Sheffield Institute for
Translational Neuroscience

Professor Christopher McDermott MBChB FRCP PhD

Professor of Translational Neurology

Honorary Consultant Neurologist

Research Interests

The main thrust of Chris McDermott’s research programme is developing the evidence base for delivering supportive and symptomatic care for patients living with motor neuron disease. He is also interested in studying mechanisms of neurodegeneration, in order to develop treatments for patients with motor neuron disease and hereditary spastic paraplegia.

His current active projects include:

  • Investigating optimal respiratory support for patients with MND
  • Establishing an evidence base for nutritional support in MND
  • Designing and evaluating assistive technologies for patients with neuromuscular weakness
  • Developing and evaluating novel service delivery mechanisms for patients with long term neurological conditions
  • Genetic and phenotypic characterisation of motor system disorders
  • Natural history study of motor system disorders
  • Collaborating with pharmaceutical companies in Phase 1, 2 and 3 studies
  • Improving symptomatic management for patients with MND
  • Epidemiology of motor neuron disease
  • Models of neurodegeneration in motor system disorders


Current Projects

Principal Funding Sources:

  • Motor Neurone Disease Association, UK
  • Medical Research Council (MRC)
  • National Institute of Health Research (NIHR)
  • Joint Programme on Neurodegeneration (JPND)

Research Team

  • Dr Ceryl Harwood
  • Dr Esther Hobson
  • Dr Tom Jenkins
  • Hannah Hollinger
  • Helen Thacker
  • Susie Crawford
  • Theresa Walsh
  • Sue Baxter
  • Theocharis Stavroulakis
  • Dr Muhammad Rafiq


Representative Publications:

DiPALS Writing Committee; DiPALS Study Group Collaborators. Safety and efficacy of diaphragm pacing in patients with respiratory insufficiency due to amyotrophic lateral sclerosis (DiPALS): a multicentre, open-label, randomized controlled trial. Lancet Neurol. 2015 Sep;14(9):883-92. doi:10.1016/S1474-4422(15)00152-0. Epub 2015 Jul 30. PubMed PMID: 26234554.

Reed H, Langley J, Stanton A, Heron N, Clarke Z, Judge S, McCarthy A, Squire G, Quinn A, Wells O, Tindale W, Baxter S, Shaw PJ, McDermott CJ. Head-Up; An interdisciplinary, participatory and co-design process informing the development of a novel head and neck support for people living with progressive neck muscle weakness. J Med Eng Technol. 2014;39:404-10.

Rafiq MK, Lee E, Bradburn M, McDermott CJ, Shaw PJ. Effect of lipid profile on prognosis in the patients with amyotrophic lateral sclerosis: Insights from the olesoxime clinical trial. Amyotroph Lateral Scler Frontotemporal Degener. 2015 Dec;16(7-8):478-84. doi: 10.3109/21678421.2015.1062517. Epub 2015 Jul 10. PubMed PMID: 26161993.

Rafiq MK, Bradburn M, Proctor AR, Billings CG, Bianchi S, McDermott CJ, Shaw PJ. A preliminary randomized trial of the mechanical insufflator-exsufflator versus breath-stacking technique in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. 2015 Dec;16(7-8):448-55. doi:10.3109/21678421.2015.1051992. Epub 2015 Jul 3. PubMed PMID: 26140500.

ProGas Study Group. Gastrostomy in patients with amyotrophic lateral sclerosis (ProGas): a prospective cohort study. Lancet Neurol. 2015 Jul;14(7):702-9. doi:10.1016/S1474-4422(15)00104-0. Epub 2015 May 28. PubMed PMID: 26027943; PubMed Central PMCID: PMC4578147.

McGeachan AJ, Hobson EV, Shaw PJ, McDermott CJ. Developing an outcome measure for excessive saliva management in MND and an evaluation of saliva burden in Sheffield. Amyotroph Lateral Scler Frontotemporal Degener. 2015 Mar;16(1-2):108-13. doi: 10.3109/21678421.2014.951942. Epub 2014 Sep 16. PubMed PMID: 25225845.

Jenkins TM, Hollinger H, McDermott CJ. The evidence for symptomatic treatments in amyotrophic lateral sclerosis. Curr Opin Neurol. 2014 Oct;27(5):524-31. doi:10.1097/WCO.0000000000000135. Review. PubMed PMID: 25110934.

Stavroulakis T, Baird WO, Baxter SK, Walsh T, Shaw PJ, McDermott CJ. The impact of gastrostomy in motor neurone disease: challenges and benefits from a patient and carer perspective. BMJ Support Palliat Care. 2014 May 21. pii: bmjspcare-2013-000609. doi: 10.1136/bmjspcare-2013-000609. [Epub ahead of print] PubMed PMID: 24848262.

Stavroulakis T, Baird WO, Baxter SK, Walsh T, Shaw PJ, McDermott CJ. Factors influencing decision-making in relation to timing of gastrostomy insertion in patients with motor neurone disease. BMJ Support Palliat Care. 2014 Mar;4(1):57-63. doi: 10.1136/bmjspcare-2013-000497. Epub 2013 Dec 11. PubMed PMID: 24644772.

Da Costa MM1, Allen CE, Higginbottom A, Ramesh T, Shaw PJ, McDermott CJ. Dis Model Mech.A new zebrafish model produced by TILLING of SOD1-related amyotrophic lateral sclerosis replicates key features of the disease and represents a tool for in vivo therapeutic screening.Dis Model Mech. 2014 Jan;7(1):73-81. doi: 10.1242/dmm.012013. Epub 2013 Oct 2.

Baxter SK, Baird WO, Thompson S, Bianchi SM, Walters SJ, Lee E, Ahmedzai SH, Proctor A, Shaw PJ, McDermott CJ.The impact on the family carer of motor neurone disease and intervention with noninvasive ventilation. J Palliat Med. 2013 Dec;16(12):1602-9.

Baxter SK, Baird WO, Thompson S, Bianchi SM, Walters SJ, Lee E, Ahmedzai SH, Proctor A, Shaw PJ, McDermott CJ. The use of non-invasive ventilation at end of life in patients with motor neurone disease: A qualitative exploration of family carer and health professional experiences. Palliat Med. 2013 Jun;27(6):516-23. doi: 10.1177/0269216313478449.Epub 2013 Mar 5.

Kirby J, Highley JR, Cox L, Goodall EF, Hewitt C, Hartley JA, Hollinger HC, Fox M, Ince PG, McDermott CJ, Shaw PJ. Lack of unique neuropathology in amyotrophic lateral sclerosis associated with p.K54E angiogenin (ANG) mutation.  Neuropathol Appl Neurobiol. 2012 Dec 10. doi: 10.1111/nan.12007. [Epub ahead of print]

Tomi Aridegbe, Ros Kandler, Theresa Walsh, Pamela J Shaw, Christopher J McDermott. The natural history of motor neurone disease: assessing the impact of specialist care. Amyotroph Lateral Scler. 2013;14:13-19

Ismail A, Cooper-Knock J, Highley JR, Milano A, Kirby J, Goodall E, Lowe J, Scott I, Constantinescu CS, Walters SJ, Price S, McDermott CJ, Sawcer S, CompstonDA, Sharrack B, Shaw PJ. Concurrence of multiple sclerosis and amyotrophic lateral sclerosis in patients with hexanucleotide repeat expansions of C9ORF72. J Neurol Neurosurg Psychiatry. 2013;84:79-87

Baxter SK, Baird WO, Thompson S, Bianchi SM, Walters SJ, Lee E, Ahmedzai SH, Proctor A, Shaw PJ, McDermott CJ. The initiation of non-invasive ventilation for patients with motor neuron disease: Patient and carer perceptions of obstacles and outcomes.  Amyotroph Lateral Scler. 2012;14:105-110.

Stavroulakis T, Walsh T, Shaw PJ, McDermott CJ; (on behalf of the Progas Study). Gastrostomy use in motor neurone disease (MND): A review, meta-analysis and survey of current practice. Amyotroph Lateral Scler. 2012;14:96-104.

McDermott C J, Maguire C, Cooper C L, Ackroyd R, Baird W O, Baudouin S, Bentley A, Bianchi S, Bourke S, Bradburn M J, Dixon S, Ealing J, Galloway S, Karat D, Maynard N, Morrison K, Mustfa N, Stradling J, Talbot K, Williams T and Shaw P J. Protocol for Diaphragm Pacing in patients with respiratory muscle weakness due to Motor Neurone Disease (DiPALS): a randomised controlled trial [Trial Registration: Current controlled trials ISRCTN53817913].  BMC Neurology. 2012;12:74

Muhammad K Rafiq, Michael Bradburn, Alison R Proctor, Catherine Billings, Stephen Bianchi, Christopher J McDermott, Pamela J Shaw. Using transcutaneous carbon dioxide monitor (TOSCA 500) to detect respiratory failure in patients with Amyotrophic Lateral Sclerosis: a validation study. Amyotroph Lateral Scler. 2012;13:528-532

Muhammad Khizar Rafiq, Alison Ruth Proctor, Christopher J McDermott, Pamela J Shaw. Respiratory management of motor neurone disease: a review of current practice and new developments. Practical Neurology. 2012;12:166-176.

Cooper-Knock J, Hewitt C, Highley JR, Brockington A, Milano A, Man S, McDermott CJ, Shaw PJ. Clinico-pathological features in amyotrophic lateral sclerosis with expansions in C9ORF72. Brain. 2012;135:751-764.

O'Neill CL, Williams TL, Peel ET, McDermott CJ, Shaw PJ, Gibson GJ, Bourke SC. Non-invasive ventilation in motor neuron disease: an update of current UK practice.  J Neurol Neurosurg Psychiatry. 2012;83:371-376

Al-Chalabi A, Shaw PJ, Young CA, Morrison KE, Murphy C, Thornhill M, . . . Leigh, PN. Protocol for a double-blind randomised placebo-controlled trial of lithium carbonate in patients with amyotrophic Lateral Sclerosis (LiCALS) [Eudract number: 2008-006891-31]. BMC Neurology. 2011;11. doi:10.1186/1471-2377-11-111

Chavada G, El-Nayal A, Lee F, Webber SJ, McAlindon M, Walsh T, Hollinger H, McDermott CJ, Shaw PJ. Evaluation of two different methods for per-oral gastrostomy tube placement in patients with motor neuron disease (MND): PIG versus PEG procedures.  Amyotroph Lateral Scler. 2010;11:531-536.

Hervé Besson, Ceryl A Harwood, Ulf Ekelund, Francis M Finucane, Christopher J McDermott, Pamela J Shaw, Nicholas J Wareham. Validation of the historical adulthood physical activity questionnaire (HAPAQ) against objective measurements of physical activity.  Int J Behav Nutr Phys Act. 2010;7:54-63.

Christopher Hewitt, Janine Kirby, J. Robin Highley, Judith A. Hartley; Rachael Hibberd, Hannah C. Hollinger, Tim L. Williams, Paul G. Ince, Christopher J. McDermott, Pamela J. Shaw. Novel FUS/TLS Mutations and Pathology in Familial and Sporadic Amyotrophic Lateral Sclerosis. Arch Neurol. 2010;67:455-461.

C Douglass, R Kandler, PJ Shaw and CJ McDermott. An evaluation of neurophysiological criteria used in the diagnosis of Motor Neurone Disease.  J Neurol Neurosurg Neuropsych 2010;81:646-9.

J Kirby, E Goodall W Smith, J R Highley, R Masanzu, JA Hartley, R Hibberd, HC Hollinger, SB Wharton, K Morrison, PG Ince, CJ McDermott and PJ Shaw. Broad clinical phenotypes associated with TAR-DNA binding protein (TARDBP) mutations in amyotrophic lateral sclerosis. Neurogenetics. 2010 May;11(2):217-25. doi: 10.1007/s10048-009-0218-9. Epub 2009 Sep 17.

RMC Trial Group. Randomised controlled trial of methotrexate for chronic inflammatory demyelinating polyradiculoneuropathy (RMC trial): a pilot, multicentre study. Lancet Neurol. 2009;8:158-64.

C Hewamadduma, C McDermott, J Kirby, A Grierson, M Panayi, A Dalton, Y Rajabally, P Shaw. New pedigrees and novel mutation expand the phenotype of REEP1-associated hereditary spastic paraplegia (HSP).  Neurogenetics. 2009;10:105-8

PR Kasher, KJ De Vos, SB Wharton, C Manser, EJ Bennett, M Bingley, JD Wood, R Milner, CJ McDermott, CC Miller, PJ Shaw, AJ Grierson. Direct evidence for axonal transport defects in a novel mouse model of mutant spastin-induced hereditary spastic paraplegia (HSP) and human HSP patients.  J Neurochem 2009;110:34-44.

CA Harwood, CJ McDermott, PJ Shaw. Physical activity as an exogenous risk factor in motor neuron disease (MND): A review of the evidence. Amyotroph Lateral Sclerosis 2009;10:191-204

CJ McDermott and PJ Shaw. Diagnosis and management of motor neurone disease.  British Medical Journal 336:658-662;2008.

AA Hewamadduma, J Kirby, C Kershaw, Ann Dalton. CJ McDermott, PJ Shaw. HSP60 is a rare cause of hereditary spastic paraparesis, but may act as a genetic modifier.  Neurology 2008;170:1717-1718

C Beetz, S Zucher, A Ashgley-Koch, M Auer-Grumbach, P Byrne, PF Chinnery, M Hutchinson, CJ McDermott, IA Meyer, AOH Nygren, M Pericak-Vance, A Pyle, GA Rouleau, J Schickel, PJ Shaw, T Deufel. Linkage to a known gene, but no mutation identified: comprehensive re-analysis of SPG4 HSP pedigrees reveals large deletions as the sole cause. Human Mutation 28:739-40,2007.

JD Wood, JA Landers, M Bingley, CJ McDermott, V Thomas McArthur, LJ Gleadall, PJ Shaw, VT Cunliffe. The microtubule severing protein spastin is required for motor axon outgrowth in the zebrafish embryo. Human Molecular Genetics 15:2763-2771;2006.

CJ McDermott, CE Burness, J Kirby, LE Cox, G Rao, M Durkie, B Sharrack, M Hadjivassiliou, PF Chinnery, A Dalton, PJ Shaw on behalf of the UK and Irish HSP Consortium. Clinical features of hereditary spastic paraparesis due to spastin mutation. Neurology 67:45-51;2006

CJ McDermott, PJ Shaw. Hereditary spastic paraplegia.  In : Handbook of Handbook of Clinical Neurology Series 3rd Edition. Motor Neuron Disorders. Elsevier. Editors Andrew Eisen, Pamela J Shaw. Chapter 17:327-352, 2006.

CJ McDermott, AJ Grierson, JD Wood, M Bingley, KDM Bushby, PJ Shaw. Hereditary spastic paraparesis: evidence of disrupted intracellular transport associated with spastin mutation.  Ann Neurol 54:748-759;2003.

CJ McDermott, RW Taylor, C Hayes, M Johnson, KMD Bushby, DM Turnbull, PJ Shaw. Investigation of mitochondrial function in hereditary spastic paraparesis.  NeuroReport 14:485-488; 2003.

CJ McDermott, D Roberts, J Tomkins, KMD Bushby, PJ Shaw. Spastin and paraplegin analysis in selected cases of motor neuron disease with prominent upper motor neuron degeneration.  ALS and related Motor Neuron Disorders 11:1-4;2003.

CJ McDermott, PJ Shaw. Hereditary spastic paraparesis.  In: Blue Book of Practical Neurology. Editors PJ Shaw and M Strong. Chapter 18, 2003, pp 435-464. Butterworth Heinemann, Philadelphia.

CJ McDermott, PJ Shaw. Hereditary spastic paraparesis.  In: International Review of Neurobiology 53:191-204;2002: Mitochondrial Disorders of the Nervous System Editor A Schapira.
Update on motor neurone disease. CJ McDermott, PJ Shaw. Update 662-667;2002.

J Tomkins, SJ Banner, CJ McDermott, PJ Shaw. Mutation screening of manganese superoxide dismutase (SOD2) in amyotrophic lateral sclerosis (ALS).  NeuroReport 12:1-4; 2001.

CJ McDermott, RK Dayaratre, J Tomkins, MA Johnson, G Casari, DM Turnbull, KMD Bushby, PJ Shaw. Paraplegin gene analysis in hereditary spastic paraparesis pedigrees in the North East of England. Neurology 56:467-471;2001.

JC Lindsey, ME Lusher, CJ McDermott, KD White, E Reid, DC Rubinztein, R Bashir, PJ Shaw, KMD Bushby. Mutation analysis of the spastin gene in patients with hereditary spastic paraparesis.  J Med Genet 37:759-765;2000.

CJ McDermott, KD White, K Bushby, PJ Shaw. Hereditary spastic paraparesis: a review of new developments.  J Neurol Neurosurg Psychiatry 69:150-160;2000.

Professional Activities

External Commitments:

  • Member of DeNDRoN MND CSG
  • Neurology Lead Clinician, STH NHS FT
  • NICE Clinical Guideline Development Group Member
  • NIHR RfPB panel member
  • Yorkshire & Humber Strategic Clinical Network's Neurology Clinical Expert Group

 Invited lectures:

  • MND Care Centre Directors Meeting, Stratford 2014
  • Oxford MND Centre 2014
  • North West MND Network Meeting 2014
  • St Christopher Hospice, London 2014
  • Walton Centre, Liverpool, 2013
  • Kings College, London, 2013
  • ENCALS, Sheffield 2013
  • Euan McDonald MND Centre 2012
  • Home Mechanical Ventilation Meeting, St Thomas Hospital, London, 2012
  • Association of British Neurologists, Bournemouth 2010
  • Familial Spastic Paraplegia Support Group UK 2008
  • European Neurological Society, Rhodes 2007


Professor of Translational Neuroscience.

Reader in Neurology.

Clinical Senior Lecturer in Neurology.

Specialist Training in Neurology (CCT), Sheffield.

Wellcome Trust Research Training Fellowship (PhD), University of Sheffield.

Clinical Research Fellow, University of Newcastle upon Tyne.

Specialist Training in Neurology, Leeds.

General professional training (MRCP), Leeds Teaching Hospitals.

Medicine (MBChB), University of Leeds.

Contact Details

University of Sheffield
Faculty of Medicine, Dentistry & Health
Department of Neuroscience
Sheffield Institute for Translational Neuroscience
385a Glossop Road
United Kingdom, S10 2HQ

T: +44 (0)114 22 22261
F: +44 (0)114 22 22290

Secretary: Rebecca Crossland
T: +44 (0)114 22 22261