Eating and metabolism in frontotemporal dementia

When thinking about frontotemporal dementia, we often focus on cognitive and behavioural symptoms. Memory loss, personality changes, and trouble speaking and understanding language are among some of the more recognised FTD symptoms. However, there is a physical side to neurodegenerative illnesses that is the subject of Dr Rebekah Ahmed’s research at NeuRA.

Eating abnormalities are present in 6 out of 10 people with frontotemporal dementia. We set out to measure whether hunger and satiety (the sensation of feeling full) were responsible for differences in eating behaviour in these people.

DSC_9153 smallWe asked carers of patients with different types of dementia to complete a questionnaire about the patients’ eating behaviours. The results showed that people with the behavioural variant of FTD have increased appetite, abnormal eating habits (such as table manners), and increased preference for sweet foods, compared with patients with Alzheimer’s disease and to healthy controls. Patients with semantic dementia (the language variant of FTD) also have similar abnormalities, but to a lesser extent. Both groups of FTD patients have increased carbohydrate and sugar intake, and as a result, an increased body mass index (BMI).

Despite these abnormalities, the two FTD groups have similar ratings of hunger and satiety to controls, suggesting that their increased food intake is not simply related to increased appetite. Similar findings have emerged in people with genetic obesity syndromes and may be related to changes in the hypothalamus, the part of the brain that regulates appetite hormones.

Metabolism in FTD

Now that we have quantified these changes in eating behaviour and nutrient intake in FTD, the question arises of how they affect metabolism. In other words, are there changes in markers of healthy metabolism in the blood, such as insulin and cholesterol?

A second study by my colleagues and I looked at exactly this question.

We assessed FTD patients using a clinical interview, neurological exam, cognitive assessment (including a questionnaire on eating habits), MRI, and measurement of blood cholesterol and insulin. As in the first study, we compared these measurements with people with Alzheimer’s disease and healthy controls.

We found peripheral insulin resistance in FTD patients, and increased incidence of diabetes mellitus in patients with the behavioural variant subtype of FTD. These patients also had abnormal ratios of ‘good’ dietary fats in their blood. These changes may be due to the abnormal eating patterns that we found in the first study, coupled with a high BMI.

This study of peripheral metabolism in FTD represents the first study of its kind. It is the first step in understanding how energy metabolism may affect the process of neurodegeneration.

Linking metabolism and neurodegeneration

There are still some questions to be answered. It is known that abnormal energy metabolism, such as in glucose regulation, is detrimental in people with Alzheimer’s disease and Parkinson’s disease. However, people with motor neurone disease (MND), which is closely related to FTD, also have distinct abnormalities in cholesterol and other dietary fats (triglycerides), but with the critical difference that these abnormalities actually seem protective against the progression of MND. In fact, a clinical trial showed an increased likelihood of death in MND patients treated with an antidiabetic medication. The theory is that MND patients have a hypermetabolic state and that increased eating and higher cholesterol and insulin levels maintain the energy balance.

Dr Rebekah Ahmed

Dr Rebekah Ahmed

Future research will examine how energy metabolism affects disease progression and whether variations in metabolism are detrimental or protective. One hypothesis in FTD is that they may be detrimental in terms of cognition, but protective in terms of muscle health in those patients who also develop MND. In the long term, studies that also examine the effects of lifestyle, sleep and physical activity on individuals with genetic susceptibility to these neurodegenerative diseases, before they have developed disease symptoms, will increase our understanding of the role of peripheral metabolism on central and peripheral neurodegeneration.

A ride for my Dad

NeuRA volunteer Lizzi Mallett explains why she decided to participate in the Memory Cycle to Cambodia in March 2015 and why fundraising for NeuRA’s research is so important to her.

Six years ago my family and I said goodbye to my dad, John Mallett, as he slipped away from our world after suffering from the rapidly progressing motor neurone disease (MND) and frontotemporal dementia for five short years. His battle with these awful diseases started when I was 15 and he was only 58. He lived life each day as if it was his last. His thirst for life took us on a wild journey and he loved every second of it. It is because of my dad that I have decided to dedicate my life to supporting and helping NeuRA. At the beginning of the year I moved to Sydney from Perth and began volunteering with NeuRA’s Foundation team.

When the opportunity for the Memory Cycle came up, I thought that there was no way someone like me, who doesn’t cycle regularly, could do a challenge like this. We would be cycling from Vietnam to Cambodia, and I would have to fundraise a minimum of $3,500 while living on the other side of the country from my entire support network. However, my mother reminded me of my dad’s determination in life and how he purchased a bike when he was ill but could not ride it. He taught me that if I put my mind to something, that I could achieve anything! And by *anything*, I mean the time he sneakily planned a trip from Perth to Hampton Island all on his own when he had already lost the ability to speak and swallow – however, that’s a story for another time. With this determination in mind, I thought, ‘yes, I can do this, I’ll cycle for my dad’. After all, Dad was the one who taught me how to ride a bike and we used to do everything together. This is something he would like to have done and if he was still here with us today, I’m sure he would have signed up for the challenge too. So I signed up and started my fundraising page. Almost instantly, thanks to social media, I raised $1,000 in under a week. Whoa!! Who knew I could do that. I am extremely close to reaching my minimum fundraising goal. See, my dad was right! The mind works wonders; you can achieve anything when you put your mind to it. The Memory Cycle is the best way to fundraise for NeuRA as I get to travel, meet new people, do really cool things with the Inspired Adventurers, and work out, all at the same time.

I had an unbelievably close bond with my dad and it breaks my heart seeing people suffering from neurological diseases just like he did. If you have a desire to help NeuRA and make a difference to so many families suffering from the illnesses that NeuRA researches, then please donate or sign up for Memory Cycle and ride in Cambodia with me. We will have an absolute ball! If you would like to contribute and cannot join the cycle team (even though you really want to!) then you are more than welcome to give a little donation on my fundraising page, and follow my journey on my blog.

I hope to see your pretty little faces on NeuRA’s Memory Cycle team; otherwise, we appreciate every little cent that comes our way. NeuRA is making a huge difference to so many people’s lives, including my own family. Your donation will help NeuRA conquer neurological disease one step at a time.

Dad and I

Dad and I


Reasons to run

This Sunday, August 10, the Sydney City2Surf will include two groups of people who have more reasons than many to get up early and run to Bondi beach.

Evan Brownsmith is organising a team of eager accountants from PKF Lawler Tamworth and Walcha, NSW to run the City2Surf in support of motor neurone disease (MND) research.

PKF team cropped

The PKF Lawler team: (L – R) Blair Richards, Kelee Hawker, Evan Brownsmith, Michelle Higgerson and Nicole Parkinson

This terrible disease has recently been diagnosed in two of PKF Lawler’s longstanding clients, with a profound impact upon their families and friends and the whole team at PKF Lawler. Through our efforts, both running and fundraising, we hope to raise awareness in the Tamworth community of the impact of MND. By partnering with NeuRA we hope to provide valuable funds for the development of life-altering treatments for MND sufferers.

Like most people, I have a love-hate relationship with running. On a good day – when there is sun and very little wind, and it’s not too hot and not too cold, and you’re feeling fit and your legs feel strong, and you’re wearing the right outfit, and the scenery is good, and no-one is watching you, and you’ve got a really good album playing in your ears – running is the best thing in the world! There’s nothing better than the sense of achievement you get from pushing yourself further as your feet pound the pavement. On every other day, running sucks!!

Most of the time, running is really hard! That’s why we come up with so many excuses for not doing it. We like the idea of running, but often we don’t enjoy running itself. What we don’t think about each time we choose not to run, though, is how lucky we are to be able to run. Most sufferers of MND will lose the ability to run. This is the inspiration for team PKF Lawler in the City2Surf, and this is what motivates me to get off the couch, put my joggers on and make the most of my good health and good fortune.

For those who don’t know, MND is the name given to a group of diseases in which the motor nerve cells (neurons) controlling the muscles that enable us to move, speak, breathe and swallow, undergo degeneration and die. Motor function is controlled by the upper motor neurons in the brain that descend to the spinal cord; these neurons activate lower motor neurons, which exit the spinal cord and directly activate muscles. With no motor neurons to activate them, muscles gradually weaken and waste away. These effects are irreversible, and as MND sufferers progressively lose function, their quality of life is diminished and independence is undermined.

On August 10 this year my colleagues and I hope to make a significant difference for these people. We cannot directly improve the lives of MND sufferers, nor can we make it easier on their families. We cannot hope to cure this terrible disease ourselves, but we know that our modest contribution to NeuRA will help the very people who just might. Perhaps more importantly, through demonstrating a willingness to ‘give it a go’, we are setting a great example to the community of Tamworth. We want to show that it’s important to get out there and have a crack, not because we should, but because we still can.

Fundraising stall

Ashlee Schwennesen and Julianne McDougall raising funds with a fresh produce stall

You can donate to the PKF Lawler team here.


Simone Riley is participating in the City2Surf with her family to support stroke rehabilitation research and to honour her Dad, a proud City2Surfer.

dad and me

On July 21, 2013, my Dad, a man who lived a healthy, active, and happy lifestyle, was stopped in his tracks by unfortunate genetics and a nasty stroke.

A man who was 64 years young, who had run 20+ City2Surfs (his favourite event) and over 60 marathons, trekked in Nepal and to the top of Mt Kilimanjaro, and was training for a trek from France to Spain, Dad was now in for the biggest challenge yet. His was an atypical stroke, meaning that he didn’t suffer the ‘usual’ deficits in movement that stroke often inflicts, but he did lose his vision and began to experience major cognitive and memory deficiencies.

Simone’s dad in Nepal

As Dad’s hospital days turned into weeks, and then months, our hope went up and down like the rollercoaster going on in Dad’s head. On my first visit to the hospital, I walked into a dark room and was faced with a body on a mattress on the floor that was heavily sedated, as the hospital staff didn’t know what else to do! When Dad woke up every now and then, to walk and eat, our hearts broke when he didn’t recognise me, or even my Mum, his wife of 43 years! Thankfully, we found a Brisbane doctor who agreed to come to the Gold Coast to assess him. This was the first hope we had. After moving hospitals, adjusting his medication, and some wonderful rehabilitation, physiotherapy, and speech therapy, we gradually saw the man who ‘once was’ slowly coming back. He once again knew who Mum was, who I was, and also recognised his other daughters, his son, his grandkids, and other people important to him. After almost six months in hospital, my dad walked out with my mum and went home.

The start of his (and her) new lives involved major adjustments; Mum was now his full time carer and also unfortunately had to take on the role of rehabilitator. I felt compelled to do and find what I could to help them with the struggle to find appropriate post-hospital rehabilitation.

While researching stroke, I stumbled across NeuRA. What better way to honour my Dad than to join the NeuRA City2Surf team and make him proud; to run his favourite event, while raising much-needed funds for a charity that will continue research into stroke rehabilitation and one day perhaps even prevent stroke altogether?

It has almost been a year since Dad’s stroke. Things are far from perfect; he has good days and bad days. Only last week, when I sat holding his hand on the couch, he knew I was there, but couldn’t, for all his wanting and trying, get my name out. His vision will never come back, but through trial and error and ups and downs he is rising, admirably, to his challenges.

pb marathon

This year, along with my sister, my best friend (who is like Dad’s fourth daughter), my Mum, some cousins and lots of Dad’s friends, Dad will once again take the trip to Sydney to run/walk/shuffle/crawl/hop/skip/jump; in short, do whatever it takes to complete his annual City2Surf! Given that Dad really doesn’t like the fact that he missed it last year, participating in this year’s race is in itself an achievement that we will all enjoy, almost as much as the cold beer we share with him at the finish line.

You can donate to Simone’s fundraising effort here.

You can also donate to the 2014 NeuRA Runners team here.

The sensitive topic of brain donation

Brain donor program coordinator Lauren Bartley says while it can be difficult to talk to people about brain donation, it’s for a very important cause.

When I first began recruiting to the brain donor program at NeuRA and the Sydney Brain Bank, I found it difficult to broach such a sensitive topic with the participant and their family members. After all, how do you ask someone you have met just briefly if you can have access to their brain tissue after they pass away?

This is a discussion I’ve had to have with all our research participants – and one that I no longer shy away from. I know that every brain is valuable and have seen firsthand that every donation brings us closer to understanding more about dementia.

Sometimes there can be confusion over whether the tissue we collect will be transplanted into another human (which it’s not); other times, I’ve found that participants think their brains won’t be useful because of their cognitive impairments.

Lauren Bartley is a brain donor program coordinator at NeuRA.

It’s actually brain tissue from people with these very impairments that is helping scientists at NeuRA understand why proteins that cause dementia begin to deposit in some people’s brains and not others, and how this occurs.

In some cases, it’s difficult for a neurologist to determine if the patient is suffering from early onset Alzheimer’s disease (AD) or frontotemporal dementia (FTD). While clinically these dementia syndromes can appear similar, the brain tissue pathology is quite different. Looking at brain tissue has been essential for understanding the differences in pathology between AD and FTD.

Thanks to people who have donated their brain tissue in the past, we now know that the brain tissue of people with Alzheimer’s disease is marked with plaques formed by the beta-amyloid protein and tangled accumulations of the tau protein.

The tau protein also accumulates in frontotemporal dementia, depositing not in tangles but as inclusions inside brain cells called Pick bodies (FTD is also known as Pick’s disease). Some people with FTD also have pathological inclusions of other proteins such as TDP-43 or FUS.

“This is at the heart of what’s driving our research: we need to come up with new ways of accurately diagnosing dementia while a person is still living.”

Because of the heterogeneity of pathology in FTD, it’s impossible to predict which protein is responsible for the illness with the clinical tools we currently have at our disposal.

I can recall many times when participants were only found to have evidence of motor neurone disease (in addition to their dementia) during the autopsy process. There have been instances where we found participants who had been diagnosed with FTD actually had Alzheimer’s disease pathology and vice versa.

If we had known the true cause of their illness during life, they may have been able to access therapies or medicine to reduce the impact of their symptoms. This will become increasingly important as new therapies for dementia syndromes become available.

This is at the heart of what’s driving our research: we need to come up with new ways of accurately diagnosing dementia while a person is still living.

Helping us improve diagnosis during life is one of the reasons why brain donation is invaluable, and it’s why I’d like to thank each and every brain donor who I’ve had the privilege of working with at NeuRA.


More information about brain donation

While I am not able to accept brain donations from the general public, we do accept brain donations for AD and FTD research from people who have participated in research at our clinic. There are also circumstances where people who we have not seen in our clinic but have had a diagnosis from a neurologist/geriatrician and previous brain imaging (preferably MRI) can also be enrolled.

After the Sydney Brain Bank at NeuRA has finalised the report identifying the protein that caused the dementia, I send this report to the families and clinicians. The tissue donation is then used in ethically approved projects performed by medical researchers across Australia and the world.

If you are interested in finding out more about brain donation for medical research into AD and FTD, please contact me at

Beyond motor symptoms in MND

Motor neurone disease (MND), as discussed in previous posts, is not a disease of pure motor symptoms. MND can also affect one’s ability to perform complex judgments (e.g. financial decision-making) and leads to changes in behaviour (e.g. a person once very active and driven can become apathetic). These non-motor symptoms and behavioural changes often go unrecognised and underdiagnosed. In a recent study we investigated how these symptoms affect carers when compared to the more well-known motor symptoms of MND. Continue reading