Work

#7183 The assessment and management of psychiatric symptoms in prion disease: an illustrative case series

Year: 2025

Type: conference-paper

Abstract: Background Prion diseases are rare, progressive, neurodegenerative conditions. Uniquely, prion diseases occur in sporadic, inherited and acquired forms. All forms of the disease are associated with complex and wide-ranging neuropsychiatric symptoms. Despite the high prevalence of psychiatric symptoms, there are no current recognised treatment protocols to guide management. Psychological and pharmacological treatments are oﬀered on a case-by- case basis depending on the presentation and treatment needs of the individual. Aim In this case series we aim to demonstrate the varied onset, nature and course of psychiatric symptoms in prion disease, as well as the diverse treatment approaches required. Method The National Prion Clinic has many years of experience in the assessment and management of neuropsychiatric symptoms in prion disease. Over 1000 patients have been followed up from diagnosis until death by prospective cohort study, providing data regarding psychiatric symptomatology and clinical management. We present a series of proﬁles that typify the common presenting symptoms and management approaches. Results Both aﬀective and psychotic symptoms are commonly observed in prion disease. However, due to the rapidly progressive nature of the condition, these symptoms may morph, resolve, or become diﬃcult to detect, in a relatively short period of time. While the general approach to these symptoms is similar to other settings, the rapidly evolving clinical picture and heightened risk of side eﬀects need to be carefully considered. As the disease progresses, it is common to observe behavioural and psychological symptoms, including so-called ‘behaviour that challenges’. In these circumstances it is important to consider environmental modiﬁcations, psychological support, and where necessary careful use of psychotropic medication. Conclusion The neuropsychiatric manifestations of prion disease are vast and can prove challenging to manage. There is both marked variation in symptoms across individuals at illness onset, and within an individual as the disease progresses. The proﬁles presented in this series demonstrate potential approaches to symptom management. Going forwards, standardised treatment protocols may prove useful.

Source: British Neuropsychiatry Association Annual Conference (BNPA)

DOI: 10.1136/jnnp-2024-bnpa.58

Authors: Lucy Pauli, Rachel Williams, Jennifer Foley, Simon Mead