What are Neurodegenerative Disorders?
Neurodegenerative disorders cause progressive degeneration of nerve cells, impacting both movement and mental functioning. Progressive supranuclear palsy (PSP) is one such disease. (1)
People living with PSP may exhibit unsteady walking, slurred speech and emotional lability such as crying. Tears may either be real or pseudobulbar (uncontrollable and distressful tears that occur suddenly and unpredictably), known as pseudobulbar affect. (2)
Australia is home to approximately 80,000 individuals living with neurodegenerative disorders (3), many of whom suffer devastating consequences for themselves and their loved ones. Research is underway in an attempt to help delay these conditions, reduce symptoms and enhance quality of life for these people.
Brain changes caused by various disorders can result in depression, anxiety and impulsive behaviour in vulnerable adults. At The Florey, a team has devised a program to support these adults while alleviating distress and improving mental health.
As Australian society ages, it is critical that we find new treatments for neurodegenerative diseases. This project aims to model emergent properties of the brain to better understand disease development and uncover possible therapies.
Neurodegenerative diseases damage nerve fibres, activating SARM1 to make them self-destruct. This happens due to its programmed breakdown of essential helper molecules like nicotinamide adenine dinucleotide (NAD+).
Prof Ashley Bush and his team at The Florey (4) are currently exploring whether blood biomarkers can be used to detect dementia earlier. This would enable healthcare providers to quickly refer patients, make appropriate services available quickly and plan for future needs more effectively. A combination of biomarkers may prove more useful as this could help differentiate among different forms of dementia more accurately.
Neurodegenerative Diseases (ND) are progressive neurological conditions which deteriorate brain cells over time, leading to reduced cognitive, physical, and social functioning as well as increased care needs and support requirements. Common examples include dementia, Multiple Sclerosis, Parkinson’s Disease Motor Neurone Disease Huntington’s Disease. (5)
Our researchers are dedicated to understanding the causes of these conditions so we can prevent or diagnose more effectively and develop therapies to treat them – this is especially important as these diseases often progress over time and do not yet have cures.
At present, our work involves researching how the protein called synuclein breaks apart and aggregates to form toxic clumps that damage nerve cells leading to their death, thus contributing to progression of neurodegenerative disease. We aim to discover methods for inhibiting this aggregation process to halt progression.
An additional focus is identifying blood biomarkers that can accurately and quickly predict what diseases a person will contract and at what stage. This will enable us to create more rapid therapies.
Recently, A/Prof Anthony White (6) discovered that certain organic molecules could redistribute copper within neurons, leading him to discover CuATSM as a possible therapeutic that is being developed by Collaborative Medicinal Development (CMD), with Professor Crouch and Dr Barnham acting as Scientific Advisors for CMD. CuATSM has shown the ability to delay disease onset while improving motor function in mouse models of MND.
What Are The Causes Of Epilepsy?
In about half of all cases of epilepsy, there’s no known cause. But it can be caused by things like head injuries, dementia, brain tumours and some blood vessel problems. It can also be the result of certain genetic conditions, such as Mesial temporal sclerosis (a scar that forms in part of the brain near the ear).
Seizures are sudden bursts of electrical energy that disrupt the rhythmic electrical impulses that pass between cells in different parts of your brain. This causes changes in awareness (including loss of consciousness), sensations and muscle movements. They can vary in length and severity. Your healthcare provider will classify your seizures based on where the electricity starts, how long you are aware of them and whether or not you have muscle movement.
If you have a seizure, your doctor will examine you and ask about your medical history. They may recommend a test called an electroencephalogram (EEG) to check the electrical patterns in your brain. They might also do a brain scan to look for things like a brain tumour or abnormal blood vessels.
Keeping a diary of your seizures can help you and your healthcare provider identify triggers. This is especially helpful if you have breakthrough seizures (sudden, uncontrolled seizures in people who have been taking their medications properly). This can help you and your healthcare provider decide if you need to change your medicine or get surgery. The most common triggers are fever, sleep deprivation and missing a dose of your medication.
Frequently Asked Questions
What are the most common neurodegenerative diseases?
Neurodegenerative diseases are neurological conditions that primarily impact your brain and spinal cord, gradually worsening over time and having a profound effect on your life. While some forms may affect movement (Pinker’s Disease, Progressive Supranuclear Palsy or Corticobasal Degeneration are examples), others can lead to cognitive decline and memory loss.
Neurodegenerative diseases typically result in a gradual decrease in mental functioning due to neurons dying throughout your brain. Alzheimer’s, amyotrophic lateral sclerosis (ALS or Lou Gehrig’s Disease), motor neurone disease and Huntington’s are examples of diseases which affect nerve cells within the brain and nervous system.
Neurodegenerative disorders that impact movement may benefit from deep brain stimulation surgery to alleviate their symptoms and slow progression, in addition to medications which may provide temporary relief from certain side effects.
Dr Ashley Bush https://florey.edu.au/researcher/ashley-bush/ and A/Prof Peter Crouch https://findanexpert.unimelb.edu.au/profile/17926-peter-crouch from the University of Melbourne are exploring potential therapeutics to treat neurodegenerative diseases, specifically dementia and ALS. Through research they have discovered that improper protein formation in the brain can contribute to dementia and ALS development; treating inflammation early could prevent such conditions from ever arising.
What are the main causes of neurodegenerative disease?
Neurodegenerative diseases occur when neurons that make up your nervous system become damaged or die off, resulting in symptoms that impact how you think, move, or breathe. Neurodegenerative conditions tend to worsen over time and are incurable.
Neurodegenerative diseases vary depending on which part of the brain they affect; examples include Alzheimer’s and Parkinson’s diseases that impact memory and movement respectively.
Neurodegenerative diseases don’t always have clear causes, but various risk factors can increase your chance of getting one – including genetics, infections and exposure to toxic materials. Ageing itself may also increase your likelihood of getting neurodegenerative conditions.
Many individuals with neurodegenerative conditions require assistance in order to take care of themselves. They may require feeding, bathing and personal assistance as well as taking medications or being driven to appointments. As their disease advances, many may lose their independence and require full-time assistance.
Neurodegenerative conditions require individuals to have discussions with loved ones about what they want in the event they can no longer take care of themselves, which may be difficult, yet these conversations are essential to creating an atmosphere of preparedness for everyone involved.
Writing down your wishes can also be helpful when making healthcare decisions on behalf of someone who cannot speak for themselves, such as family, friends or health professionals. Taken as part of advance care planning, this step may enable your loved ones and healthcare providers to make the best possible decisions on your behalf if needed.
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- Progressive Supranuclear Palsy February 01, 2021; Rodger J. Elble, MD, PhD, Professor of Neurology, Director, Parkinson Disease and Movement Disorders Center, Southern Illinois University School of Medicinehttps://rarediseases.org/rare-diseases/progressive-supranuclear-palsy/
- Online Reference Mayo Clinic https://www.mayoclinic.org/diseases-conditions/pseudobulbar-affect/symptoms-causes/syc-20353737
- The Walter and Eliza Hall Institute of Medical Research Victoria 2023 https://www.wehi.edu.au/research/diseases/neurodegenerative-disorders/
- The Florey, The Oxidation Biology Group, Mental Health and Dementia Research by Professor Ashley Bush MBS, DPM, FRANZCP, PhD, FTSE, FAAHMS, FAPA 2023 ‘Exploring whether blood biomarkers can be used to detect dementia earlier”
- CSF ferritin in the clinicopathological progression of Alzheimer’s disease and associations with APOE and inflammation biomarkers Journal of Neurology, Neurosurgery and Psychiatry 2022 Ayton, S., Janelidze, S., Kalinowski, P., Palmqvist, S., Belaidi, A.A., Stomrud, E., Roberts, A., Roberts, B., Hansson, O. & Bush, A.I.
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