FTD (frontotemporal degeneration or frontotemporal dementia) refers to a group of disorders that causes progressive damage to the temporal and frontal lobes of the brain associated with personality, behavior and language. Loss of function in this area of the brain can lead to impulsive behavior and speech difficulties. Usually FTD does not affect the parts of the nervous system that control muscle movement, but about 10-15% of people with FTD also experience motor neuron degeneration called FTD with motor neuron disease (FTD/MND) or FTD with ALS. Over the past 15 years, doctors and scientists’ knowledge of the connection of these diseases has rapidly grown through genetic discovery, brain imaging studies and biomarker studies. Specifically, researchers were able to confirm the connection between FTD and ALS when the TAR DNA-binding protein 43 (TDP-43) was identified as the central protein in both ALS and the most common type of FTD. Additionally, up to 40% of FTD cases have been found to carry a C9orf72 gene mutation, which is most common in genetic causes of ALS.
In many cases, FTD symptoms are noted prior to the diagnosis of ALS. FTD symptoms include:
- increase in inappropriate actions
- loss of empathy and other interpersonal skills
- lack of judgment and inhibition
- repetitive compulsive behavior
- decline in personal hygiene
- changes in eating habits
- lack of awareness of thinking or behavioral problems
- difficulty in using and understanding written and spoken language
- loss of knowledge of word meaning
Paula Richard describes what it is like to experience a loved one living with the symptoms of FTD/ALS:
In 2015 my husband was finally given a diagnosis of ALS/FTD . ALS alone is a terrible disease, but to have FTD with it is awful. Seeing my loved one go through the everyday struggles is heartbreaking. The cognitive issues outweigh the ALS part sometimes. He has bad comprehension and doesn’t know right from wrong. His behavior is like taking care of a little kid. I’m always getting onto him to not do something or to do something. I know none of this is his fault, but dealing with it is very stressful and overwhelming. I get angry with him and then I feel guilty. In addition to having FTD , he has also has PBA ( excessive laughing). When he laughs it’s like a kid laughing at you. He can embarrass you. My loved one is slowly having his life taken away from him. Not being able to do the things he loved to do and just everyday things. Having a support system is very important, having patience and understanding is a must, but very challenging. Life is so very different now, but having God in our lives helps. We just try and take one day at a time.
As Paula mentions, FTD with ALS creates additional challenges for caregivers and it is important to have a strong support system and for caregivers to be able to acknowledge the changes in the behavior and thinking of their loved one.
Some helpful recommendations for caregivers include:
- Simplify communication with the affected person. Break up sentences into short phrases. Ask yes/no questions. Slow down when speaking.
- Provide supervision and accompany the person to all appointments to make sure information is accurately relayed and retained.
- Set realistic expectations for the person with ALS and set realistic expectations for yourself. If your requests introduce frustration, irritability or withdrawal for either you or the person with ALS, your expectations need to be modified and most likely simplifed to meet the needs of your loved one’s current thinking abilities. Also, if you are feeling overwhelmed do not wait until you are feeling underwater. Think ahead to what options you have for help or time away, if needed.
- Educate providers and caregivers about where to set expectations for your loved one. In an ideal world, all healthcare providers would be on the same page about what to do and how to accomplish it. But many healthcare provides outside of dedicated ALS clinics may not have much experience with ALS/FTD and how it affects people.
- Continue to enjoy activities that bring joy and can be conducted safely, while refraining from activities the result in stress or risk of safety or liability.
While FTD with ALS is rare, current research suggests that up to 50% of people with ALS might experience some degree in change in thinking and behavior. It is important for all who are impacted by ALS to know that cognitive impairment is possible and how to help those affected adjust to the changes. For more information on FTD visit The Association for Frontotemporal Degeneration.