Faculty profile

Bernard Brais

ResearcherNeurology and NeurosurgeryMcGill University

This profile is built from public research funding records (CIHR, NSERC and SSHRC) and PubMed. We have not imported them from a McGill University directory, so their courses may be missing. Find their university profile.

Bernard Brais

Researcher

Neurology and Neurosurgery

Health Sciences

McGill University

Montreal, Quebec, Canada

Supervision signals

Has led research grants, which requires an independent research appointment. Ask their department whether they supervise graduate students.
98 publications in the last five years, latest in 2026.
Senior (last) author on 8 recent papers, a sign of leading a research group.
Principal investigator on 5 research grants (5 CIHR), most recently in 2023.
Supervisor on 1 trainee award (1 CIHR), most recently in 2012.

These signals come from public records, including CIHR, NSERC and SSHRC funding decisions. Ask the professor or the graduate program whether they are taking new students.

Academic metrics

179

Publications

Citations

Collaborators

Grants

Publications by year

Counts cover the publications linked to this profile. Citations and the h-index appear when the source records them.

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Signals and details

Research

Ataxia Proteomics Transcriptomics Natural History Aging Cerebellum Chanelopathy Gait

Latest papers

Longitudinal progression, metrics, age-dependence, and modifiers of ataxia severity in SCA27B: a multicentre study of 219 patients.
EBioMedicine · 2026
Muscle Strength, Balance, and Indoor Mobility in Oculopharyngeal Muscular Dystrophy: An Exploratory Canadian Multicenter Study.
Muscle & nerve · 2026
Establishment and characterization of three human pluripotent stem cell lines from patients with spinocerebellar ataxia 27B (SCA27B).
Stem cell research · 2026

All 179 publications

Latest funding

Characterization of the newly uncovered internationally prevalent GAA triplet expansion late-onset SCA27B ataxia that is more common in Quebec: from improved phenotyping, genotyping to molecular pathological understanding
CIHR · 2023 · Nominated PI
$367,200
Designing a toolbox of paradigmatic treatments for a targeted molecular medicine approach to autosomal-recessive ataxias
CIHR · 2020 · Nominated PI
$225,000
PROSPAX: an integrated multimodal progression chart in spastic ataxias
CIHR · 2019 · Nominated PI
$224,436

All 8 grants and awards

From the 150 most recent of 179 publications.

Longitudinal progression, metrics, age-dependence, and modifiers of ataxia severity in SCA27B: a multicentre study of 219 patients.

Traschütz A, Hilgers RD, Erdlenbruch F, Depienne C, Wirth T, Delvallée C, Nümann A, Ashton C, Pellerin D, Indelicato E, Heindl F, Renaud M, Borsche M, Grobe-Einsler M, Faber J, Klockgether T, Schöls L, Brais B, Anheim M, Timmann D, Synofzik M

EBioMedicine2026

DOI: 10.1016/j.ebiom.2026.106437 PMID: 42603514

Muscle Strength, Balance, and Indoor Mobility in Oculopharyngeal Muscular Dystrophy: An Exploratory Canadian Multicenter Study.

Bélair N, Brisson JD, Brais B, Rodrigue X, Lochmüller H, Hébert LJ, MacGregor J, Dennison B, Lessard I, Côté I, Duchesne E, Gagnon C

Muscle & nerve2026

DOI: 10.1002/mus.70343 PMID: 42439050

Establishment and characterization of three human pluripotent stem cell lines from patients with spinocerebellar ataxia 27B (SCA27B).

Pellerin D, Rebelo A, Zafeer MF, Ditmeyer M, Iruzubieta P, Van de Vondel L, Rodriguez Y, Jacobs EH, Dicaire MJ, Danzi MC, Napierala M, Saporta M, Brais B, Zuchner S

Stem cell research2026

DOI: 10.1016/j.scr.2026.104055 PMID: 42391783

Establishment and characterization of two human pluripotent stem cell lines from patients with ATX-FGF14/spinocerebellar ataxia 27A (SCA27A).

Pellerin D, Rebelo A, Zafeer MF, Iruzubieta P, Türkdogan D, Smolina N, Tekgül Ş, Ditmeyer M, Van de Vondel L, Rodriguez Y, Jacobs EH, Gül-Demirkale T, Yesilyurt A, Dicaire MJ, Danzi MC, Brais B, Napierala M, Başak AN, Zuchner S

Stem cell research2026

DOI: 10.1016/j.scr.2026.104050 PMID: 42372627

Expert commentary for "Episodic cerebellar ataxia mimicking stroke - diagnostic and therapeutic lessons from SCA27B".

Iruzubieta P, Brais B, Pellerin D

Parkinsonism & related disorders2026

DOI: 10.1016/j.parkreldis.2026.108329 PMID: 42055934

Cerebellar cognitive affective syndrome (CCAS) and [18F]-FDG PET findings in spinocerebellar ataxia type SCA27B.

Cros L, Pellerin D, Palpacuer T, Puisieux S, Frismand S, Hocquel A, Wandzel M, Roth V, Pourié C, Brais B, Bonnet C, Meyer M, Dillier C, Jardel A, Hopes L, Verger A, Renaud M, Clément G

Journal of neurology2026

DOI: 10.1007/s00415-026-14015-5 PMID: 42481725

Toward a Neuroimaging Consensus for the Work-up of Adult Genetic Leukoencephalopathies on Behalf of the White Matter Rounds Network: State of Practice.

Wong AD, Airas L, Alvarez E, Antel J, Araujo D, Bernard G, Boudjani H, Brais B, Cocozza S, Corboy JR, Fadda G, Imitola J, Lacasse MC, Longbrake EE, Macaron G, Narayanan S, Orthmann-Murphy J, Ortiz J, Shor N, Uggetti C, Venkateswaran S, Wilson N, Miller E, La Piana R, White Matter (WM) Rounds Network

AJNR. American journal of neuroradiology2026

DOI: 10.3174/ajnr.a9127 PMID: 41371978

Frequency and phenotype of GAA-FGF14 disease in bilateral vestibulopathy syndromes: insights from repeat expansion carriers, including a case of co-occurrence with RFC1-related CANVAS.

Pellerin D, Heindl F, Traschütz A, Iruzubieta P, Dicaire MJ, Zuchner S, Hartmann AM, Rujescu D, Houlden H, Brais B, Strupp M, Synofzik M

Journal of neurology2026

DOI: 10.1007/s00415-026-13867-1 PMID: 42178418

Identification of FGF14 GAA Expansions in Polish Patients with Undiagnosed Cerebellar Ataxia - A Preliminary Study.

Matlawska M, Ziora-Jakutowicz K, Dicaire MJ, Pera J, Pellerin D, Brais B, Iruzubieta P, Elert-Dobkowska E, Sulek A

Cerebellum (London, England)2026

DOI: 10.1007/s12311-026-02003-4 PMID: 42096001

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.

Fortin J, Synofzik M, Pedneault-Tremblay ÉA, Hermle D, Thieme A, Timmann D, La Piana R, Brais B, Dolbec J, Côté I, Gagnon C, Traschütz A

Movement disorders : official journal of the Movement Disorder Society2026

DOI: 10.1002/mds.70201 PMID: 41669957

Characterization of the newly uncovered internationally prevalent GAA triplet expansion late-onset SCA27B ataxia that is more common in Quebec: from improved phenotyping, genotyping to molecular pathological understanding

Nominated PICIHR2023Project Grant

Principal investigators: Brais, Bernard

Keywords: Aging; Ataxia; Cerebellum; Chanelopathy; Gait; Neurodegeneration

$367,200

Designing a toolbox of paradigmatic treatments for a targeted molecular medicine approach to autosomal-recessive ataxias

Nominated PICIHR2020Team Grant: E-Rare Joint Transnational Call

Principal investigators: Brais, Bernard

Keywords: Ataxia; Biomarker Discovery; Biomarker Validation; Drug Development; Drug Validation; Proteomics; Transcriptomics; Transgenic Mice Models

$225,000

PROSPAX: an integrated multimodal progression chart in spastic ataxias

Nominated PICIHR2019Team Grant: E-Rare Joint Transnational Call

Principal investigators: Brais, Bernard

Keywords: Ataxia; Clinical Scales; Digital Biomarkers; Genetics; Molecular Biomarkers; Natural History; Neuromuscular Diseases; Proteomics; Registry; Transcriptomics

$224,436

POLR3-related leukodystrophy: From bench to bedside

Co-investigatorCIHR2015Operating Grant

Principal investigators: Bernard, Geneviève

Keywords: 4h Leukodystrophy; Hypomyelination; Magnetic Resonance Imaging; Mouse Model; Natural History Study; Next Generation Sequencing; Pathophysiological Mechanisms; Polr3-Related Leukodystrophy; Polymerase Iii; Rna Sequenging

$100,000

PREPARE: Preparing for therapies in autosomal recessive ataxias

Nominated PICIHR2015Team Grant: E-Rare Joint Transnational Call

Principal investigators: Brais, Bernard

Keywords: Ataxia; Exomes; International Collaboration; Longitudinal Study; Natural History; Preclinical Trial; Rare Disease; Register; Transgenic Mice

Characterization of the Pol III-related hypomyelinating leukodystrophies

SupervisorCIHR2012Master's Award: Canada Graduate Scholarships

Principal investigators: Choquet, Karine

Keywords: 4h Syndrome; Expression Rna Chips; Knock-Down; Leukodystrophie; Myelin; Polr3a; Polr3b; Rna Polymerase Iii; Sequencing; Transcription Efficiency

$17,500

Clinical, molecular and pathophysiological characterization of Pol III-related leukodystrophies

Co-investigatorCIHR2012Operating Grant

Principal investigators: Bernard, Geneviève

Keywords: Clinical Characterization; Exome Sequencing; Hypomyelination; Magnetic Resonance Imaging; Mouse Model; Pathophysiological Mechanisms; Pol Iii-Related Leukodystrophies; Polymerase Iii; Rna Expression Array

$453,883

New emerging team on Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS): from models to treatment strategies

Nominated PICIHR2011Emerging Team Grant: Rare Diseases - Basic/Clinical

Principal investigators: Brais, Bernard; Gagnon, Cynthia; Gehring, Kalle B; Mathieu, Jean; McBride, Heidi M; Mckinney, Rebecca A; McPherson, Peter S; Shoubridge, Eric A; Young, Jason C

Keywords: Ataxia; Autosomal Recessive Spatique Ataxia Of Cherlevoix-Saguenay; Chaperone; Drug Screening; French-Canadian Population; Mitochondria; Orphan Disease; Structural Biology; Transgenic Knockout Mice

$1,250,000

From CIHR, NSERC and SSHRC funding decisions: CIHR since 2008, NSERC since 1991 and SSHRC since 1998, including their latest published competition results.

Write to this professor

A short, specific email works best. This draft uses one of their recent papers; replace the parts in brackets with your own details before sending.

Applying for

Paper to mentionMention their CIHR grant from 2023

Subject: Prospective PhD student: Ataxia Dear Professor Brais, My name is [your name], and I am a [your current degree or role] at [your institution]. I am applying for a PhD position starting in [term and year]. I read your paper "Longitudinal progression, metrics, age-dependence, and modifiers of ataxia severity in SCA27B: a multicentre study of 219 patients." (2026), and it connects closely to my own work on [your research topic]. [One or two sentences on what you did and what you learned.] I also saw your CIHR-funded project "Characterization of the newly uncovered internationally prevalent GAA triplet expansion late-onset SCA27B ataxia that is more common in Quebec: from improved phenotyping, genotyping to molecular pathological understanding" (2023), and I would be glad to contribute to work in that direction. Are you accepting new PhD students for [term and year]? I have attached my CV and transcripts, and I would be glad to talk at a time that suits you. Thank you for your time. Kind regards, [your name]

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Bernard Brais

Research

Latest papers

Latest funding

Longitudinal progression, metrics, age-dependence, and modifiers of ataxia severity in SCA27B: a multicentre study of 219 patients.

Muscle Strength, Balance, and Indoor Mobility in Oculopharyngeal Muscular Dystrophy: An Exploratory Canadian Multicenter Study.

Establishment and characterization of three human pluripotent stem cell lines from patients with spinocerebellar ataxia 27B (SCA27B).

Establishment and characterization of two human pluripotent stem cell lines from patients with ATX-FGF14/spinocerebellar ataxia 27A (SCA27A).

Expert commentary for "Episodic cerebellar ataxia mimicking stroke - diagnostic and therapeutic lessons from SCA27B".

Cerebellar cognitive affective syndrome (CCAS) and [18F]-FDG PET findings in spinocerebellar ataxia type SCA27B.

Toward a Neuroimaging Consensus for the Work-up of Adult Genetic Leukoencephalopathies on Behalf of the White Matter Rounds Network: State of Practice.

Frequency and phenotype of GAA-FGF14 disease in bilateral vestibulopathy syndromes: insights from repeat expansion carriers, including a case of co-occurrence with RFC1-related CANVAS.

Identification of FGF14 GAA Expansions in Polish Patients with Undiagnosed Cerebellar Ataxia - A Preliminary Study.

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.

Frequent collaborators

Cynthia Gagnon

Roberta La Piana

Cynthia Gagnon

Bernard Brais

Jodi Warman Chardon

Jacek Majewski

Antoine Duquette

Kalle Gehring

Gerd Melkus

Guy Rouleau

Jason Karamchandani

Elise Duchesne

Bernard Brais

Jason Karamchandani

Bernard Brais

Lawrence Korngut

John Woulfe

Daniela Pohl

Maryam Oskoui

Gerald Pfeffer

Taila Hartley

Elsa Rossignol

Craig Campbell

Angela Genge

Krista Best

Geneviève Bernard

Mark Tarnopolsky

Jack Antel

Mary-Ellen Harper

Constantin Polychronakos

Anne-Marie Laberge

Siegfried Hekimi

Andrea Richter

Alex Parker

Kevin Petrecca