By Scientia Professor Matthew Colm Kiernan

Journal articles

Kiernan MC; Vucic S; Talbot K; McDermott CJ; Hardiman O; Shefner JM; Al-Chalabi A; Huynh W; Cudkowicz M; Talman P; Van den Berg LH; Dharmadasa T; Wicks P; Reilly C; Turner MR, 2021, 'Improving clinical trial outcomes in amyotrophic lateral sclerosis', Nature Reviews Neurology, 17, pp. 104 - 118, http://dx.doi.org/10.1038/s41582-020-00434-z

Trinh T; Park SB; Murray J; Pickering H; Lin CSY; Martin A; Friedlander M; Kiernan MC; Goldstein D; Krishnan AV, 2021, 'Neu-horizons: neuroprotection and therapeutic use of riluzole for the prevention of oxaliplatin-induced neuropathy—a randomised controlled trial', Supportive Care in Cancer, 29, pp. 1103 - 1110, http://dx.doi.org/10.1007/s00520-020-05591-x

Vucic S; Kiernan MC; Menon P; Huynh W; Rynders A; Ho KS; Glanzman R; Hotchkin MT, 2021, 'Study protocol of RESCUE-ALS: A Phase 2, randomised, double-blind, placebo-controlled study in early symptomatic amyotrophic lateral sclerosis patients to assess bioenergetic catalysis with CNM-Au8 as a mechanism to slow disease progression', BMJ open, 11, pp. e041479, http://dx.doi.org/10.1136/bmjopen-2020-041479

Shefner JM; Andrews JA; Genge A; Jackson C; Lechtzin N; Miller TM; Cockroft BM; Meng L; Wei J; Wolff AA; Malik FI; Bodkin C; Brooks BR; Caress J; Dionne A; Fee D; Goutman SA; Goyal NA; Hardiman O; Hayat G; Heiman-Patterson T; Heitzman D; Henderson RD; Johnston W; Karam C; Kiernan MC; Kolb SJ; Korngut L; Ladha S; Matte G; Mora JS; Needham M; Oskarsson B; Pattee GL; Pioro EP; Pulley M; Quan D; Rezania K; Schellenberg KL; Schultz D; Shoesmith C; Simmons Z; Statland J; Sultan S; Swenson A; Berg LHVD; Vu T; Vucic S; Weiss M; Whyte-Rayson A; Wymer J; Zinman L; Rudnicki SA, 2021, 'A Phase 2, Double-Blind, Randomized, Dose-Ranging Trial Of Reldesemtiv In Patients With ALS', Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 22, pp. 287 - 299, http://dx.doi.org/10.1080/21678421.2020.1822410

Bedlack R; Armon C; Barkhaus P; Barnes B; Bereman M; Bertorini T; Carter G; Chen A; Crayle J; Cudkowicz M; Jackson C; Kiernan M; Levitsky G; McDermott C; Pattee G; Pioro E; Salmon K; Staats K; Stephens J; Wicks P, 2021, 'ALSUntangled 57: Vinpocetine', Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 22, pp. 316 - 319, http://dx.doi.org/10.1080/21678421.2020.1786942

Dharmadasa T; Howells J; Matamala JM; Simon NG; Burke D; Vucic S; Kiernan MC, 2021, 'Cortical inexcitability defines an adverse clinical profile in amyotrophic lateral sclerosis', European Journal of Neurology, 28, pp. 90 - 97, http://dx.doi.org/10.1111/ene.14515

Rinaldi S; Davies A; Fehmi J; Beadnall HN; Wang J; Hardy TA; Barnett MH; Broadley SA; Waters P; Reddel SW; Irani SR; Brilot F; Dale RC; Ramanathan S, 2021, 'Overlapping central and peripheral nervous system syndromes in MOG antibody-associated disorders', Neurology(R) neuroimmunology & neuroinflammation, 8, http://dx.doi.org/10.1212/NXI.0000000000000924

Caga J; Zoing MC; Foxe D; Ramsey E; D’Mello M; Mioshi E; Ahmed RM; Kiernan MC; Piguet O, 2021, 'Problem-focused coping underlying lower caregiver burden in ALS-FTD: implications for caregiver intervention', Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 22, pp. 434 - 441, http://dx.doi.org/10.1080/21678421.2020.1867180

Hannaford A; Vucic S; Kiernan MC; Simon NG, 2021, 'Review article “spotlight on ultrasonography in the diagnosis of peripheral nerve disease: The evidence to date”', International Journal of General Medicine, 14, pp. 4579 - 4604, http://dx.doi.org/10.2147/IJGM.S295851

Ahmed RM; Bocchetta M; Todd EG; Tse NY; Devenney EM; Tu S; Caga J; Hodges JR; Halliday GM; Irish M; Kiernan MC; Piguet O; Rohrer JD, 2021, 'Tackling clinical heterogeneity across the amyotrophic lateral sclerosis-frontotemporal dementia spectrum using a transdiagnostic approach', Brain Communications, 3, http://dx.doi.org/10.1093/braincomms/fcab257

De Boer EMJ; Orie VK; Williams T; Baker MR; De Oliveira HM; Polvikoski T; Silsby M; Menon P; Van Den Bos M; Halliday GM; Van Den Berg LH; Van Den Bosch L; Van Damme P; Kiernan M; Van Es MA; Vucic S, 2021, 'TDP-43 proteinopathies: A new wave of neurodegenerative diseases', Journal of Neurology, Neurosurgery and Psychiatry, 92, pp. 86 - 95, http://dx.doi.org/10.1136/jnnp-2020-322983

Katzeff JS; Bright F; Lo K; Kril JJ; Connolly A; Crossett B; Ittner LM; Kassiou M; Loy CT; Hodges JR; Piguet O; Kiernan MC; Halliday GM; Kim WS, 2020, 'Altered serum protein levels in frontotemporal dementia and amyotrophic lateral sclerosis indicate calcium and immunity dysregulation', Scientific Reports, 10, http://dx.doi.org/10.1038/s41598-020-70687-7

Kiernan MC, 2020, 'Fasciculation anxiety syndrome in clinicians: FASICS', Practical Neurology, 20, pp. 433 - 434, http://dx.doi.org/10.1136/practneurol-2020-002786

Henden L; Twine NA; Szul P; McCann EP; Nicholson GA; Rowe DB; Kiernan MC; Bauer DC; Blair IP; Williams KL, 2020, 'Identity by descent analysis identifies founder events and links SOD1 familial and sporadic ALS cases', npj Genomic Medicine, 5, http://dx.doi.org/10.1038/s41525-020-00139-8

Park SB; Thurbon R; Kiernan MC, 2020, 'Isaacs syndrome: The frontier of neurology, psychiatry, immunology and cancer', Journal of Neurology, Neurosurgery and Psychiatry, 91, pp. 1243 - 1244, http://dx.doi.org/10.1136/jnnp-2020-324675

Kiernan MC, 2020, 'Metabolomic insights into neurodegene­ rative disease', Journal of Neurology, Neurosurgery and Psychiatry, 91, pp. 1250, http://dx.doi.org/10.1136/jnnp-2020-324856

Cooper-Knock J; Zhang S; Kenna KP; Moll T; Franklin JP; Allen S; Nezhad HG; Iacoangeli A; Yacovzada NY; Eitan C; Hornstein E; Ehilak E; Celadova P; Bose D; Farhan S; Fishilevich S; Lancet D; Morrison KE; Shaw CE; Al-Chalabi A; Blair I; Wray N; Kiernan M; Neto MM; Chio A; Cauchi R; Robberecht W; van Damme P; Corcia P; Couratier P; Hardiman O; McLaughlin R; Gotkine M; Drory V; Ticozzi N; Silani V; Veldink J; van den Berg L; de Carvalho M; Pardina JM; Povedano M; Andersen P; Wber M; Başak N; Shaw C; Shaw P; Morrison K; Landers J; Glass J; Veldink JH; Kirby J; Snyder MP; Shaw PJ, 2020, 'Rare Variant Burden Analysis within Enhancers Identifies CAV1 as an ALS Risk Gene', Cell Reports, 33, http://dx.doi.org/10.1016/j.celrep.2020.108456

Farrar MA; Kiernan MC, 2020, 'Spinal muscular atrophy — the dawning of a new era', Nature Reviews Neurology, 16, pp. 593 - 594, http://dx.doi.org/10.1038/s41582-020-00410-7

Farrar MA; Kiernan MC, 2020, 'Treating adults with spinal muscular atrophy with nusinersen', Journal of Neurology, Neurosurgery and Psychiatry, 91, pp. 1139, http://dx.doi.org/10.1136/jnnp-2020-324036

Tammi SM; Tounsi WA; Sainio S; Kiernan M; Avent ND; Madgett TE; Haimila K, 2020, 'Next-generation sequencing of 35 RHD variants in 16 253 serologically D- pregnant women in the Finnish population', BLOOD ADVANCES, 4, pp. 4994 - 5001, http://dx.doi.org/10.1182/bloodadvances.2020001569

Su L; Hironaka CE; Chen FY; Couper GS; Kiernan MS; Kawabori M, 2020, 'Untwist the twist: instant hemodynamic improvement in known HeartMate 3 complication', JOURNAL OF ARTIFICIAL ORGANS, 24, pp. 365 - 367, http://dx.doi.org/10.1007/s10047-020-01220-w

Kiernan MC; Shefner JM; Kaji R; Burke D, 2020, 'Amyotrophic lateral sclerosis: A new diagnostic paradigm', Journal of Neurology, Neurosurgery and Psychiatry, 91, pp. 903 - 904, http://dx.doi.org/10.1136/jnnp-2020-324006

Tracy JA; Taylor BV; Kiernan M; Dyck PJ; Crum BA; Mauermann ML; Amrami KK; Spinner RJ; Dyck PJB, 2020, 'Nerve Pathology Distinguishes Focal Motor Chronic Inflammatory Demyelinating Polyradiculoneuropathy from Multifocal Motor Neuropathy', Journal of Clinical Neuromuscular Disease, 22, pp. 1 - 10, http://dx.doi.org/10.1097/CND.0000000000000279

Timmins HC; Li T; Kiernan MC; Baron-Hay S; Marx G; Boyle F; Goldstein D; Park SB, 2020, 'Taxane-induced peripheral neuropathy: differences in patient report and objective assessment', Supportive Care in Cancer, 28, pp. 4459 - 4466, http://dx.doi.org/10.1007/s00520-020-05299-y

Shefner JM; Al-Chalabi A; Baker MR; Cui LY; de Carvalho M; Eisen A; Grosskreutz J; Hardiman O; Henderson R; Matamala JM; Mitsumoto H; Paulus W; Simon N; Swash M; Talbot K; Turner MR; Ugawa Y; van den Berg LH; Verdugo R; Vucic S; Kaji R; Burke D; Kiernan MC, 2020, 'A proposal for new diagnostic criteria for ALS', Clinical Neurophysiology, 131, pp. 1975 - 1978, http://dx.doi.org/10.1016/j.clinph.2020.04.005

Li T; Timmins HC; King T; Kiernan MC; Goldstein D; Park SB, 2020, 'Characteristics and risk factors of bortezomib induced peripheral neuropathy: A systematic review of phase III trials', Hematological Oncology, 38, pp. 229 - 243, http://dx.doi.org/10.1002/hon.2706

Besomi M; Hodges PW; Clancy EA; Van Dieën J; Hug F; Lowery M; Merletti R; Søgaard K; Wrigley T; Besier T; Carson RG; Disselhorst-Klug C; Enoka RM; Falla D; Farina D; Gandevia S; Holobar A; Kiernan MC; McGill K; Perreault E; Rothwell JC; Tucker K, 2020, 'Consensus for experimental design in electromyography (CEDE) project: Amplitude normalization matrix', Journal of Electromyography and Kinesiology, 53, http://dx.doi.org/10.1016/j.jelekin.2020.102438

Timmins HC; Li T; Huynh W; Kiernan MC; Baron-Hay S; Boyle F; Goldstein D; Park SB, 2020, 'Electrophysiological and phenotypic profiles of taxane-induced neuropathy', Clinical Neurophysiology, 131, pp. 1979 - 1985, http://dx.doi.org/10.1016/j.clinph.2020.02.028

Edwards LS; Halmagyi GM; Mallawaarachchi A; Thompson EO; Kiernan MC, 2020, 'Fatal cerebellar oedema in adult Leigh syndrome', Practical Neurology, 20, pp. 338 - 339, http://dx.doi.org/10.1136/practneurol-2019-002409

Howells J; Sangari S; Matamala JM; Kiernan MC; Marchand-Pauvert V; Burke D, 2020, 'Interrogating interneurone function using threshold tracking of the H reflex in healthy subjects and patients with motor neurone disease', Clinical Neurophysiology, 131, pp. 1986 - 1996, http://dx.doi.org/10.1016/j.clinph.2020.03.028

Upadhyay V; Katz M; Kiernan M; Henderson R, 2020, 'Review of the revised amyotrophic lateral sclerosis diagnostic criteria', Clinical Neurophysiology, 131, pp. 1767 - 1768, http://dx.doi.org/10.1016/j.clinph.2020.05.008

Kiernan MC; Cornblath DR, 2020, 'Subacute sensory neuronopathy and cancer: The identification of paraneoplastic syndromes', Journal of Neurology, Neurosurgery and Psychiatry, 91, pp. 793 - 794, http://dx.doi.org/10.1136/jnnp-2020-323171

Tu S; Wang C; Menke RAL; Talbot K; Barnett M; Kiernan MC; Turner MR, 2020, 'Regional callosal integrity and bilaterality of limb weakness in amyotrophic lateral sclerosis', Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 21, pp. 396 - 402, http://dx.doi.org/10.1080/21678421.2020.1733020

Mahoney CJ; Dharmadasa T; Huynh W; Halpern JP; Vucic S; Mowat D; Kiernan MC, 2020, 'A novel phenotype of hereditary spastic paraplegia type 7 associated with a compound heterozygous mutation in paraplegin', Muscle and Nerve, 62, pp. E44 - E45, http://dx.doi.org/10.1002/mus.26882

Kiernan MC, 2020, 'The Journal of Neurology, Neurosurgery and Psychiatry centenary milestone award 2020', Journal of neurology, neurosurgery, and psychiatry, 91, pp. 677, http://dx.doi.org/10.1136/jnnp-2020-322976

Abate W; Alrammah H; Kiernan M; Tonks AJ; Jackson SK, 2020, 'Lysophosphatidylcholine acyltransferase 2 (LPCAT2) co-localises with TLR4 and regulates macrophage inflammatory gene expression in response to LPS', SCIENTIFIC REPORTS, 10, http://dx.doi.org/10.1038/s41598-020-67000-x

Ahmed RM; Devenney EM; Strikwerda-Brown C; Hodges JR; Piguet O; Kiernan MC, 2020, 'Phenotypic variability in ALS-FTD and effect on survival', Neurology, 94, pp. E2005 - E2013, http://dx.doi.org/10.1212/WNL.0000000000009398

Menon P; Higashihara M; van den Bos M; Geevasinga N; Kiernan MC; Vucic S, 2020, 'Cortical hyperexcitability evolves with disease progression in ALS', Annals of Clinical and Translational Neurology, 7, pp. 733 - 741, http://dx.doi.org/10.1002/acn3.51039

Higashihara M; Van den Bos MAJ; Menon P; Kiernan MC; Vucic S, 2020, 'Interneuronal networks mediate cortical inhibition and facilitation', Clinical Neurophysiology, 131, pp. 1000 - 1010, http://dx.doi.org/10.1016/j.clinph.2020.02.012

Kiernan MC, 2020, 'Queen Square: a history of the National Hospital and its Institute of Neurology', JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 91, pp. 560 - 561, http://dx.doi.org/10.1136/jnnp-2020-322776

Vucic S; Higashihara M; Sobue G; Atsuta N; Doi Y; Kuwabara S; Kim SH; Kim I; Oh KW; Park J; Kim EM; Talman P; Menon P; Kiernan MC, 2020, 'ALS is a multistep process in South Korean, Japanese, and Australian patients', Neurology, 94, pp. E1657 - E1663, http://dx.doi.org/10.1212/WNL.0000000000009015

Weiss M; Macklin E; McIlduff C; Vucic OS; Wainger B; Kiernan M; Goutman S; Goyal N; Rutkove S; Ladha S; Chen A; Harms M; Brannagan T; Lacomis D; Zivkovic S; Ma M; Wang L; Simmons Z; Rivner M; Shefner J; Cudkowicz M; Atassi N, 2020, 'Effects of Mexiletine on Cortical and Peripheral Nerve Hyperexcitability in Sporadic ALS (2531)', Neurology, 94, http://dx.doi.org/10.1212/wnl.94.15_supplement.2531

Kandula T; Farrar MA; Cohn RJ; Carey KA; Johnston K; Kiernan MC; Krishnan AV; Park SB, 2020, 'Changes in long term peripheral nerve biophysical properties in childhood cancer survivors following neurotoxic chemotherapy', Clinical Neurophysiology, 131, pp. 783 - 790, http://dx.doi.org/10.1016/j.clinph.2019.12.411

Dharmadasa T; Matamala JM; Howells J; Vucic S; Kiernan MC, 2020, 'Early focality and spread of cortical dysfunction in amyotrophic lateral sclerosis: A regional study across the motor cortices', Clinical Neurophysiology, 131, pp. 958 - 966, http://dx.doi.org/10.1016/j.clinph.2019.11.057

Turner MR; Barohn RJ; Corcia P; Fink JK; Harms MB; Kiernan MC; Ravits J; Silani V; Simmons Z; Statland J; Van Den Berg LH; Mitsumoto H, 2020, 'Primary lateral sclerosis: Consensus diagnostic criteria', Journal of Neurology, Neurosurgery and Psychiatry, 91, pp. 373 - 377, http://dx.doi.org/10.1136/jnnp-2019-322541

Huynh W; Sharplin LE; Caga J; Highton-Williamson E; Kiernan MC, 2020, 'Respiratory function and cognitive profile in amyotrophic lateral sclerosis', European Journal of Neurology, 27, pp. 685 - 691, http://dx.doi.org/10.1111/ene.14130

Wan HWY; Carey KA; D'Silva A; Vucic S; Kiernan MC; Kasparian NA; Farrar MA, 2020, 'Health, wellbeing and lived experiences of adults with SMA: A scoping systematic review', Orphanet Journal of Rare Diseases, 15, pp. 70, http://dx.doi.org/10.1186/s13023-020-1339-3

Huynh W; Ahmed R; Mahoney CJ; Nguyen C; Tu S; Caga J; Loh P; Lin CSY; Kiernan MC, 2020, 'The impact of cognitive and behavioral impairment in amyotrophic lateral sclerosis', Expert Review of Neurotherapeutics, 20, pp. 281 - 293, http://dx.doi.org/10.1080/14737175.2020.1727740

Mahoney CJ; Kiernan MC, 2020, 'Expanding the availability of medications for amyotrophic lateral sclerosis in Australia', Medical Journal of Australia, 212, pp. 189 - 189.e1, http://dx.doi.org/10.5694/mja2.50482

Swash M; Burke D; Turner MR; Grosskreutz J; Leigh PN; Decarvalho M; Kiernan MC, 2020, 'Occasional essay: Upper motor neuron syndrome in amyotrophic lateral sclerosis', Journal of Neurology, Neurosurgery and Psychiatry, 91, pp. 227 - 234, http://dx.doi.org/10.1136/jnnp-2019-321938

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