Bernard Brais

Bernard Brais profile picture

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

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Biography

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

Warman-Chardon, J, Smith, IC, Brais, B. Oculopharyngeal Muscular Dystrophy Is Not Responsive to Immunosuppressant Treatment. J Clin Neuromuscul Dis. 2025.26 (4)i PMID:40513069

Yau, WY, Sullivan, R, O'Connor, E, Pellerin, D, Parkinson, MH, Giunti, P et al.. Diagnostic yield and limitations of whole-genome sequencing for hereditary cerebellar ataxia. Brain Commun. 2025.7 (3)fcaf188 PMID:40488180

Lessard, I, Hébert, LJ, Brais, B, Duchesne, E, Rodrigue, X, Brisson, JD et al.. Selection of Clinical Outcome Assessments for Trial Readiness in ARSACS - 2-year Progression and Responsiveness to Change Part 1: Disease Severity, Swallowing, Upper Limb Function, and Participation. Cerebellum. 2025.24 (4)106 PMID:40450178

Lessard, I, Duchesne, E, Hébert, LJ, Brais, B, Rodrigue, X, Routhier, F et al.. Selection of Clinical Outcome Assessments for Trial Readiness in ARSACS - 2-year Progression and Responsiveness to Change Part 2: Mobility, Balance, and Lower Limb Coordination. Cerebellum. 2025.24 (4)95 PMID:40332679

Beijer, D, Mengel, D, Önder, D, Wilke, C, Traschütz, A, Faber, J et al.. The genetic landscape of sporadic adult-onset degenerative ataxia: a multi-modal genetic study of 377 consecutive patients from the longitudinal multi-centre SPORTAX cohort. EBioMedicine. 2025.115 105715 PMID:40273470

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