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

Matlawska, M, Ziora-Jakutowicz, K, Dicaire, MJ, Pera, J, Pellerin, D, Brais, B et al.. Identification of FGF14 GAA Expansions in Polish Patients with Undiagnosed Cerebellar Ataxia - A Preliminary Study. Cerebellum. 2026.25 (3) PMID:42096001

Iruzubieta, P, Brais, B, Pellerin, D. Expert commentary for "Episodic cerebellar ataxia mimicking stroke - diagnostic and therapeutic lessons from SCA27B". Parkinsonism Relat Disord. 2026. 108329 PMID:42055934

Fortin, J, Synofzik, M, Pedneault-Tremblay, ÉA, Hermle, D, Thieme, A, Timmann, D et al.. The Cerebellar Cognitive-Affective Syndrome Scale Reveals Consistent, Early, and Progressive Neuropsychological Deficits in Autosomal-Recessive Spastic Ataxia of Charlevoix-Saguenay: A Large International Cross-Sectional Study. Mov Disord. 2026.41 (4)1034-1040 PMID:41669957

Bustamante, ML, Miranda, M, Pellerin, D, Barreto, M, Silva, C, Miranda, AC et al.. Repeat Expansions in a Chilean Cohort with Adult-Onset Cerebellar Ataxia. Cerebellum. 2025.25 (1)3 PMID:41428128

Wong, AD, Airas, L, Alvarez, E, Antel, J, Araujo, D, Bernard, G et al.. Towards a neuroimaging consensus for the workup of adult genetic leukoencephalopathies on behalf of the White Matter Rounds Network: State of Practice. AJNR Am J Neuroradiol. 2025. PMID:41371978

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