Motor neurone disease (MND) is a neurodegenerative disease that causes rapidly progressive muscle weakness. Specifically, the disease affects nerve cells (motor neurons) that control the muscles that enable you to move, speak, breathe and swallow.
MND is also known as Amyotrophic Lateral Sclerosis (ALS) or Lou Gehrig's disease. Approximately 1400 people in Australia are living with this disease. MND typically affects people in their mid-50s and survival is approximately 2-5 years from the onset of symptoms. Although there is currently no cure for MND, an anti-glutamatergic medication, riluzole, is available and slows the progression of the disease.
At Neuroscience Research Australia, we are trying to improve our understanding of what causes the neurons to die by studying patients with MND using novel electrical and magnetic tests. We also in the process of conducting a drug trial in the hope that it slows the progression of this devastating illness.
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.
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.
The Kiernan Group 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.
So far, our scientific discoveries have:
Within the Kiernan Group, we are currently running a drug trial for people diagnosed with MND.
Using the aforementioned neurophysiological techniques, we are also exploring the physiological changes in children who develop an early form of the MND, called spinal muscular atrophy.
In collaboration with the Hodges Group, we are investigating cognitive impairment and memory problems that some people with MND develop.