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

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. 2024. PMID:39704271

Clément, G, Puisieux, S, Ashton, C, Pellerin, D, Danzi, MC, Bonnet, C et al.. Oculomotor findings in spinocerebellar ataxia 27B: a case series. Can J Neurol Sci. 2024. 1-6 PMID:39648743

Alshimemeri, S, Alsaghan, L, Alsamh, DA, Zhou, L, Furtado, S, Kraft, S et al.. Rare Spinocerebellar Ataxia Types in Canada: A Case Series and Review of the Literature. Can J Neurol Sci. 2024. 1-10 PMID:39618416

Gerhart, BJ, Pellerin, D, Danzi, MC, Zuchner, S, Brais, B, Matos-Rodrigues, G et al.. Assessment of the Clinical Interactions of GAA Repeat Expansions in FGF14 and FXN. Neurol Genet. 2024.10 (6)e200210 PMID:39574782

Koutsis, G, Kartanou, C, Kontogeorgiou, Z, Koniari, C, Mitrousias, A, Pellerin, D et al.. Screening for SCA27B, CANVAS and other repeat expansion disorders in Greek patients with late-onset cerebellar ataxia suggests a need to update current diagnostic algorithms. J Neurol Sci. 2024.467 123309 PMID:39571249

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