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Although Huntington’s disease is commonly characterized as a movement disorder, this inherited condition is clinically defined by a triad of motor, cognitive and behavioral symptoms. The neuropsychiatric features often precede motor dysfunction.
“Dr. George Huntington himself noticed that people with Huntington’s chorea tend to be impulsive and compulsive and present with a lot of self-harming behavior,” says Kasia Rothenberg, MD, PhD, a geriatric neuropsychiatrist in Cleveland Clinic’s Lou Ruvo Center for Brain Health. “Now we understand it’s a combination of two things ― being quite disorganized in their thinking and having trouble with control regulation due to lack of executive function.”
Dr. Rothenberg collaborates with an interdisciplinary team caring for patients with Huntington’s disease at Cleveland Clinic’s Center for Neurological Restoration. In a recent episode of Cleveland Clinic’s Neuro Pathways podcast, she discusses working with this patient population on the neuropsychiatric challenges they face. Dr. Rothenberg covers:
Click the podcast player above to listen to the 25-minute episode now, or read on for a short edited excerpt. Check out more Neuro Pathways episodes at clevelandclinic.org/neuropodcast or wherever you get your podcasts. This activity has been approved for AMA PRA Category 1 Credit™ and ANCC contact hours. After listening to the podcast, you can claim your credit here.
Podcast host Glen Stevens, DO, PhD: Let’s talk a bit about the neuropsychiatric manifestations of Huntington’s disease ― when they start, their implications, those types of things.
Dr. Rothenberg: They start really early. And it can be a problem that certain symptoms may in fact go unnoticed. I don’t think we yet fully understand how the disease may present early on. We know for a fact that the degeneration in the brains of people with Huntington’s disease is something we notice mostly in certain areas, basically the basal ganglia, and this type of neurodegeneration is responsible for chorea. But we know by now through all kinds of pathological studies that the same type of degeneration happens in the frontal lobes, which means that certain types of behavioral changes may occur even earlier than chorea. I use the term “behavioral” rather broadly, in the sense of both psychiatric and cognitive symptoms.
Imagine someone with dysfunctional frontal lobes. This person may become disinhibited and their control is different than it should be. They become repetitive, obsessive, compulsive. There is a lack of top-down control of behavior. So, yes, psychiatric and cognitive symptoms start really early.
Dr. Stevens: So when should we be concerned that someone might have Huntington’s disease that may be manifesting only in terms of behavioral changes prior to any manifestations of a movement disorder?
Dr. Rothenberg: It’s not easy because so many different things might influence people’s behavior. But if we have a good suspicion that the genetic mutation may play a role here, we of course look for certain changes in human behavior. And we of course treat them symptomatically as early as possible.
There’s another aspect that can influence everything in Huntington’s disease, but predominantly behavior and other psychiatric symptoms, and that’s disordered sleep regulation. We rarely talk about it, but Huntington-type degeneration happens really early in certain brain areas that regulate our circadian rhythm. So, it may be good practice for us to pay attention to how people sleep, how they regulate themselves.