Sleep disorders are common in HD. They can include trouble falling asleep, staying asleep, waking up early, and disrupting day-night cycles causing day-time sleepiness. Drug treatments for sleep disorders in HD are similar to those recommended for sleep disorders in other neurodegenerative diseases. If drugs are used, drug choice should be influenced by the presence of other symptoms of HD.

Psychosis in HD is defined by the presence of delusions and/or hallucinations that are not caused by other illness (like infection or trauma) that can have features of psychosis with confusion. Psychosis of HD is reported less commonly than other neuropsychiatric symptoms in HD, but some experts thought it may be underdiagnosed. Psychosis is a severe symptom that imposes significant burden on both the individual with HD and carers. Its treatment in HD is similar to treatment of psychosis in other diseases.

Apathy is a lack of motivation. This includes lack of initiative, lack of interests or concerns, and lack of emotional response. Apathy of HD can occur before motor onset, and it increases in severity with disease stage. Though it can be hard to tell the difference between apathy of HD from the apathy of depression, apathy of HD lacks the sadness, negative, and suicidal thoughts that goes along with depression.

Anxiety can occur in HD before motor symptoms and at all stages of disease after motor diagnosis. Anxiety as it presents in HD is similar to that in the general population. It can take the form of a general feeling of tenseness, or generalized anxiety that is more severe and can interfere with day to day function, or social anxiety and panic. The experts agree that anxiety is a treatable symptom complication of HD. In general, the treatment recommendations in HD are similar to those for the general population except that drug choice is influenced by other HD symptoms which may be present.

Agitation is an extreme irritability behavior. It is not unique to HD, and often occurs in other diseases causing cognitive impairment and dementia. It can also be the result of any severe medical illness, trauma, or be related to drug use or withdrawal. Agitation in HD shows up in many ways. In milder forms it looks like general mental or physical restlessness but can progress to more severe forms with verbal or physically agressive behaviors. It can come on quickly without warning, but more commonly is preceded by other neuropsychiatric symptoms of HD.

Because agitation can be thought of as extreme irritability, identifying and and treating milder forms of irritability is vital. When agitation occurs in HD, whether quickly or gradually, the treatment broadly follows recommendtions for agitation when it occurs with other medical conditions.

Expert-based recommendations for the management of agitation, anxiety, apathy, psychosis and sleep disorders in Huntington's disease (HD) are now available from the Journal of Huntington’s Disease. This information is important for doctors and HD families because medical care in HD is not just about offering a cure in the future, it's about reducing burdensome symptoms and improving quality of life now. Because there is too much information in the journal publication to put into a single HDDW article, this introduction will be followed by separate articles that deal with each of the symptoms. The goal is to help HD families learn about the recommendations and be able to use them to partner with local doctors.

Important take home points for medical providers and for families affected by HD is that treatment for each of these symptoms in HD is quite similar to treatment of that same symptom in other conditions. However when more than one symptom is present the choice of drug will be influenced by the combination of symptoms.

After clinical trial results were announced for deutetrabenazine (Austedo), I hoped we might be getting a drug that didn't cause as many side effects of other chorea treatments (antipsychotics or the old tetrabenazine). And now that I have prescribed it, it has proved better than I had expected based on the clinical trial results. Though not successful in everyone, it has clearly decreased chorea in most of my patients who were candidates for this drug. But what I was not expecting is how much this drug improves some of the functional activities that chorea impacts.

For sure Austedo is not the cure, nor does it treat the cognitive or behavioral complications of HD , nor does it work for everybody, nor does it take all the chorea away, it is the best drug we have -- and for the first time in the history of our disease we can make people better by improving motor function. As Ralf Reilman has put it, "It's not a revolution, but a welcome evolution for treating chorea".

Bowel and bladder problems cause much distress for individuals with Huntington's disease (HD) and their families.  How common are these complaints? How "real" are these complaints? Are these just obsessive behaviors?  Are they imagined and just "in the head"? Or what?

Although there surely are others, four non-drug "things" that this author believes can make a huge difference for those with HD or at risk of it is the focus of this article.  The "things" include:

  • Exercise for body and brain
  • Knowledge of and early recognition and management of symptoms
  • Healthy Sleep
  • Attention to carer needs

Although a study testing bupropion (Wellbutrin) for treating apathy has recently been completed, there has been no high level study of any drug for treatment of any behavioral or neuropsychiatric symptom in Huntington's disease (HD).  Nor is there study on adverse side effects of the drugs commonly used in HD.  However experts believe, and families know that available drugs can be helpful. Unfortunately there is little available guidance for doctors or families on when and how to use these drugs, or what side effects to look for.

Lack of guidance leads to opposing problems: Some with HD have too little drug therapy, while others likely have too much . . .