Huntington Disease: When Apathy Meets Irritability

28 May 2024
Huntington's disease

This transcript has been edited for clarity.

Hi. I'm Dr Karen Anderson. I'm the director of the Huntington's Disease Care, Education and Research Center at Georgetown MedStar University Hospital. Today we're going to talk about Huntington disease and the apathy and irritability-aggression conundrum.

It is an interesting thing in Huntington's that some patients develop both apathy and irritability and aggression. Let's get started with some definitions.

Apathy is a disorder of motivation. People with apathy may have trouble getting started on tasks. They may have trouble completing tasks. They may even have difficulty with things in daily life, like getting dressed or eating, if they're not reminded and supervised. Irritability is an internal state where people become easily annoyed by small things.

Aggression is when someone has a physical reaction. This could be verbal threats. This could be anger and throwing things. It can even escalate into violence toward property or other people. So, how can patients have both of these conditions at the same time?

There are patients who have apathy and who sit on the couch all day. They may not do much. They may not interact with the family. But if they're asked to do something, they do become very irritable and aggressive. It can be a real problem figuring out how to treat these patients.

This happens because of the frontal lobe circuitry in Huntington disease. There's disruption of the caudate frontal connections, and this is why the patients can have both apathy with lowered motivation but also disinhibition, impulsivity, and aggression. So, how can we deal with these problems?

It's important to remember that patient caregiver education and family education can go a long way before you get to a pharmacologic intervention.

For apathy, it's important for families to look at the environment the patient is in. Is there too much going on? Is it a boring environment? Perhaps the shades are closed all day, there's nothing to look at in the room, and no one interacts with the patient. Perhaps the patient needs a little more stimulation. Maybe picture books can be brought in, or the shades can be opened so the patient can look out and see more nature, for example.

With irritability and aggression, the patient may have too much stimulation. It may be that the patient is overstimulated and is telling us this by reacting rather than using words and saying, "This is too much."

For example, a patient who has little dogs running around, grandchildren running around — it may just be too much and they may act out. It may be that there's not enough going on in the environment. Again, patients sometimes act out when this is going on. Perhaps there are times of day when everyone's very busy.

Maybe the family is trying to get dinner ready and the patient feels ignored. These are times when patients sometimes act out. People with Huntington's can have difficulty recognizing their internal states. They may not realize they're too hot, they're too cold, they're hungry, they're thirsty. Their sleep schedule may be disrupted by Huntington disease.

All of these are important considerations and good discussions to have with patients and their family members. When I think about treating apathy, usually my first step for a psychopharmacologic treatment is to take away medications. If the patient is on an SSRI antidepressant, I will try to taper that down or even stop it because that can make apathy much worse.

If a patient is on sedating medications, particularly antipsychotics or mood stabilizers, perhaps we can move those to the evening to make sleep better and also make the patient less apathetic during the day. For irritability and aggression, if we have enough time, if the patient is not in danger of harming himself or someone else in the family, we might try an antidepressant.

Usually, I'll start with an SSRI antidepressant. Bu keep in mind that those can take a month or two to really work. If we need to do something faster because someone's in danger of being harmed, then I might go with a small dose of an antipsychotic.

Olanzapine is one of my favorites because it can be more sedating; or small doses of risperidone, usually given a couple times each day. Mood stabilizers can also be really helpful — for example, valproate and carbamazepine. Lamotrigine sometimes is not quite as helpful for irritability and aggression.

In a patient who has both apathy and irritability and aggression, it really comes down to a balance and figuring out the best environment for that patient, looking at medications, figuring out the right balance of medications that don't make him or her too apathetic, but which also don't tend to cause the patient to become too irritable. Also important is educating the families and caregivers to understand what's going on with the patient, and that this is part of the condition.

Thank you so much for joining us today to talk about apathy and irritability and aggression in Huntington disease, and both the behavioral and psychopharmacologic interventions. I'm Dr Karen Anderson.

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