Background:
Psychosis is considered rare in Huntington’s Disease, with an estimated prevalence of 3–11%. However, it has a profound impact on quality of life and disease burden. This study uses the Enroll-HD database to determine the prevalence, onset, and severity of psychosis in Huntington’s Disease and to determine demographic and disease characteristics associated with psychosis.
Methods:
Data were obtained from Enroll-HD. Adults with manifest Huntington’s Disease were included. Descriptive statistics were calculated. Simple logistic regression was used to calculate the odds ratio with 95% confidence interval for association with each characteristic.
Results:
7,966 manifest Huntington’s Disease participants were analyzed, and 12.95% had a history of psychosis. Mean age of psychosis onset (48.34 years, SD 13.26) mirrored Huntington’s Disease onset. Family history of psychosis in a first degree relative was documented in 23.6% of participants with psychosis. Variables significantly (p < 0.05) associated with presence of psychosis in manifest HD included lower education level, unemployment, single marital status, depression, decreased verbal fluency score, and decreased total functional capacity & functional assessment score.
Discussion:
Psychosis in Huntington’s Disease is more prevalent than many prior studies have reported. It is associated with several demographic & psychiatric features, decreased cognitive capacity, and worse functional outcomes.
Highlights:
Psychosis in HD is more prevalent than prior studies have reported. It is associated with a range of demographic and psychiatric variables, worse cognition, and worse functional outcomes suggesting several features that may be used to predict onset of psychosis and improve understanding and management of psychosis in HD.
A 65-year-old woman with hypertension, hyperlipidemia, ascending aortic aneurysm repair and valve replacement, and periprocedural atrial fibrillation (not on anticoagulation) presented with sudden-onset imbalance. The patient was on a treadmill when she experienced acute gait instability without vertigo. She held onto the bars to stay midline; otherwise, she leaned rightward. Examination revealed past-pointing and ataxic gait without dysarthria or nystagmus. Brain MRI demonstrated small superior vermis infarct. MRA revealed no significant stenosis. CTA of the chest showed an increase in known aortic pseudoaneurysm (Figure). Such pseudoaneurysms are not known to form thrombi or become embolic sources; she continued aspirin without anticoagulation. Gait instability and vertigo are common presenting signs of cerebellar infarction1; isolated lateropulsion may be seen in lesions involving the vermis.2
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