
Huntington’s disease (HD) is an incurable, inherited, progressive, neurodegenerative disorder that is characterized by a triad of the motor, cognitive, and psychiatric problems. Despite the noticeable increase in therapeutic trials in HD in the last 20 years, there have, to date, been very few significant advances. Prevalence in the Western populations is estimated at 10.6 to 13.7 individuals per 1 lakh. The mean age at onset of symptoms is 30 to 50 years. In some cases, symptoms start before the age of 20 years with behavior disturbances and learning difficulties at school. Diagnosis is based on clinical symptoms and signs in an individual with a parent with proven HD and is confirmed by DNA determination. Pre-manifest diagnosis should only be performed by multidisciplinary teams in healthy at-risk adult individuals who want to know whether they carry the mutation or not. Until now an actual remedy for the HD is not available. Management should be multidisciplinary and is based on treating symptoms to improve quality of life. In this review, we discuss the status of and supporting evidence for, potential novel treatments of HD.