Leading Irish Neurologist Receives International Award for Motor Neurone Disease Research

Posted on: 29 April 2009

The American Academy of Neurology (AAN) presented HRB Clinician Scientist, Professor Orla Hardiman, and Consultant Neurologist at Beaumont Hospital, Dublin and Clinical Professor of Neurology at Trinity College Dublin with the prestigious Sheila Essey Award. The award recognises people who have made significant contributions in the search for the cause, prevention of, and cure for Motor Neurone Disease and was presented on April 28th in Seattle.

One in every four hundred is at risk of developing Motor Neurone Disease (MND) during their lifetime.  Motor Neurone Disease causes a gradual degradation and death of motor neurones. The only available treatment increases survival by three months.

Professor Hardiman’s project has been funded through the years primarily by the Health Research Board, with support from the American Muscular Dystrophy Association, American ALS Association, the Irish Motor Neurone Disease Foundation, and the Irish Motor Neurone Disease Association.   Her team has made a number of significant discoveries and include:

o The discovery of a new gene for Motor Neurone Disease. Mutations in the gene ANG, (which codes for the protein angiogenin), lead to motor neurone death.  Hardiman’s research group is working with groups in Trinity College Dublin, and the University of Massachusetts to find new treatments based on the ANG work.

o The discovery that particular variations in genes can make certain populations more susceptible to Motor Neurone Disease than others.

o The discovery that Irish people with Motor Neurone Disease come from families where other neurodegenerative conditions such as Dementia and Parkinsons are more common.

o The finding that changes in thinking, memory and language (cognitive impairment) occurs in up to 50% of Irish MND patients, and that genes that cause cognitive impairment may be related to genes for MND.

Commenting on the significance of the research, Professor Hardiman said: “The more we understand about the genes that make us prone to neurodegeneration in general, the more likely we are to find new treatments.  The genetic make up of the Irish population is very similar and knowledge of the Irish genetic structure is advanced, so we are particularly well placed to conduct this type of research.”

Professor Hardiman and her research group are also studying the genetically diverse populations in the Caribbean, and comparing the results with the Irish data.  The results show that the frequency of MND is lowest in members of Caribbean population who are genetically most mixed.  “This indicates that mixing genes from different populations may help reduce the risk of Motor Neurone Disease,” she explains.     

On receiving the award, Professor Hardiman said: “This is a great honour for my research team made up of a highly talented group of young neurologists and scientists. Our work would not be possible without the support of our collaborators- notably Professor Dan Bradley at Trinity College’s School of Genetics and Microbiology and Dr Niall Pender, Director of Neuropsychology at Beaumont Hospital. This award will be used to help further develop our research with the overall aim of finding new pathways in Motor Neurone Disease that can be harnessed to develop new treatments.”

For media enquiries contact TCD Press Officer, Caoimhe Ní Lochlainn, tel: 8962310/ 087-9958014, [email protected].

Notes to Editors
The Sheila Essey Award of $25,000 which is used towards continuing research into the Motor Neurone Disease, Amyotrophic Lateral Sclerosis (ALS). is sponsored by the American Academy of Neurology and the ALS Association and is supported through the philanthropy of the Essey family.

The award was presented at the AAN Annual Meeting, one of the world’s largest gatherings of neurology professionals which takes place in the Washington State Convention and Trade Centre in Seattle.

Professor Hardiman is a Fellow of the American Academy of Neurology, an association of more than 21,000 neurologists and neuroscience professionals, dedicated to promoting the highest quality patient-centred neurologic care. She is a previous recipient of the AAN Palatucci Award for Advocacy.