Motor neurone disease

EXTRA INFORMATION

Improving our understanding of the causes of MND

WHAT WE KNOW

Symptoms

The effects of MND vary greatly from person to person. MND may appear initially as a tingling or weakness in the hands and feet. Some people begin to stumble and can no longer hold objects in their hands easily. MND also affects the throat and tongue muscles, and some people with MND begin to slur their speech and have difficulty swallowing. In most cases, the disease eventually leads to widespread muscle wasting and weakness.

Common causes

The precise cause of MND and its disease process remains a complete mystery. Some researchers are looking into possible environmental triggers – such as exposure to toxins or electrical injury.

About our research

NeuRA is at the forefront of a new research field: the study of nerve excitability and its related disorders. We use novel physiological techniques to study the transmission of electrical signals through nerves so we can better understand why these signals fail in MND and related disorders.

Our hope is that with a better understanding of nerve physiology and function we may be able to provide new therapeutic strategies for neurological disorders such as MND. We are investigating the potential for new clinical tests to complement standard nerve conduction studies used in the diagnosis of MND.

Our research is intrinsically linked to local clinical services, particularly at the Prince of Wales Hospital Multidisciplinary MND Clinic located in Randwick, NSW. We also contribute to the Australian Motor Neurone Disease Registry.

What we have discovered

So far, our scientific discoveries have identified a number of potential new therapeutic targets. We were the lead Australian site for the GSK sponsored Study NOG112264 (a Phase II Study of Ozanezumab (GSK1223249) versus Placebo in the Treatment of MND).

Current projects

Patient-centered investigations

  • Cognition in MND
  • Brain Imaging in MND

Tissue based research

  • Neuropathology: these studies focus on understanding the internal cellular changes that occur in MND and how these relate to the structural imaging changes noted in life, and also to their genetic status. We are focussing on the TDP-43 protein and how it progresses through the brain in patients who have died at different stages of their disease. We are also assessing any changes in memory circuits and also in brain circuits important for movement and balance.
See what’s going on at NeuRA

FEEL THE BUZZ IN THE AIR? US TOO.

‘Progressive. Incurable. Terminal. Nothing matters… I am going to die.’

‘There are days that I just cry like a baby. I’m meant to be the provider, the strong one. No son should have to change the underwear of their 57 year old father.’ Shin Liu is a man you want to know... kind, articulate and with love in his heart. At 57 years old however, Shin has planned his funeral. Two years ago, Shin was diagnosed with motor neurone disease (MND). Unlike many cancers or heart disease, there is not a single thing the medical profession can do to stop MND. This excruciating disease twists and contorts the human body in the most horrific way, and it quickly destroys the ability to move, speak, swallow and breathe. Life expectancy post diagnosis is 2.5 years. But NeuRA researchers are making exciting progress toward it's defeat. After years of meticulous research, we've learnt that in more than 90% of MND cases a protein called TDP-43 is responsible for the changes in motor neurones. In pre-clinical (non-human) trials, we have found that this protein can be controlled by a specially engineered peptide sequence (i.e. medication) which has the potential to stop MND in its tracks. But here is the most exciting development… we are observing improvements in movement, behaviour and memory upon administering this medication! This is innovative, ground-breaking research and we need your help to accelerate this research, which will in time enable clinical trials in people living with MND. Will you support or research today?
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