New Guidelines on Neuropsychiatric Symptoms in Huntington's

February 08, 2019

New guidelines for the management of some of the more common neuropsychiatric symptoms of Huntington's disease have been published.

The guidelines cover the routine management of agitation, anxiety, apathy, psychosis, and sleep disorders in Huntington's disease, and were published in the Journal of Huntington's Disease.

The current publication adds to previously published guidelines by the same group on other behavioral symptoms that occur in Huntington's — irritability, obsessive compulsive disorder (OCD), and chorea.

"We developed guidelines for irritability, OCD, and chorea first as we felt these were the symptoms that that had the greatest need," lead author Karen E. Anderson, Georgetown University, Washington, D.C., told Medscape Medical News.

"The current publication covers a larger set of guidelines dealing with another five symptoms that commonly occur in Huntington's patients."

The authors note that changes in behavior and mental state are a central feature of Huntington's disease and often cause considerable distress and difficulty to patients, their relatives, and caregivers. Most people with Huntington's will experience multiple neuropsychiatric symptoms or disorders during the course of their illness, which may be related to the underlying progressive neurodegeneration of the brain but can also be a psychological reaction to having the disease. They may also arise as a side effect of some Huntington's medications; for example, some agents used to treat chorea may worsen apathy, depression, and other psychiatric symptoms.

Anderson pointed out that while Huntington's is classified as a neurologic condition, it has prominent psychiatric symptoms; there is, however, a real lack of psychiatric expertise in Huntington's disease and most patients do not have access to a psychiatrist, she said.

"They may see a neurologist but only about 30% of Huntington's patients in the US have access to a specialist with expertise in the condition," Anderson said. "The rest are just treated by their local family doctor who may never have seen a Huntington's patient before."

She said the guidelines are directed mainly at generalist clinicians who may only see one or two patients with Huntington's over their whole career. "So they may lack insight into the many psychiatric symptoms that can occur in these patients. These guidelines will give clinicians somewhere to start when caring for these patients."

"Physicians need to understand that while there is no treatment yet that can slow the progression of the disease itself, there is actually a lot that you can do for these patients. The neuropsychiatric symptoms can really impact the quality of life for these patients. If we can reduce them — which we can by following the recommendations made in these guidelines — we can ensure real improvements in the life they have left," Anderson stressed. 

The guidelines are also likely to be used by patients and their families to ensure they are getting the best care. "It is important for patients and families to understand that these symptoms are part of the disease and can be treated," she said.  

"These psychiatric symptoms are often untreated — it is not well understood that these symptoms are part of the disease. The guidelines will help in educating doctors and patients/families about these symptoms and that the patient is not just being difficult," she added.   

The guidelines recommend that nonpharmacologic interventions addressing environmental factors or behavioral interventions should be considered first when treating these neuropsychiatric symptoms. But they also suggest suitable drugs for each of the symptoms. 

"When pharmacologic agents are required, one should be aware of possible pharmacodynamic interactions. A key strength of the pharmacologic recommendations is that they purposefully take co-existing symptoms into account," the authors write.

The guidelines were developed based on a modified Institute of Medicine guideline process that accounted for a lack of evidence base. An international committee of 11 multidisciplinary experts proposed a series of statements regarding the description and management of each symptom. Statement assessment and validation was performed using a web-based survey tool with 84 international Huntington's experts (neurologists and psychiatrists) indicating their level of agreement.

High-level agreement (≥85% experts strongly agreed or agreed) was reached for 107 of the 110 statements that have been incorporated into the guidelines.

Anderson noted that they did not include depression in the list of symptoms covered by the guidelines. "Although depression is very common in Huntington's patients, its treatment is very similar to that in other patients so we felt there was less need for guidelines on this," she said.  

Funding for this work was obtained from the Griffin Foundation and Auspex Pharmaceuticals. Neither funding organization was involved in drug development for any of the symptoms included in this project. Anderson reports consultancy for Teva, Roche, CHDI Foundation, Prana, and Lundbeck; participation in studies funded by Teva and Vaccinex, and research funding from Cures Within Reach.

Journal of Huntington's Disease. 2019;7:355-366.  Published November 24, 2018. Full text.  

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