Criminality in Huntington Disease

Abstract

Introduction:Huntington’s Disease (HD) is an inherited neurodegenerative condition associated with varying degrees of motor, cognitive, and behavioral abnormalities. Although aggression, irritability, lack of insight and poor impulse are well-recognized nonmotor manifestations of the disease, very little is known about their association with criminality, which is broadly defined as acts that are punishable by law.Methods:We explored the prevalence of criminality in patients with HD seen between 1/1/2017 – 12/31/2021 within a large academic medical center in the state of Illinois. To determine rates of criminality, we examined “criminal behavior” (defined as illegal actions that do not necessarily lead to criminal prosecution) through a retrospective medical record review of keywords or phrases related to criminality. We also examined “crime” (defined as the presence of a criminal record) in this same sample of patients through a search of official publicly available online databases from the state of Illinois and the federal government.Results:Of the 210 patients included, ninety-seven (46.2%) had criminal behavior documented in their medical records. Based on public database information, eighty-nine patients (42.4%) had a criminal record and twenty-six patients (12.4%) had been arrested. Traffic violations were committed by 94.4% of the cohort and were the most common infractions. The majority of these violations were petty offenses and only punishable by fines. Physical aggression towards caregivers was the most common criminal behavior identified in medical records. This tended to occur in advance stages of the disease and rarely led to criminal charges. Crime occurred at any point in the disease course of HD, including before and after the development of clinical signs and the formal diagnosis of HD.Conclusion:The presence of criminality was not infrequent in our cohort, but rarely did this result in criminal charges and rarely did these charges result in arrests or incarcerations. Discussions surrounding driving safety as well as management of physical aggression towards caregivers should be part of regular outpatient visits with patients with HD. Ultimately, it is unknown if criminality is a common feature of HD, and assessments need to be developed to determine its true prevalence.

The Implications of Moral Neuroscience for Brain Disease: Review and Update

“…Individuals with Huntington disease, which impairs frontal-subcortical tracts as they traverse the basal ganglia, appear to have increased moral behavioral violations. Among individuals with this disease, 11-46.2% exhibit criminal behavior such as traffic violations and violence against caregivers (McDonell et al, 2021;Olvera et al, 2022), and this behavior is associated with early disease stages, male gender, decreased caudate function, decreased empathy, decreased recognition of the emotions of others, poor insight into own behavior, and increased trinucleotide repeats (McDonell et al, 2021;Olvera et al, 2022). As dementias progress into the frontal brain structures, moderately to severely impaired individuals with any dementia can manifest disinhibited behavior, including sexual disinhibition, as well as psychotic beliefs that lead to criminal acts (Mendez, 1996(Mendez, , 2022c.…”

The Role of Innovation Technology in the Rehabilitation of Patients Affected by Huntington’s Disease: A Scoping Review

“…The latter, in fact, resulted to be strongly impacted by motor, cognitive, and psychiatric disorders related to HD. Involuntary choreic movements along with cognitive and behavioral disturbances constitute pathognomonic symptoms of pathology [7][8][9][10]. Specifically, the most common motor disorders involve involuntary movements that begin in the distal extremities, including the facial muscles, and then gradually progress to the more proximal and axial muscles, increasing in amplitude [7,8].…”

“…Additionally, dysarthria and dysphagia, dystonia, tics, and cerebellar signs, such as ataxia, may occur. As for behavioral and psychiatric symptoms, they often precede motor symptoms and resemble frontal lobe dysfunction, with poor attention, impulsiveness, and irritability [10,11]. Other possible symptoms include apathy, loss of intuition and creativity, psychosis, lack of awareness, and depression, often associated with suicide attempts [11,12].…”