ataxia Centre experience. J Neurol. 2025.272 (10)681 PMID:41065930
Iruzubieta, P, Pellerin, D, Ashton, C, Villa, F, Renaud, M, Dicaire, MJ et al.. Delineating the pathogenic threshold and phenotypic spectrum of SCA27B: findings from a large French-Canadian cohort. J Neurol. 2025.272 (9)636 PMID:40974444
Indelicato, E, Fleszar, Z, Pellerin, D, Nachbauer, W, Zuchner, S, Traschütz, A et al.. GAA-FGF14 Expansions and CACNA1A Variants: Phenotypic Overlap and Diagnostic Implications. Mov Disord. 2025. PMID:40879304
Di Folco, C, Dubec-Fleury, C, Traschütz, A, Kessler, C, Reich, S, Gagnon, C et al.. Spastic Ataxia Composite (SPAXCOM): A Scale to Evaluate the Progression of Subjects with Spasticity and Ataxia. Mov Disord. 2025. PMID:40832806
Olmedo-Saura, G, Iruzubieta, P, Bernal, S, Gonzalez-Quereda, L, Pont-Sunyer, C, Navalpotro-Gómez, I et al.. Expanding the clinical-radiological phenotype of SCA34 and its role in the differential diagnosis of the hot cross bun sign. J Neurol. 2025.272 (9)566 PMID:40788501
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