People with HD may consult with a psychiatrist, a neurologist and a psychologist for their diverse symptoms, but what they really need, says Hugh Rickards, FRCPsych, MD, is a “Huntingtonologist”: A doctor who has been trained in all the diverse manifestations of the disease, from motor control to emotional issues to cognitive changes. Rickards, who is consultant in neuropsychiatry and honorary professor at the University of Birmingham, says that the emotional and cognitive problems of HD tend to get underplayed by doctors and researchers—and it shouldn’t be that way.
Rickards is a neuropsychiatrist at a large clinic for people with HD, and is also involved in recruiting people for research, including Enroll-HD. His own interest in the emotional landscape of HD has led him to spot similarities between HD and Asperger’s syndrome, the autism-like developmental disorder involving difficulty understanding other people’s feelings and states of mind.
What got you interested in HD?
I’m always interested in things that are between mind and brain, and it’s difficult here to see where physical stops and mental starts: Mood, physical condition and cognition all mixed up together.
Why do you talk about HD as “acquired Asperger’s syndrome”?
I think there is increasing evidence that people with HD in mid age start to acquire the social and cognitive deficits of Asperger’s: The insistence on sameness, lack of empathy, difficulty in tests of “theory of mind,” and inability to understand people’s states of mind.
The main ones are the inability to understand other people’s states of mind, and inability to do future envisioning, that is to say: envisioning alternative scenarios apart from the one that is there at the moment. Both are all about the inability to imagine something that’s not happening right in front of me right now. That requires a sort of mind’s eye, and people with HD seem to lose that. It’s hard on people around them. In clinic, you might ask the patient how they’re doing and the patient says they’re fine. But the wife or husband sees the trouble. The patient doesn’t understand what’s happening—or the partner’s distress. These are fundamental deficits in HD, and they’re the most disabling.
It’s an interesting idea, but why does that matter for people in HD families?
It makes a massive difference. If you understand the mental state of HD, you can help carers manage better, which could mean the difference between them staying at home or being in a nursing home. For example, if you ensure that their environment is quite predictable, you might do quite well, whereas if you keep treating them as the same old person they used to be, you might end up in a lot of fights.
Why do you say that we should train doctors as “Huntingtonologists”?
If I ruled the world, we’d train people in the whole of HD: the complex mental changes, the complex motor and cognitive problems we see. I don’t think you can understand those things in isolation.
There’s no such thing as “just movement”; it involves cognition, mood, and mental state. To separate them is a philosophical error. Anyone who knows anything about how humans work knows that motor and cognitive and emotion systems are intrinsically part of one another.
I think that research needs to think of more subtle ways of understanding the relationships between motion and emotion. My personal view is that we [in HD] are hung up on motor disorders. We’ve privileged that as being the big deal, and I don’t think it is. Emotional symptoms are quite often early and quite often devastating.
Motor disorder is a cognitive disorder, by the way: Isn’t a motor disorder coordination? And isn’t that cognitive? It’s semantic, really. But it stops us from developing more meaningful language.
What kind of research are you doing now?
I’m primarily a clinician with an interest in research. I’m interested in [neuro]imaging of social cognition, and the biology of social cognition. There’s a story that would suggest that social cognitive deficits might be mediated by oxytocin [a brain chemical involved in relationships].
You have a big clinic, with about 350 patients with HD, and you’ve been working with HD for more than 20 years. What keeps you going? The patients are great. They have a horrible illness, but they really like to do research. Also, the global research effort has been fantastic. We’ve got a good collaborative approach.
The biggest thing for me, is that when I started as a junior doctor, everybody with HD was in the back wards of the asylums and thought of as being not treatable. Now, they’re giving the first gene suppression to people with HD. What could be more exciting than that?
This story was originally published in the Autumn 2015 issue of Enroll!