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

<h3>Background</h3> 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 o&amp;fflig;ered on a case-by- case basis depending on the presentation and treatment needs of the individual. <h3>Aim</h3> 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. <h3>Method</h3> 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 pro&amp;filig;les that typify the common presenting symptoms and management approaches. <h3>Results</h3> Both a&amp;fflig;ective 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 di&amp;ffilig;cult 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 e&amp;fflig;ects 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 modi&amp;filig;cations, psychological support, and where necessary careful use of psychotropic medication. <h3>Conclusion</h3> 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 pro&amp;filig;les presented in this series demonstrate potential approaches to symptom management. Going forwards, standardised treatment protocols may prove useful.

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Published
2025-02-01
DOI
https://doi.org/10.1136/jnnp-2024-bnpa.58
Primary Topic
Prion Diseases and Protein Misfolding
Type
article
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article

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

Lucy Pauli, R. H. B. Williams, Jennifer A. Foley, Simon Mead
Prion Diseases and Protein Misfolding
article

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

Lucy Pauli, R. H. B. Williams, Jennifer A. Foley, Simon Mead
article en

Abstract

<h3>Background</h3> 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 o&amp;fflig;ered on a case-by- case basis depending on the presentation and treatment needs of the individual. <h3>Aim</h3> 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. <h3>Method</h3> 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 pro&amp;filig;les that typify the common presenting symptoms and management approaches. <h3>Results</h3> Both a&amp;fflig;ective 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 di&amp;ffilig;cult 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 e&amp;fflig;ects 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 modi&amp;filig;cations, psychological support, and where necessary careful use of psychotropic medication. <h3>Conclusion</h3> 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 pro&amp;filig;les presented in this series demonstrate potential approaches to symptom management. Going forwards, standardised treatment protocols may prove useful.

University College London Hospitals NHS Foundation Trust (GB), MRC Prion Unit (GB), National Hospital for Neurology and Neurosurgery (GB), Sheffield Health and Social Care NHS Foundation Trust (GB), University College London (GB)
Openalex Percentile: Top 98%
Prion Diseases and Protein Misfolding
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