Article

Broad, Rebecca J, Gabel, Matt C, Dowell, Nicholas G, Schwartzman, David J, Seth, Anil K, Zhang, Hui, Alexander, Daniel C, Cercignani, Mara and Leigh, P Nigel (2018) Neurite orientation and dispersion density imaging (NODDI) detects cortical and corticospinal tract degeneration in ALS. Journal of Neurology, Neurosurgery and Psychiatry. ISSN 0022-3050

Chen, Jason A, Chen, Zhongbo, Won, Hyejung, Huang, Alden Y, Lowe, Jennifer K, Wojita, Kevin, Yokoyama, Jennifer S, Bensimon, Gilbert, Leigh, P Nigel, Payan, Christine, Shatunov, Aleksey, Jones, Ashley R, Lewis, Cathryn M, Deloukas, Panagiotis, Amouyel, Philippe, Tzourio, Christophe, Dartigues, Jean-Francois, Ludolph, Albert, Boxer, Adam L, Bronstein, Jeff M, Al-Chalabi, Ammar, Geschwind, Daniel H and Coppola, Giovanni (2018) Joint genome-wide association study of progressive supranuclear palsy identifies novel susceptibility loci and genetic correlation to neurodegenerative diseases. Molecular Neurodegeneration, 13 (41). pp. 1-11. ISSN 1750-1326

Jabbari, E, Woodside, J, Tan, Mmx, Shoai, M, Pittman, A, Ferrari, R, Mok, K Y, Zhang, D, Reynolds, R H, de Silva, R, Grimm, M J, Respondek, G, Müller, U, Al-Sarraj, S, Gentleman, S M, Lees, A J, Warner, T T, Hardy, J, Revesz, T, Höglinger, G U, Holton, J L, Ryten, M, Morris, H R, Leigh, PN and PROSPECT-UK consortium, (2018) Variation at the TRIM11 locus modifies Progressive Supranuclear Palsy phenotype. Annals of neurology. ISSN 1531-8249

Fang, Ton, Al Khleifat, Ahmed, Meurgey, Jacques-Henri, Jones, Ashley, Leigh, Nigel, Bensimon, Gilbert and Al-Chalabi, Ammar (2018) Stage at which riluzole treatment prolongs survival in patients with amyotrophic lateral sclerosis: a retrospective analysis of data from a dose-ranging study. Lancet Neurology, 17 (5). pp. 416-422. ISSN 1474-4422

van Eijk, Ruben P A, Jones, Ashley R, Sproviero, William, Shatunov, Aleksey, Shaw, Pamela J, Leigh, Nigel, Young, Carolyn A, Shaw, Christopher E, Mora, Gabriele, Mandrioli, Jessica, Borghero, Giuseppe, Volanti, Paolo, Diekstra, Frank P, van Rheenen, Wouter, Verstraete, Esther, Eijkemans, Marinus J C, Veldink, Jan H, Chio, Adriano, Al-Chalabi, Ammar, van den Berg, Leonard H, van Es, Michael A and Unset (2017) Meta-analysis of pharmacogenetic interactions in amyotrophic lateral sclerosis clinical trials. Neurology, 89 (18). pp. 1915-1922. ISSN 1526-632X

Barritt, Andrew W, Anderson, Stuart J, Leigh, P Nigel and Ridha, Basil H (2017) Late-onset Tay-Sachs disease. Practical Neurology, 17 (5). pp. 396-399. ISSN 1474-7766

Martin, Sarah, Trevor-Jones, Emma, Khan, Sabyha, Shaw, Keelan, Marchment, Deepti, Kulka, Anna, Ellis, Catherine E, Burman, Rachel, Turner, Martin R, Carroll, Liam, Mursaleen, Leah, Leigh, P Nigel, Shaw, Christopher E, Pearce, Neil, Stahl, Daniel and Al-Chalabi, Ammar (2017) The benefit of evolving multidisciplinary care in ALS: a diagnostic cohort survival comparison. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration. pp. 1-7. ISSN 2167-9223

Glasmacher, Stella Andrea, Leigh, Nigel and Saha, Romi Anirban (2017) Predictors of survival in progressive supranuclear palsy and multiple system atrophy: a systematic review and meta-analysis. Journal of Neurology, Neurosurgery and Psychiatry, 88 (5). pp. 402-411. ISSN 1468-330X

Smith, Bradley N, Topp, Simon D, Fallini, Claudia, Shibata, Hideki, Chen, Han-Jou, Troakes, Claire, King, Andrew, Ticozzi, Nicola, Kenna, Kevin P, Soragia-Gkazi, Athina, Miller, Jack W, Sato, Akane, Dias, Diana Marques, Jeon, Maryangel, Vance, Caroline, Wong, Chun Hao, de Majo, Martina, Kattuah, Wejdan, Mitchell, Jacqueline C, Scotter, Emma L, Parkin, Nicholas W, Sapp, Peter C, Nolan, Matthew, Nestor, Peter J, Simpson, Michael, Weale, Michael, Lek, Monkel, Baas, Frank, Vianney de Jong, J M, ten Asbroek, Anneloor L M A, Redondo, Alberto Garcia, Esteban-Pérez, Jesús, Tiloca, Cinzia, Verde, Federico, Duga, Stefano, Leigh, Nigel, Pall, Hardev, Morrison, Karen E, Al-Chalabi, Ammar, Shaw, Pamela J, Kirby, Janine, Turner, Martin R, Talbot, Kevin, Hardiman, Orla, Glass, Jonathan D, De Belleroche, Jacqueline, Maki, Masatoshi, Moss, Stephen E, Miller, Christopher, Gellera, Cinzia, Ratti, Antonia, Al-Sarraj, Safa, Brown Jr, Robert H, Silani, Vincenzo, Landers, John E and Shaw, Christopher E (2017) Mutations in the vesicular trafficking protein annexin A11 are associated with amyotrophic lateral sclerosis. Science Translational Medicine, 9 (388). eaad9157 1 -15. ISSN 1946-6242

McLaughlin, Russell L, Leigh, P Nigel, Project MinE, GWAS Consortium and Schizophrenia Working Group of the Psychiatric, Genomics Consortium (2017) Genetic correlation between amyotrophic lateral sclerosis and schizophrenia. Nature Communications, 8. a14774. ISSN 2041-1723

Glasmacher, Stella, Leigh, Nigel and Saha, Romi (2017) Predictors of survival in progressive supranuclear palsy and multiple system atrophy: a systematic review and meta-analysis. Journal of Neurology, Neurosurgery and Psychiatry, 88 (16). ISSN 0022-3050

Meininger, Vincent, Genge, Angela, van den Berg, Leonard H, Robberecht, Wim, Ludolph, Albert, Chio, Adriano, Kim, Seung H, Leigh, P Nigel, Kiernan, Matthew C, Shefner, Jeremy M, Desneulle, Claude, Morrison, Karen, Petri, Susanne, Boswell, Diane, Temple, Jane, Mohindra, Rajat, Davies, Matt, Bullman, Jonathan, Rees, Paul and Lavrov, Arseniy (2017) Safety and efficacy of ozanezumab in patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled, phase 2 trial. Lancet Neurology, 16 (3). pp. 208-216. ISSN 1474-4422

Ton, Fang, Al Khleifat, Ahmad, Stahl, Daniel R, Lazo Dal Torre, Claudia, Murphy, Caroline, Young, Carolyn, Shaw, Pamela J, Leigh, P. Nigel, Al-Chalabi, Ammar and UK-MND, LicALS (2017) Comparison of the King's and MiToS staging systems for ALS. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 18 (3-4). pp. 227-232. ISSN 2167-8421

Fang, Ton, Al Khleifat, Ahmed, Stahl, Daniel R, Lazo La Torre, Claudia, Murphy, Caroline, Young, Carolyn, Shaw, Pamela J, Leigh, P. Nigel, Al-Chalabi, Ammar and LiCALS, UK-MND (2017) Comparison of the King’s and MiToS staging systems for ALS. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 18 (3-4). pp. 227-232. ISSN 2167-8421

Polkey, Michael I, Lyall, Rebecca A, Yang, Ke, Johnson, Erin, Leigh, P. Nigel and Moxham, John (2017) Respiratory muscle strength as a predictive biomarker for survival in amyotrophic lateral sclerosis. American Journal of Respiratory and Critical Care Medicine, 195 (1). pp. 86-95. ISSN 1073-449X

Knibb, Jonathan A, Noa, Keren, Kulka, Anna, Leigh, Nigel, Shaw, Christopher E, Tsuda, Miho, Al-Chalabi, Ammar and Sarah, Martin (2016) A clinical tool for predicting survival in ALS. Journal of Neurology, Neurosurgery and Psychiatry, 87 (12). pp. 1361-1367. ISSN 0022-3050

Cercignani, Mara, Guilietti, Giovanni, Dowell, Nick G, Gabel, Matt, Broad, Rebecca, Leigh, P. Nigel, Harrison, Neil A and Bozzali, Marco (2016) Characterizing axonal myelination within the healthy population: a tract-by-tract mapping of effects of age and gender on the fiber g-ratio. Neurobiology of Aging, 49. pp. 109-118. ISSN 0197-4580

Gaastra, Benjamin, Shatunov, Aleksey, Pulit, Sara, Jones, Ashley, Sproviero, William, Gillett, Alexandra, Chen, Zhongbo, Kirby, Janine, Fogh, Isabella, Powell, John F, Leigh, P. Nigel, Morrison, Karen, Shaw, Pamela J, Shaw, Christopher E, van den Berg, Leonard H, Veldink, Jan H, Lewis, Cathryn M and Al-Chalabi, Ammar (2016) Rare genetic variation in UNC13A may modify survival in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 17 (7-8). pp. 593-599. ISSN 2167-8421

Martin, Naomi H, Lawrence, Vanessa, Murray, Joanna, Janssen, Anna, Higginson, Irene, Lyall, Rebecca, Burman, Rachel, Leigh, P Nigel, Al-Chalabi, Ammar and Goldstein, Laura H (2016) Decision making about gastrostomy and noninvasive ventilation in amyotrophic lateral sclerosis. Qualitative Health Research, 26 (10). pp. 1366-1381. ISSN 1049-7323

Leigh, P. Nigel and et al, (2016) Genome-wide association analyses identify new risk variants and the genetic architecture of amyotrophic lateral sclerosis. Nature Genetics, 48 (9). pp. 1043-1048. ISSN 1061-4036

Kenna, Kevin P, Doormaal, Perry, Dekker, Annelot, Ticozzi, Nicola, Kenna, Brendan, Diekstra, Frank, van Rheenen, Wouter, Leigh, P Nigel and et al, (2016) NEK1 variants confer susceptibility to amyotrophic lateral sclerosis. Nature Genetics, 48 (9). pp. 1037-1042. ISSN 1061-4036

Fogh, Isabella, Lin, Kuang, Tiloca, Cinzia, Rooney, James, Gellera, Cinzia, Diekstra, Frank P, Ratti, Antonia, Shatunov, Aleksay, van Es, Michael, Proitis, Petroula, Jones, Ashley, Sproviero, William, Chiò, Adriano, Mclaughlin, Russell Lewis, Sorarù, Gianni, Corrado, Lucia, Stahl, Daniel, Del Bo, Roberto, Cereda, Christina, Castellotti, Barbara, Glass, Jonathan D, Newhouse, Steven, Dobson, Richard, Smith, Bradley N, Topp, Simon, van Rheenen, Wouter, Meininger, Vincent, Melki, Judith, Morrison, Karen E, Shaw, Pamela J, Leigh, P. Nigel, Andersen, Peter M, Comi, Giacomo P, Ticozzi, Nicola, Mazzini, Letizia, D'Alfonso, Sandra, Traynor, Bryan J, Van Damme, Phillip, Robberecht, Wim, Brown, Robert H, Landers, John E, Hardiman, Orla, Lewis, Cathryn M, van den Berg, Leonard H, Shaw, Christopher E, Veldink, Jan H, Silani, Vincenzo, Al-Chalabi, Ammar and Powell, John (2016) Association of a locus in the CAMTA1 gene with survival in patients with sporadic amyotrophic lateral sclerosis. JAMA Neurology, 73 (7). pp. 812-820. ISSN 2168-6149

Rutter-Locher, Zoe, Turner, Martin R, Leigh, Nigel and Al-Chalabi, Ammar (2016) Analysis of terms used for the diagnosis and classification of amyotrophic lateral sclerosis and motor neuron disease. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 17 (7-8). pp. 600-604. ISSN 2167-8421

Tsermentseli, Stella, Leigh, Nigel, Taylor, Lorna J, Radunovic, Aleksandar, Catani, Marco and Goldstein, Laura H (2015) Syntactic processing as a marker for cognitive impairment in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 17 (1-2). pp. 69-76. ISSN 2167-8421

van der Kleij, Lisa A, Jones, Ashley R, Steen, I Nick, Young, Carolyn A, Shaw, Pamela J, Shaw, Christopher E, Leigh, Nigel, Turner, Martin R and Al-Chalabi, Ammar (2015) Regionality of disease progression predicts prognosis in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 16 (7-8). ISSN 2167-8421

Broad, R and Leigh, P N (2015) Recognising facial onset sensory motor neuronopathy syndrome: insight from six new cases. Practical Neurology, 15 (4). pp. 293-297. ISSN 1474-7758

Greenaway, L P, Martin, N H, Lawrence, V, Janssen, A, Al-Chalabi, A, Leigh, P N and Goldstein, L H (2015) Accepting or declining non-invasive ventilation or gastrostomy in amyotrophic lateral sclerosis: patients' perspectives. Journal of Neurology, 262 (4). pp. 1002-1013. ISSN 0340-5354

Leigh, P N, Al-Sarraj, S and DiMauro, S (2015) Impact commentaries. Subacute necrotising encephalomyelopathy (Leigh's disease; Leigh syndrome). Journal of Neurology, Neurosurgery and Psychiatry, 86 (4). pp. 363-5. ISSN 0022-3050

Balendra, Rubika, Jones, Ashley, Jivraj, Naheed, Steen, I Nick, Young, Carolyn A, Shaw, Pamela J, Turner, Martin R, Leigh, Nigel, Al-Chalabi, Ammar, UK-MND LiCALS Study Group, and Mito Target ALS Study Group, (2015) Use of clinical staging in amyotrophic lateral sclerosis for phase 3 clinical trials. Journal of Neurology, Neurosurgery and Psychiatry, 86 (1). pp. 45-49. ISSN 0022-3050

Worker, Amanda, Blain, Camilla, Jarosz, Jozef, Chaudhuri, K Ray, Barker, Gareth J, Williams, Steven C R, Brown, Richard, Leigh, P Nigel and Simmons, Andrew (2014) Cortical thickness, surface area and volume measures in Parkinson's disease, multiple system atrophy and progressive supranuclear palsy. PLoS ONE, 9 (12). e114167. ISSN 1932-6203

Al-Chalabi, Ammar, Calvo, Andrea, Chio, Adriano, Colville, Shuna, Ellis, Cathy M, Hardiman, Orla, Heverin, Mark, Howard, Robin S, Huisman, Mark H B, Keren, Noa, Leigh, Nigel, Mazzini, Letizia, Mora, Gabriele, Orrell, Richard W, Rooney, James, Scott, Kirsten M, Scotton, William J, Seelen, Meinie, Shaw, Christopher E, Sidle, Katie S, Swingler, Robert, Tsuda, Miho, Veldink, Jan H, Visser, Anne E, van den Berg, Leonard H and Pearce, Neil (2014) Analysis of amyotrophic lateral sclerosis as a multistep process: a population-based modelling study. Lancet Neurology, 13 (11). pp. 1108-1113. ISSN 1474-4422

Martin, Naomi H, Landau, Sabine, Janssen, Anna, Lyall, Rebecca, Higginson, Irene, Burman, Rachel, McCrone, Paul, Sakel, Mohammed, Ellis, Catherine M, Shaw, Christopher E, Al-Chalabi, Ammar, Leigh, P Nigel and Goldstein, Laura H (2014) Psychological as well as illness factors influence acceptance of non-invasive ventilation (NIV) and gastrostomy in amyotrophic lateral sclerosis (ALS): a prospective population study. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration, 15 (5-6). pp. 376-387. ISSN 2167-9223

Diekstra, Frank P, Van Deerlin, Vivianna M, van Swieten, John C, Al-Chalabi, Ammar, Ludolph, Albert C, Weishaupt, Jochen H, Hardiman, Orla, Landers, John E, Brown, Robert H, van Es, Michael A, Pasterkamp, R Jeroen, Koppers, Max, Andersen, Peter M, Estrada, Karol, Rivadeneira, Fernando, Hofman, Albert, Uitterlinden, André G, van Damme, Philip, Melki, Judith, Meininger, Vincent, Shatunov, Aleksey, Shaw, Christopher E, Leigh, P Nigel, Shaw, Pamela J, Morrison, Karen E, Fogh, Isabella, Chiò, Adriano, Traynor, Bryan J, Czell, David, Weber, Markus, Heutink, Peter, de Bakker, Paul I W, Silani, Vincenzo, Robberecht, Wim, van den Berg, Leonard H and Veldink, Jan H (2014) C9orf72 and UNC13A are shared risk loci for amyotrophic lateral sclerosis and frontotemporal dementia: a genome-wide meta-analysis. Annals of Neurology, 76 (1). pp. 120-33. ISSN 0364-5134

Balendra, Rubika, Jones, Ashley, Jivraj, Naheed, Knights, Catherine, Ellis, Catherine M, Burman, Rachel, Turner, Martin R, Leigh, Nigel, Shaw, Christopher E and Al-Chalabi, Ammar (2014) Estimating clinical stage of amyotrophic lateral sclerosis from the ALS functional rating scale. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration, 15 (3-4). pp. 279-284. ISSN 2167-8421

Meininger, Vincent, Pradat, Pierre-François, Corse, Andrea, Al-Sarraj, Safa, Rix Brooks, Benjamin, Caress, James B, Cudkowicz, Merit, Kolb, Stephen J, Lange, Dale, Leigh, P Nigel, Meyer, Thomas, Milleri, Stefano, Morrison, Karen E, Orrell, Richard W, Peters, Gary, Rothstein, Jeffrey D, Shefner, Jeremy, Lavrov, Arseniy, Williams, Nicola, Overend, Phil, Price, Jeffrey, Bates, Stewart, Bullman, Jonathan, Krull, David, Berges, Alienor, Abila, Bams, Meno-Tetang, Guy and Wurthner, Jens (2014) Safety, pharmacokinetic, and functional effects of the nogo-a monoclonal antibody in amyotrophic lateral sclerosis: a randomized, first-in-human clinical trial. PLoS ONE, 9 (5). e97803. ISSN 1932-6203

Worker, Amanda, Blain, Camilla, Jarosz, Jozef, Chaudhuri, K Ray, Barker, Gareth J, Williams, Steve C R, Brown, Richard G, Leigh, P Nigel, Dell'Acqua, Flavio and Simmons, Andrew (2014) Diffusion tensor imaging of Parkinson's disease, multiple system atrophy and progressive supranuclear palsy: a tract-based spatial statistics study. PLoS ONE, 9 (11). e112638. ISSN 1932-6203

Keren, Noa, Scott, Kirsten M, Tsuda, Miho, Barnwell, Jessica, Knibb, Jonathan A, Ellis, Cathy M, Leigh, P Nigel, Shaw, Christopher E and Al-Chalabi, Ammar (2014) Evidence of an environmental effect on survival in ALS. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 15 (7-8). pp. 528-533. ISSN 2167-9223

Jones, Ashley R, Jivraj, Naheed, Balendra, Rubika, Murphy, Caroline, Kelly, Joanna, Thornhill, Marie, Young, Carolyn, Shaw, Pamela J, Leigh, Nigel, Turner, Martin R, Steen, I Nick, McCrone, Paul and Al-Chalabi, Ammar (2014) Health utility decreases with increasing clinical stage in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 15 (3-4). pp. 285-291. ISSN 2167-9223

Al-Sarraj, Safa, King, Andrew, Cleveland, Matt, Pradat, Pierre-François, Corse, Andrea, Rothstein, Jeffrey D, Leigh, Peter Nigel, Abila, Bams, Bates, Stewart, Wurthner, Jens and Meininger, Vincent (2014) Mitochondrial abnormalities and low grade inflammation are present in the skeletal muscle of a minority of patients with amyotrophic lateral sclerosis; an observational myopathology study. Acta Neuropathologica Communications, 2 (1). p. 165. ISSN 2051-5960

Stanton, Biba R, Leigh, P Nigel, Howard, Robert J, Barker, Gareth J and Brown, Richard G (2013) Behavioural and emotional symptoms of apathy are associated with distinct patterns of brain atrophy in neurodegenerative disorders. Journal of Neurology, 260 (10). pp. 2481-2490. ISSN 0340-5354

Jones, Ashley R, Woollacott, Ione, Shatunov, Aleksey, Cooper-Knock, Johnathan, Buchman, Vladimir, Sproviero, William, Smith, Bradley, Scott, Kirsten M, Balendra, Rubika, Abel, Olubunmi, McGuffin, Peter, Ellis, Catherine M, Shaw, Pamela J, Morrison, Karen E, Farmer, Anne, Lewis, Cathryn M, Leigh, P Nigel, Shaw, Christopher E, Powell, John F and Al-Chalabi, Ammar (2013) Residual association at C9orf72 suggests an alternative amyotrophic lateral sclerosis-causing hexanucleotide repeat. Neurobiology of Aging, 34 (9). 2234.e1-2234.e7. ISSN 0197-4580

Saleem, Tariq Z, Higginson, Irene J, Chaudhuri, K Ray, Martin, Anne, Burman, Rachel and Leigh, P Nigel (2013) Symptom prevalence, severity and palliative care needs assessment using the Palliative Outcome Scale: a cross-sectional study of patients with Parkinson's disease and related neurological conditions. Palliative Medicine, 27 (8). pp. 722-731. ISSN 0269-2163

Marquand, Andre F, Filippone, Maurizio, Ashburner, John, Girolami, Mark, Mourao-Miranda, Janaina, Barker, Gareth J, Williams, Steven C R, Leigh, P Nigel and Blain, Camilla R V (2013) Automated, high accuracy classification of Parkinsonian disorders: a pattern recognition approach. PLoS ONE, 8 (7). e69237. ISSN 1932-6203

Al-Chalabi, Ammar, Kwak, Shin, Mehler, Mark, Rouleau, Guy, Siddique, Teepu, Strong, Michael and Leigh, Peter Nigel (2013) Genetic and epigenetic studies of amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration, 14 (S1). pp. 44-52. ISSN 2167-9223

Leigh, P N and UKMND-LiCALS Study Group, (2013) Lithium in patients with amyotrophic lateral sclerosis (LiCALS): a phase 3 multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurology, 12 (4). pp. 339-345. ISSN 1474-4422

Leigh, P Nigel and ALSGEN consortium, (2013) Age of onset of amyotrophic lateral sclerosis is modulated by a locus on 1p34.1. Neurobiology of Aging, 34 (1). 357.e7-357.e19. ISSN 1558-1497

Higginson, Irene J, Gao, Wei, Saleem, Tariq Zaffer, Chaudhuri, K Ray, Burman, Rachel, McCrone, Paul and Leigh, Nigel (2012) Symptoms and quality of life in late stage Parkinson syndromes: a longitudinal community study of predictive factors. PLoS ONE, 7 (11). e46327. ISSN 1932-6203

Taylor, Lorna J, Brown, Richard G, Tsermentseli, Stella, Al-Chalabi, Ammar, Shaw, Christopher E, Ellis, Catherine M, Leigh, Nigel and Goldstein, Laura H (2012) Is language impairment more common than executive dysfunction in amyotrophic lateral sclerosis? Journal of Neurology, Neurosurgery and Psychiatry, 84. pp. 494-498. ISSN 1468-330X

Johnson, Julia, Leigh, Nigel, Shaw, Christopher E, Ellis, Cathy, Burman, Rachel and Al-Chalabi, Ammar (2012) Eating-derived pleasure in amyotrophic lateral sclerosis as a predictor of non-oral feeding. Amyotrophic Lateral Sclerosis, 13 (6). pp. 555-559. ISSN 1471-180X

Cuddy, Marion, Papps, Benjamin J, Thambisetty, Madhav, Leigh, Nigel and Goldstein, Laura H (2012) Processing and memory for emotional and neutral material in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis, 13 (6). pp. 592-598. ISSN 1471-180X

Scotton, W J, Scott, K M, Moore, D H, Almedom, L, Wijesekera, L C, Janssen, A, Nigro, C, Sakel, M, Leigh, N, Shaw, C and Al-Chalabi, A (2012) Prognostic categories for amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis, 13 (6). pp. 502-508. ISSN 1471-180X

Roche, Jose C, Rojas-Garcia, Ricardo, Scott, Kirsten M, Scotton, William, Ellis, Catherine E, Burman, Rachel, Wijesekera, Lokesh, Turner, Martin R, Leigh, Nigel, Shaw, Christopher E and Al-Chalabi, Ammar (2012) A proposed staging system for amyotrophic lateral sclerosis. Brain, 135 (3). pp. 847-852. ISSN 1460-2156

Tsermentseli, Stella, Leigh, Nigel and Goldstein, Laura H (2012) The anatomy of cognitive impairment in amyotrophic lateral sclerosis: more than frontal lobe dysfunction. Cortex, 48 (2). pp. 166-182. ISSN 1973-8102

Rojas-Garcia, Ricardo, Scott, Kirsten M, Roche, Jose Carlos, Scotton, William, Martin, Naomi, Janssen, Anna, Goldstein, Laura H, Leigh, Nigel, Ellis, Cathy M, Shaw, Christopher E and Al-Chalabi, Ammar (2012) No evidence for a large difference in ALS frequency in populations of African and European origin: a population based study in inner city London. Amyotrophic Lateral Sclerosis, 13 (1). pp. 66-68. ISSN 1471-180X

Diekstra, Frank P, Saris, Christiaan G J, van Rheenen, Wouter, Franke, Lude, Jansen, Ritsert C, van Es, Michael A, van Vught, Paul W J, Blauw, Hylke M, Groen, Ewout J N, Horvath, Steve, Estrada, Karol, Rivadeneira, Fernando, Hofman, Albert, Uitterlinden, Andre G, Robberecht, Wim, Andersen, Peter M, Melki, Judith, Meininger, Vincent, Hardiman, Orla, Landers, John E, Brown, Robert H, Shatunov, Aleksey, Shaw, Christopher E, Leigh, Nigel, Al-Chalabi, Ammar, Ophoff, Roel A, van den Berg, Leonard H and Veldink, Jan H (2012) Mapping of gene expression reveals CYP27A1 as a susceptibility gene for sporadic ALS. PLoS ONE, 7 (4). e35333. ISSN 1932-6203

Goldstein, L H, Newsom-Davis, I C, Bryant, V, Brammer, M, Leigh, N and Simmons, A (2011) Altered patterns of cortical activation in ALS patients during attention and cognitive response inhibition tasks. Journal of Neurology, 258 (12). pp. 2186-2198. ISSN 1432-1459

Hanby, Martha F, Scott, Kirsten M, Scotton, William, Wijesekera, Lokesh, Mole, Thomas, Ellis, Catherine E, Leigh, Nigel, Shaw, Christopher E and Al-Chalabi, Ammar (2011) The risk to relatives of patients with sporadic amyotrophic lateral sclerosis. Brain, 134 (12). pp. 3454-3457. ISSN 1460-2156

McCrone, Paul, Payan, Christine Anne Mary, Knapp, Martin, Ludolph, Albert, Agid, Yves, Leigh, Nigel and Bensimon, Gilbert (2011) The economic costs of progressive supranuclear palsy and multiple system atrophy in France, Germany and the United Kingdom. PLoS ONE, 6 (9). e24369. ISSN 1932-6203

Rolland, Yan, Vérin, Marc, Payan, Christine A, Duchesne, Simon, Kraft, Eduard, Hauser, Till K, Jarosz, Josef, Deasy, Neil, Defevbre, Luc, Delmaire, Christine, Dormont, Didier, Ludolph, Albert C, Bensimon, Gilbert and Leigh, Nigel (2011) A new MRI rating scale for progressive supranuclear palsy and multiple system atrophy: validity and reliability. Journal of Neurology, Neurosurgery and Psychiatry, 82 (9). pp. 1025-1032. ISSN 1468-330X

Payan, Christine A M, Viallet, François, Landwehrmeyer, Bernhard G, Bonnet, Anne-Marie, Borg, Michel, Durif, Franck, Lacomblez, Lucette, Bloch, Frédéric, Verny, Marc, Fermanian, Jacques, Agid, Yves, Ludolph, Albert C, Leigh, Nigel and Bensimon, Gilbert (2011) Disease severity and progression in progressive supranuclear palsy and multiple system atrophy: validation of the NNIPPS-Parkinson Plus Scale. PLoS ONE, 6 (8). e22293. ISSN 1932-6203

Blain, C R V, Brunton, S, Williams, V C, Leemans, A, Turner, M R, Andersen, P M, Catani, M, Stanton, B R, Ganesalingham, J, Jones, D K, Williams, S C R, Leigh, N and Simmons, A (2011) Differential corticospinal tract degeneration in homozygous 'D90A' SOD-1 ALS and sporadic ALS. Journal of Neurology, Neurosurgery and Psychiatry, 82 (8). pp. 843-849. ISSN 1468-330X

Vivekananda, Umesh, Manjalay, Zita-Rose, Ganesalingam, Jeban, Simms, Jacqueline, Shaw, Christopher E, Leigh, Nigel, Turner, Martin R and Al-Chalabi, Ammar (2011) Low index-to-ring finger length ratio in sporadic ALS supports prenatally defined motor neuronal vulnerability. Journal of Neurology, Neurosurgery and Psychiatry, 82 (6). pp. 635-637. ISSN 1468-330X

Al-Chalabi, Ammar, Shaw, Pamela J, Young, Carolyn A, Morrison, Karen E, Murphy, Caroline, Thornhill, Marie, Kelly, Joanna, Steen, I Nicholas and Leigh, Nigel (2011) 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, 11 (1). pp. 111-125. ISSN 1471-2377

Al-Chalabi, A, Fang, F, Hanby, M F, Leigh, P N, Shaw, C E, Ye, W and Rijsdijk, F (2010) An estimate of amyotrophic lateral sclerosis heritability using twin data. Journal of Neurology, Neurosurgery, and Psychiatry, 81 (12). pp. 1324-6. ISSN 1468-330X

Shatunov, Aleksey, Leigh, P Nigel and et al, (2010) Chromosome 9p21 in sporadic amyotrophic lateral sclerosis in the UK and seven other countries: a genome-wide association study. Lancet Neurology, 9 (10). pp. 986-994. ISSN 1474-4422

Manjaly, Zita R, Scott, Kirsten M, Abhinav, Kumar, Wijesekera, Lokesh, Ganesalingam, Jeban, Goldstein, Laura H, Janssen, Anna, Dougherty, Andrew, Willey, Emma, Stanton, Biba R, Turner, Martin R, Ampong, Mary-Ann, Sakel, Mohammed, Orrell, Richard W, Howard, Robin, Shaw, Chris E, Leigh, Nigel and Al-Chalabi, Ammar (2010) The sex ratio in amyotrophic lateral sclerosis: A population based study. Amyotrophic Lateral Sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases, 11 (5). pp. 439-42. ISSN 1471-180X

Guevara, C A, Blain, C R, Stahl, D, Lythgoe, D J, Leigh, P N and Barker, G J (2010) Quantitative magnetic resonance spectroscopic imaging in Parkinson's disease, progressive supranuclear palsy and multiple system atrophy. European Journal of Neurology, 17 (9). pp. 1193-202. ISSN 1351-5101

Brown, Richard G, Lacomblez, Lucette, Landwehrmeyer, Bernard G, Bak, Thomas, Uttner, Ingo, Dubois, Bruno, Agid, Yves, Ludolph, Albert, Bensimon, Gilbert, Payan, Christine and Leigh, P Nigel (2010) Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy. Brain, 133 (8). pp. 2382-2393. ISSN 0006-8950

Atkins, Louise, Brown, Richard G, Leigh, Nigel and Goldstein, Laura H (2010) Marital relationships in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis, 11 (4). pp. 344-350. ISSN 1471-180X

Taylor, L, Wicks, P, Leigh, N and Goldstein, L H (2010) Prevalence of depression in amyotrophic lateral sclerosis and other motor disorders. European Journal of Neurology, 17 (8). pp. 1047-1053. ISSN 1468-1331

Tudor, E L, Galtrey, C M, Perkinton, M S, Lau, K-F, De Vos, K J, Mitchell, J C, Ackerley, S, Hortobágyi, T, Vámos, E, Leigh, N, Klasen, C, McLoughlin, D M, Shaw, C E and Miller, C C J (2010) Amyotrophic lateral sclerosis mutant vesicle-associated membrane protein-associated protein-B transgenic mice develop TAR-DNA-binding protein-43 pathology. Neuroscience, 167 (3). pp. 774-785. ISSN 1873-7544

Leigh, P Nigel and Wijesekera, Lokesh C (2010) Motor neuron disease: Focusing the mind on ALS: updated practice parameters. Nature Reviews Neurology, 6 (4). pp. 191-192. ISSN 1759-4758

Maekawa, Satomi, Leigh, P Nigel, King, Andrew, Jones, Edith, Steele, John C, Bodi, Istvan, Shaw, Christopher E, Hortobagyi, Tibor and Al-Sarraj, Safa (2009) TDP-43 is consistently co-localized with ubiquitinated inclusions in sporadic and Guam amyotrophic lateral sclerosis but not in familial amyotrophic lateral sclerosis with and without SOD1 mutations. Neuropathology : official journal of the Japanese Society of Neuropathology, 29 (6). pp. 672-83. ISSN 1440-1789

van Es, Michael A, Leigh, P Nigel and et al, (2009) Genome-wide association study identifies 19p13.3 (UNC13A) and 9p21.2 as susceptibility loci for sporadic amyotrophic lateral sclerosis. Nature Genetics, 41 (10). pp. 1083-7. ISSN 1061-4036

Lewis, Dylan, Ampong, Mary-Ann, Rio, Alan, Willey, Emma, Johnson, Julia, Shaw, Christopher E, Ellis, Catherine M, Al-Chalabi, Ammar, Leigh, P Nigel and Sidhu, Paul S (2009) Mushroom-cage gastrostomy tube placement in patients with amyotrophic lateral sclerosis: a 5-year experience in 104 patients in a single institution. European Radiology, 19 (7). pp. 1763-71. ISSN 0938-7994

Landers, John E, Leigh, P Nigel and et al, (2009) Reduced expression of the Kinesin-Associated Protein 3 (KIFAP3) gene increases survival in sporadic amyotrophic lateral sclerosis. Proceedings of the National Academy of Sciences of the United States of America, 106 (22). pp. 9004-9. ISSN 1091-6490

Strong, Michael J, Grace, Gloria M, Freedman, Morris, Lomen-Hoerth, Cathy, Woolley, Susan, Goldstein, Laura H, Murphy, Jennifer, Shoesmith, Christen, Rosenfeld, Jeffery, Leigh, P Nigel, Bruijn, Lucie, Ince, Paul and Figlewicz, Denise (2009) Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases, 10 (3). pp. 131-46. ISSN 1471-180X

Gordon, Paul H, Corcia, Philippe, Lacomblez, Lucette, Pochigaeva, Ksenia, Abitbol, Jean-Louis, Cudkowicz, Merit, Leigh, P Nigel and Meininger, Vincent (2009) Defining survival as an outcome measure in amyotrophic lateral sclerosis. Archives of Neurology, 66 (6). pp. 758-61. ISSN 0003-9942

Stevenson, Alison, Yates, Darran M, Manser, Catherine, De Vos, Kurt J, Vagnoni, Alessio, Leigh, P Nigel, McLoughlin, Declan M and Miller, Christopher C J (2009) Riluzole protects against glutamate-induced slowing of neurofilament axonal transport. Neuroscience Letters, 454 (2). pp. 161-4. ISSN 0304-3940

Koritnik, Blaž, Azam, Sheba, Andrew, Chris M, Leigh, P Nigel and Williams, Steven C R (2009) Imaging the brain during sniffing: a pilot fMRI study. Pulmonary pharmacology & therapeutics, 22 (2). pp. 97-101. ISSN 1094-5539

Vance, Caroline, Rogelj, Boris, Hortobágyi, Tibor, De Vos, Kurt J, Nishimura, Agnes Lumi, Sreedharan, Jemeen, Hu, Xun, Smith, Bradley, Ruddy, Deborah, Wright, Paul, Ganesalingam, Jeban, Williams, Kelly L, Tripathi, Vineeta, Al-Saraj, Safa, Al-Chalabi, Ammar, Leigh, Nigel, Blair, Ian P, Nicholson, Garth, de Belleroche, Jackie, Gallo, Jean-Marc, Miller, Christopher C and Shaw, Christopher E (2009) Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science (New York, N.Y.), 323 (5918). pp. 1208-11. ISSN 1095-9203

Simpson, Claire L, Leigh, P Nigel and et al, (2009) Variants of the elongator protein 3 (ELP3) gene are associated with motor neuron degeneration. Human molecular genetics, 18 (3). pp. 472-81. ISSN 1460-2083

Wicks, P, Abrahams, S, Papps, B, Al-Chalabi, A, Shaw, C E, Leigh, P N and Goldstein, L H (2009) SOD1 and cognitive dysfunction in familial amyotrophic lateral sclerosis. Journal of neurology, 256 (2). pp. 234-41. ISSN 1432-1459

Turner, Martin R, Kiernan, Matthew C, Leigh, P Nigel and Talbot, Kevin (2009) Biomarkers in amyotrophic lateral sclerosis. Lancet Neurology, 8 (1). pp. 94-109. ISSN 1474-4422

Stanton, Biba R, Shinhmar, Daisy, Turner, Martin R, Williams, Victoria C, Williams, Steven C R, Blain, Camilla R V, Giampietro, Vincent P, Catani, Marco, Leigh, P Nigel, Andersen, Peter M and Simmons, Andrew (2009) Diffusion tensor imaging in sporadic and familial (D90A SOD1) forms of amyotrophic lateral sclerosis. Archives of Neurology, 66 (1). pp. 109-15. ISSN 0003-9942

Bensimon, Gilbert, Ludolph, Albert, Agid, Yves, Vidailhet, Marie, Payan, Christine, Leigh, P Nigel and Unset (2009) Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: the NNIPPS study. Brain, 132 (1). pp. 156-171. ISSN 1460-2156

Wijesekera, Lokesh C and Leigh, P Nigel (2009) Amyotrophic lateral sclerosis. Orphanet Journal of Rare Diseases, 4. p. 3. ISSN 1750-1172

Al-Chalabi, Ammar, Dürr, Alexandra, Wood, Nicholas W, Parkinson, Michael H, Camuzat, Agnes, Hulot, Jean-Sébastien, Morrison, Karen E, Renton, Alan, Sussmuth, Sigurd D, Landwehrmeyer, Bernhard G, Ludolph, Albert, Agid, Yves, Brice, Alexis, Leigh, Nigel and Bensimon, Gilbert (2009) Genetic variants of the alpha-synuclein gene SNCA are associated with multiple system atrophy. PLoS ONE, 4 (9). e7114. ISSN 1932-6203

Scott, Kirsten M, Abhinav, Kumar, Stanton, Biba R, Johnston, Clare, Turner, Martin R, Ampong, Mary-Ann, Sakel, Mohamed, Orrell, Richard W, Howard, Robin, Shaw, Christopher E, Leigh, P Nigel and Al-Chalabi, Ammar (2009) Geographical clustering of amyotrophic lateral sclerosis in South-East England: a population study. Neuroepidemiology, 32 (2). pp. 81-8. ISSN 1423-0208

Meininger, Vincent, Drory, Vivian E, Leigh, Nigel, Ludolph, Albert, Robberecht, Wim and Silani, Vincenzo (2009) Glatiramer acetate has no impact on disease progression in ALS at 40 mg/day: a double- blind, randomized, multicentre, placebo-controlled trial. Amyotrophic Lateral Sclerosis, 10 (5-6). pp. 378-383. ISSN 1471-180X

Ganesalingam, Jeban, Stahl, Daniel, Wijesekera, Lokesh, Galtrey, Clare, Shaw, Christopher E, Leigh, Nigel and Al-Chalabi, Ammar (2009) Latent cluster analysis of ALS phenotypes identifies prognostically differing groups. PLoS ONE, 4 (9). e7107. ISSN 1932-6203

Wain, Louise V, Pedroso, Inti, Landers, John E, Breen, Gerome, Shaw, Christopher E, Leigh, Nigel, Brown, Robert H, Tobin, Martin D and Al-Chalabi, Ammar (2009) The role of copy number variation in susceptibility to amyotrophic lateral sclerosis: genome-wide association study and comparison with published loci. PLoS ONE, 4 (12). e8175. ISSN 1932-6203

Landers, John E, Leigh, Nigel and et al, (2008) A common haplotype within the PON1 promoter region is associated with sporadic ALS. Amyotrophic Lateral Sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases, 9 (5). pp. 306-14. ISSN 1471-180X

Manser, Catherine, Stevenson, Alison, Banner, Steven, Davies, Jennifer, Tudor, Elizabeth L, Ono, Yoshitaka, Leigh, P Nigel, McLoughlin, Declan M, Shaw, Christopher E and Miller, Christopher C J (2008) Deregulation of PKN1 activity disrupts neurofilament organisation and axonal transport. FEBS letters, 582 (15). pp. 2303-8. ISSN 0014-5793

Landers, J E, Leigh, Nigel and et al, (2008) New VAPB deletion variant and exclusion of VAPB mutations in familial ALS. Neurology, 70 (14). pp. 1179-85. ISSN 1526-632X

Sreedharan, Jemeen, Leigh, Nigel and et al, (2008) TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science, 319 (5870). pp. 1668-72. ISSN 1095-9203

Wicks, Paul, Turner, Martin R, Abrahams, Sharon, Hammers, Alexander, Brooks, David J, Leigh, Nigel and Goldstein, Laura H (2008) Neuronal loss associated with cognitive performance in amyotrophic lateral sclerosis: an (11C)-flumazenil PET study. Amyotrophic Lateral Sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases, 9 (1). pp. 43-9. ISSN 1471-180X