Professor Matthew Kiernan
Bushell Chair of Neurology
Medicine, Central Clinical School
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Professor Matthew Kiernan was recently appointed as the Bushell Chair of Neurology at the University of Sydney. His clinical research unit is now located at the Brain and Mind Institute. He was appointed Professor of Neurology, Royal Prince Alfred Hospital (RPAH) in 2013 and Senior Staff Specialist RPAH. He is also a Senior Scientist at Neuroscience Research Australia Professor Kiernan is the head of the Clinical Neuroscience research group comprising 20-strong team of clinicians, scientists, biomedical engineers, doctoral & postdoctoral students with focus on neurological disease. His research team's focus is clinical neurology, in particular disease pathophysiology and treatment strategies of frontotemporal dementia and motor neurone sydnromes. Currently his team is investigating the mechanisms and the possible prevention of neurodegeneration in motor neurone disease; frontotemporal dementia; chemotherapy-induced neurotoxicity; stroke; Machado-Joseph disease; spinal muscular atrophy and other inherited neuropathies. We are also involved in clinical trials investigating potential drug treatments for motor neurone disease, multiple sclerosis and chronic inflammatory demyelinating polyneuropathy. His team's research is intrinsically linked to the provision of clinical services, particularly the ForeFront Multidisciplinary Motor Neurone Disease & Fronto temporal Dementia Clinic and diagnostic neurophysiology clinics. Professor Kiernan is the Editor-in-Chief of the Journal of Neurology, Neurosurgery and Psychiatry (BMJ Publishing Group). Professor Kiernan is Vice President of the Australian Brain Foundation, the largest priming institution for neuroscience in Australia, responsible for distributing funding towards research and medical education for the treatment and prevention of neurological disorders.
Clinical Neurophysiology - peripheral nerve excitability and cortical nerve excitability
Frontotemporal Dementia and Motor Neurone Syndromes
- Site of onset
- Mechanism of disease
- Progression and spread of disease
- Novel treatment strategies including regenerative medicine
IMAGING OF NERVE AND MUSCLE INJURY IN NEUROLOGICAL DISEASE
Recent advances in imaging technology have generated the potential to visualise the peripheral nervous system in increasing detail. These advances include the development of portable high resolution ultrasound units and high field strength magnetic imaging (MRI) scanners. The aim of this study is to utilise these advances in imaging technology to develop non-invasive biomarkers of muscle denervation in motor neurone disease. Existing neurophysiological measures of peripheral nerve injury will be correlated with peripheral nerve imaging modalities.
BRAIN AND SPINAL CORD DONATION FOR MOTOR NEURONE DISEASE RESEARCH
In order to develop effective interventions for people with MND, more information is required on the cellular causes and how genetic variation affects the cellular mechanisms. At present the changes in the brain and spinal cord cannot be predicted by either the type of symptoms a person has, or by their genetic profile. The only way to determine the cellular changes at present is with a brain and spinal cord autopsy.
Ultimately our goal is to find a cure for MND. Our current research goal is to understand the type of cellular changes occurring in the brain and spinal cord, and by studying patients with different clinical and genetic subtypes of MND, we will be able to determine if there is a single type of change, or a variety of underlying cellular changes that will require treatment. To determine treatable cellular causes, it is essential that brain tissue be obtained to elucidate the cellular changes that are associated with the different clinical and genetic subtypes of MND.
AUSTRALIAN MOTOR NEURONE DISEASE REGISTRY
The Australian Motor Neurone Disease Registry was launched by Professor Matthew Kiernan on Motor Neurone Disease Global Awareness Day, 21 June 2005, at the Annual Conference for Health and Community CareProviders.
The Australian Motor Neurone Disease Registry is a clinical database that provides a means to facilitate the collection and analysis of Motor Neurone Disease patient data such as demographics, site of onset, diagnosis data, treatment type, changes in functional capacity, complications related to disease progression and the impact of new treatments and interventions for Motor Neurone Disease. The goals of the registry are to improve patient care through continuous evaluation of patient management and associated outcomes and to form significant scientific research collaborations with organisations and individuals to further the understanding of Motor Neurone Disease.
Visithttp://www.amndr.org for more information.
ASSESSMENT OF NERVE AND CORTICAL EXCITABILITY IN HEREDITARY AND ACQUIRED NEUROLOGICAL DISORDERS
The aim of this study is to investigate the primary pathogenesis, development, rate of progression and magnitude of nerve degeneration in a number of hereditary and acquired neurological diseases using nerve and cortical excitability studies. Furthermore we wish to make comparisons according to clinical and genetic distinctions.
CLINICAL ASSESSMENT OF FUNCTIONAL DISABILITY IN MOTOR NEURON DISEASE
The purpose is to investigate the contributions of motor dysfunction as well as possible changes in thinking and reasoning on the performance of everyday life tasks. This project aims to assess the practical impact of Motor Neurone Disease (MND) on both patients and their family members. Cognitive ability and behavioural changes are measured in a longitudinal fashion after 6 and 12 months with the aim of investigating how these changes can affect disease progression, prognosis and provision of care. In conjunction the entire neural/motor axis in MND will be assessed using Transmagnetic Stimulation, peripheral axonal excitability studies and brain imaging techniques to examine how structural changes relate to everyday disability.
PATIENT DECISION MAKING IN MOTOR NEURON DISEASE
This study is exploring the barriers and facilitators to the delivery of patient-centred care in a terminal and progressive disease, and in chronic disease self-management. The aim of this study if to investigate what influences patients’ decision-making for symptom management and quality of life. This research is important as poorly timed decision-making impacts on MND patients’ health and quality of life.
IDENTIFYING CLINICAL MARKERS OF C9ORF72 MUTATION CARRIERS
To define the clinical, neuropsychological, behavioural and imaging features ofC9orf72mutation carries in comparison with non-carriers with the aim to identify the phenotype of the C9orf72 mutation in patients with motor neurone disease, frontotemporal dementia and FTD-MND
EATING, AUTONOMIC AND SEXUAL DYSFUNCTION IN FRONTOTEMPORAL DEMENTIA AND MOTOR NEURONE DISEASE
This project aims to examine the incidence and underlying causes of eating, autonomic and sexual dysfunction in motor neuron disease (MND) and frontotemporal dementia (FTD). Eating abnormalities have been well documented in FTD, this study aims to extend this work into MND. There is increasing evidence for an overlap between FTD and MND at a clinical and pathological level which has gained impetus with the recent discovery of the C9orj72 gene mutation in cases with familial FTD, FTD-MND and MND.
The term frontotemporal dementia (FTD) refers to a group of neurodegenerative disorders characterised by shrinkage of the frontal and anterior temporal lobes of the brain. FTD presents with changes in personality and behaviour and/or progressive language problems. Change in eating patterns including reduced satiety, binge eating, increased carbohydrate consumption and stereotypic eating is a major criterion for the diagnosis of behavioral variant FTD. It is not known whether these same eating abnormalities are present in MND, but given the increasing evidence of an overlap, it is possible that they are. In FTD patients it has been found that those with eating abnormalities, have shrinkage of the posterior hypothalamus. The hypothalamus also controls the autonomic nervous system, which regulates internal bodily functions such as blood pressure control, stomach, bowel and urinary function. The hypothalamus is also involved in controlling sexual function. This project aims to examine the incidence of eating, autonomic and sexual symptoms in MND and FTD and by imaging methods and endocrine analysis examine the possible underlying causes and then possible interventions to reduce the impact of these symptoms on the quality of life of patients and carers.
BIOMARKERS FOR MOTOR NEURONE DISEASE
Motor Neurone Disease is an incurable neurodegenerative condition, usually resulting in death within 2-3 years of symptom onset. Currently available therapies act to prolong life expectancy only by around 3 months. This study will explore all the major pathological proteins present in body fluids in the different clinical, pathological and genetic subtypes of MND. The ultimate aim is to develop a biomarker platform for differentiating these subtypes and to track the changes in the levels of these proteins over time. The identification of biomarkers may allow early detection and characterization of MND and its pathological and genetic subtypes, and assist with monitoring future mechanistic therapies.
SENIOR NEUROLOGIST, Institute of Clinical Neurosciences, Royal Prince Alfred Hospital
EDITOR-IN-CHIEF, Journal of Neurology, Neurosurgery and Psychiatry (BMJ Publishing Group, UK)
VISITING MEDICAL OFFICER, Prince of Wales Hospital
SENIOR STAFF SPECIALIST, Prince of Wales Hospital
SENIOR PRINCIPAL RESEARCH SCIENTIST, Neuroscience Research Australia
CONJOINT PROFESSOR OF MEDICINE, Faculty of Medicine, University of New South Wales
CONSULTANT NEUROLOGIST AND NEUROPHYSIOLOGIST, Sydney Children’s Hospital
VICE PRESIDENT, Australian Brain Foundation
DIRECTOR, Motor Neurone Disease Research Institute of Australia
DIRECTOR, Neuroscience Trials Australia
MEDICAL ADVISOR, Motor Neurone Disease Association
MEDICAL ADVISOR, Muscular Dystrophy Association
MEDICAL REFERENCE GROUP, Machado Joseph Disease Foundation
MEMBER, Greater Metropolitan Transitional CareTaskforce
MEMBER, Australian and New Zealand Association of Neurologists Council
MEMBER, Australasian College of Physicians
Awards and honours
2013 Recipient RG Menzies Foundation Medal for Medical Research
2012 Inaugural Paul Brock Fellowship Award, NSW Office of Science and Medical Research, for Clinical Translation
2011 UNSW Inventor of the Year Awards 2011-runner up
2010 M.A. Brazier Prize to supervised Ph.D. student (Dr Steve Vucic, International Federation of Critical Neurophysiology)
2008 Eric Susman Prize, The Royal Australian College of Physicians
2009 2006 James G. Golseth Award, to supervised Ph.D. student (Dr Steve Vucic), American Association of Electrodiagnostic Medicine
2004 James G. Golseth Award, to supervised Ph.D. student (Dr Arun Krishnan), American Association of Electrodiagnostic Medicine
2003 Australian Physicians Independent Committee Neuroscience Award
2003 Leonard Cox Award for Excellence in Neuroscience Research, Australian and New Zealand Association of Neurologists
2002 Clinical Investigatorship, Viertel Charitable Foundation
2003 Clinical Research Award, Ramaciotti Foundation
1999-2002 CJ Martin Travelling Fellowship, National Health and Medical Research Council of Australia (top applicant in Australia)
1999-2002 RG Menzies Fellow, The Sir Robert Menzies Memorial Foundation (NHMRC Travelling Fellow)
1998 JJ Billings Overseas Travelling Fellowship, Royal Australasian College of Physicians
1998 Australian Association of Neurologists Overseas Training Position, The National Hospital for Neurology and Neurosurgery, London, UK
1997 Travelling Fellowship of the International Federation of Clinical Neurophysiology
1996 The Young Investigator Award, Annual Scientific Meeting of the Australian Association of Neurologists
1995 Royal Australasian College of Physicians NSW State Committee Advanced Trainees Award for Best Research
1995 National Health and Medical Research Council Medical Postgraduate Research Scholarship
1994 Faculty of Medicine Scholarship, University of New South Wales
1994 Whitmont Fellowship, Australian Brain Foundation
In the media
Prof Kiernan welcometo University of Sydney http://sydney.edu.au/bmri/news/2013/kiernan.php
Kiernan Group page at NeuRA http://www.neura.edu.au/research/themes/kiernan-group
JNNP BMJ Twitter https://twitter.com/jnnp_bmj
- Motor Neuron Disease: A multidisciplinary approach to unravel the clinical phenotypes, and to improve diagnosis and management outcomes; Huynh W, Kiernan M; DVC Research/Postdoctoral Research Fellowship Scheme.
- Dementia and motor neurone disease: Extending research and translating into clinical practice; Kiernan M; Research Grant/..
- Frontotemporal dementia and motor neurodegenerative syndromes; Halliday G, Gotz J, Ittner L, Kril J, Hodges J, Kiernan M; National Health and Medical Research Council (NHMRC)/Program Grants.