Psychiatric Illness in Huntington’s Disease




Neuroscience and Neurology CategoryHuntington’s disease can be a devastating illness for patients and their families. The disease is directly inherited — if you have a parent with Huntington’s disease, you have a 50% chance of inheriting the abnormal huntingtin gene yourself. Unfortunately, if you inherit the huntingtin gene, you will certainly develop the disease. While there is no cure for Huntington’s disease, researchers in both clinical and basic neuroscience fields are working to unlock the mysteries of this disease and to find a cure.

The most striking feature of the Huntington’s disease is the bizarre and uncontrollable movement of various muscle groups. In fact, until recently the disease was more commonly referred to as Huntington’s chorea, since the choreatic, dance-like movements are a hallmark of the disease. While chorea as it is used in the word choreaography is usually rhythmic and graceful, chorea as a symptom is quite random and irregular. It is this disorder of movement that researchers in both the laboratory and the clinic have focused much of their efforts.

DepressionWhile chorea should be controlled if at all possible, there are other features of the disease that must also be recognized. George Huntington’s first description of the disease in 1872 included a comment about psychiatric illness as a cardinal feature of the disease [1]. He writes that the disease is invariably recognized by,

1. Its hereditary nature,
2. A tendency to insanity and suicide,
3. Its manifesting itself as a grave disease only in adult life.

In modern medicine we have mostly done away with the term “insanity,” yet the presence of psychiatric illness and suicide still exists in patients of Huntington’s disease today. Patients with Huntington’s disease suffer from psychological issues such as anxiety disorders, memory disturbances and depression. Estimates of the prevalence of neuropsychiatric symptoms in Huntington’s disease are between 33 to 76 percent of patients [2] although the true incidence and prevalence may be much higher. While we do not understand the precise neurobiological cause, it is clear that psychiatric symptoms occur much more frequently in patients of Huntington’s disease than in the general population. Further, neuropsychiatric symptoms are often the most distressing aspect of the disease for patients and their families and many times they necessitate hospitalization [3]. The most frequent psychiatric symptoms that occur with Huntington’s are depression, anxiety, irritability, obsession and compulsions and psychosis. Also, patients may have difficulty forming new memories and performing complex tasks.

Despite the fact that psychiatric illness was described in the earliest report on the disease, research as to how best to treat these conditions has been lacking. More than 218 papers have been published on the pharmacological treatment of Huntington’s disease and every one of them used chorea as the endpoint [4]. When it comes to treating psychiatric illness in Huntington’s disease, physicians do not have any clear guidelines or protocol. While limited anecdotal evidence exists for certain atypical antipsychotics and newer antidepressants, nothing even resembling a consensus exists for pharmacological treatment of these comorbid conditions.

Until there is a cure for Huntington’s disease, managing symptoms remains the primary focus of care. Psychiatric illnesses occur quite often in the disease — they should not go undiagnosed or untreated. While likely to contain few participants, properly controlled and blinded trials should be conducted that focus on psychiatric endpoints in Huntington’s disease such as anxiety, psychosis and depression. We have a growing armamentarium of treatments for psychiatric illness — exceedingly larger than what was known in George Huntington’s time. Physicians need to know which medicines are safe and which are most effective in patients that are already enduring so much. That knowledge will only come through the careful study of psychiatric illness as it occurs in Huntington’s disease.

References

1. Huntington G: On chorea. Med Surg Rep 1872; 26:317–32.

2. E. van Duijn, E.M. Kingma, R.C. van der Mast (2007). Psychopathology in Verified Huntington’s Disease Gene Carriers Journal of Neuropsychiatry, 19 (4), 441-448 DOI: 10.1176/appi.neuropsych.19.4.441

3. J M Hamilton (2003). Behavioural abnormalities contribute to functional decline in Huntington’s disease Journal of Neurology, Neurosurgery & Psychiatry, 74 (1), 120-122 DOI: 10.1136/jnnp.74.1.120

4. Raphael M. Bonelli, Gregor K. Wenning (2006). Pharmacological Management of Huntington’s Disease: An Evidence- Based Review Current Pharmaceutical Design, 12 (21), 2701-2720 DOI: 10.2174/138161206777698693

  • Tammy

    Hi,

    I am a caregiver for my best friend Nola. Nola has not received any neurological attention for nearly 8 years. She seems to thrive with much social stimuli. If there are time-lines you want for the progression of Nola’s disease, I would be happy to help. I have known Nola since 1987. Her first sign of HD was in February 1989. Nola is nearly in her 20th year of HD, and I have been around for nearly all of it. I have also been around her daughter since she was 3 1/2, and feel certain she has the gene. I’ve known her son since he was born 15 years ago. I’ve so far not sensed HD in him, the way I sense it in Nola’s daughter.

    Sincerely,
    T. Fortner

  • maryjane

    Hi my name is Maryjane Roberts and I have a husband who has HD. We have been together for 13 yrs and he is driving me insane and others around me. I am looking for support to help us both through this.

    • JOAN EVANS

      MARYJANE, HD runs in my family. I have lost my dad, sister and recently, my brother. It’s just me left and I was lucky enough to be tested a few years ago and do not carry the gene myself. My entire life i have been caring for someone with HD. It is only now that my brother is gone that i am realizing the psychological toll it has taken on me. My dad got sick when i was a little girl. When he died we thought that was it. But 2 years later my little sister started showing signs. When she passed i thought my big brother and i would be the lucky ones. But no such luck. 2 years after our sister passed he started showing signs. He lived nearly 20 years with the disease. I would imagine that your husband has had a very similar life. My entire life, until i got tested, that was always there in the back of my mind. I tried to ignore it but realize now that my life has gone by and i haven’t enjoyed it. My time was spent feeling guilty about not having HD and cared for each of them the way i would have wanted to be cared for. It is a terrible disease. Your husband can’t help what he does. No matter what drug they give them, i can’t even imagine how i would feel if i had been diagnosed. I know i would have been very mad, and i’m sure that is what they feel. Their entire life is taken away and the horrible part is that they no doubt watched many a member of the family go through this and they know where it will lead. It is very sad, i am so sorry for you and your husband. The only advise i can give you is Be strong for him. Find something that makes you feel good and when things get tough, drown yourself in it. Find something that consumes your mind to take you away from that place you don’t want to be. Keep in mind that no matter how bad things get, you can get up and walk in the fresh air and feel the sun on your face. He has been condemned to a long, drawn out life of suffering knowing that it is just leading to death and it’s only going to get worse. Most of all, have faith that you are where you are for a reason. If he is lucky, you will stand by him. If he isn’t, besides having this horrible disease, he will go through it alone. His friends will leave because they can’t stand to see him decline. The famous saying is “I WANT TO REMEMBER HIM AS HE WAS”. This attitude is what really hurts you because you have to watch as he goes down hill or you can walk away, just like they did. This has been my experience. My brother was lucky enough to have three good childhood friends that came to see him until the end. Only a couple times a year but it meant so much to my brother and I, especially me. I would love to hear back from you and see how you are doing. Be strong.

      • Danielle Cunningham

        Wow! You nailed it:) We Carers of our HD people speak the same language we could tell our story’s in unison. And our hearts are broken in the same way. How can we get the help we deserve and our HD people deserve. Why are we so neglected as a Disease as opposed to Motor Nuerone Parkinsons Alzeimers let alone Cancer & Diabetes. The world & it’s Governments do not even know that we exist, how could they when they don’t know Huntingtons Disease exists! We need to get up, stand up for our rights. The only support we have is in each other and we are mentally physically financially and emotionally exhausted…

      • Danielle Cunningham

        PS Joan Evans r u in Australia too? My husbands HD status is from here in Australia from his Welsh side of the family… Evans

  • Renee cunningham

    Thanks its so nice to relise your not alone needing support with this my nan died of hd and now my is in last stage of hd. Two of my aunts have died from hd and now im facing the testing stage .. so scared and there is no support for us . Your right we need to make it clear to our community what it is and we need help .

Michael Sapko, MD, PhD

Michael Sapko, MD, PhD, is a distinguished medical doctor. He holds a MD/PhD from the University of Maryland School of Medicine. He has been honored by the National Institute of Neurological Diseases and Stroke (NINDS) with the National Research Service Award. Moreover, he was bestowed the American Society for Neurochemistry Young Investigator Educational Enhancement Award in his early medical career.
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