Abstract
It is generally believed that treatments are available to manage obsessive-compulsive behaviors (OCB's) in Huntington’s disease (HD). However, lack of an evidence base prevents guideline development. The research literature fails to address the indications for behavioral interventions, drug selection, drug dosing, management of inadequate response to a single drug, and preferred drugs when additional behavioral symptoms comorbid to OCBs are present. In an effort to inform clinical decision-making, we surveyed an international group of experts to address these points. Survey results showed that experts utilized behavioral therapy only for patients with mild cognitive impairment. There was expert agreement that a selective serotonin reuptake inhibitor (SSRI) was the first choice drug, although clomipramine (CMI) was cited as a monotherapy choice by the smaller number of experts familiar with its use. Perceived efficacy for control of OCBs was similar for both SSRIs and CMI. Though less favored choices overall, antipsychotics (APDs) and antiepileptic mood stabilizers (AEDs) were most often used as augmentation strategies. In addition to survey results, this report reviews available studies, and lastly presents an algorithm for the treatment of OCBs in HD based on practice-based preferences obtained from this survey.
Highlights
Huntington’s disease (HD) is an inherited neurodegenerative illness characterized by a combination of motor, cognitive and behavioral abnormalities
The research literature fails to address the indications for behavioral interventions, drug selection, drug dosing, management of inadequate response to a single drug, and preferred drugs when additional behavioral symptoms comorbid to obsessive-compulsive behaviors (OCBs) are present
Recognizing the limits of expert opinion, and with the expectation that future clinical research will provide evidence-based information, we present survey results to provide direction for the management of OCBs in HD
Summary
Huntington’s disease (HD) is an inherited neurodegenerative illness characterized by a combination of motor, cognitive and behavioral abnormalities. A wide variety of behavioral disturbances have been described in HD patients, including perseveration and obsessive-compulsive symptoms. For the purposes of this discussion we use the single term, obsessive-compulsive behaviors (OCBs) to encompass this entire range of these symptoms. Research into OCBs in HD is limited; reports suggest that OCBs occur in 20 to 50% of HD patients [1][2][3][4][5][6][7]. OCBs have been reported in prediagnosed individuals who carry the HD gene expansion [8][9][10]. Case reports have described instances of OCBs [11][12] , and an instance of obsessive gambling in an HD pedigree [13]
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