Clinical overview and therapeutic management of the cognitive and behavorial aspects of Huntington’s disease

Background: Huntington’s disease (HD) is an autosomal dominant neurodegenerative disease classified among chorea that, in addition to motor symptoms, is characterized by neuropsychiatric disorders. Objectives: to analyze the cognitive and behavioral clinical aspects of Huntington’s disease and the therapeutic management of these symptoms. Methods: a literature review was carried out from the Scielo and PubMed database, using “Huntington’s disease”, “Cognition”, “Behavior” and “Treatment” as descriptors, where 11 articles were selected between 2001 and 2018. Results: the nature of cognitive and behavioral symptoms in HD are very dynamic, and cognitive dysfunctions are present prior to diagnosis. The most common clinical challenges include executive dysfunction, mainly slow thinking and attention disorders, apathy, depression and irritability. One study used data from the European Huntington’s Disease Network and evaluated almost 2.000 carriers of the mutation, in which 47.4% reported apathy, while depression and irritability occurred in 42.1%, and aggression and psychosis occurred in 38.6%. Other studies have confirmed apathy as an early manifestation related to its progression. For the therapeutic management of chorea, tetrabenazine is used, while antidepressants can be effective in mood symptoms. On the other hand, antipsychotics can lead to the advance and rapid progress of the disease. Small controlled studies with atomoxetine, donepezil and rivastigmine have found no positive effects on patients’ cognition. Recent publications have shown that circulating levels of brain-derived neutrotrophic factors in HD correlate with mood, cognition and motor function and can serve as markers of treatment success, while growth factor I is associated with cognitive decline and can provide biomarker targets for treatment validation. Conclusion: cognitive and behavioral symptoms in HD are very diversified and some strategies may have potential therapies and/or deleterious ones.