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Systematic review
Prion diseases motor and neuropsychiatric symptom cluster pharmacotherapy: structured scoping review
  1. Roseanagh Hogg1,
  2. John Centola2,
  3. Eugene Ace McDermott3,
  4. Francesca Mastaglio4,
  5. Anna Grundy5,
  6. Terri Awe3,6,
  7. Matthew Carey7,8,
  8. Mary Miller9,
  9. Chloe Antoinette Chin10,
  10. Rachel Quibell11,
  11. Tomasz Bajorek12,
  12. Suvankar Pal3 and
  13. Victoria Bradley13
  1. 1 Oxford University Hospitals NHS Foundation Trust Palliative Care, Oxford, Oxfordshire, UK
  2. 2 Neurology, NHS Tayside, Dundee, UK
  3. 3 University of Edinburgh, National CJD Research and Surveillance Unit, Edinburgh, UK
  4. 4 Palliative Medicine, NHS Tayside, Dundee, UK
  5. 5 Palliative Medicine, North Tees and Hartlepool NHS Foundation Trust, Hartlepool, UK
  6. 6 Department of Clinical Neurosciences, NHS Lothian, Edinburgh, UK
  7. 7 Palliative Care, Sir Michael Sobell House Hospice, Oxford, Oxfordshire, UK
  8. 8 Oxford University Hospitals NHS Foundation Trust, Oxford, UK
  9. 9 Palliative Care, Oxford University Hospitals NHS Foundation Trust, Oxford, Oxfordshire, UK
  10. 10 Palliative Medicine, Cambridge University Hospitals NHS Foundation Trust, Cambridge, Cambridgeshire, UK
  11. 11 RVI Palliative Care, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK
  12. 12 Psychological Medicine, Oxford University Hospitals NHS Foundation Trust, Oxford, Oxfordshire, UK
  13. 13 Sobell House, Oxford University Hospitals NHS Foundation Trust, Oxford, Oxfordshire, UK
  1. Correspondence to Dr Roseanagh Hogg; rosie.hogg{at}ouh.nhs.uk

Abstract

Background Prion diseases are a group of rare, neurodegenerative conditions that are invariably fatal and cause a variety of symptoms, which can prove challenging to control. Through this paper, we aim to review the current evidence regarding pharmacological management of neuropsychiatric and motor symptoms of prion disease as well as draw on experts’ and relatives’ experience, to evaluate the current evidence and provide recommendations moving forwards.

Methods A scoping review of the literature for pharmacological management of symptoms was conducted using the systematic review tool, COVIDENCE, with searches conducted through four databases. 120 papers were selected for inclusion, and data extraction was carried out by two independent reviewers. Given the lack of high-quality data and small numbers, no further attempt at statistical analysis was made, and results are presented in a thematic synthesis.

Results Although a broad range of approaches and pharmacotherapies are trialled to manage these challenging symptoms, there are patterns emerging of some efficacy seen with the use of benzodiazepines, antipsychotic and anticonvulsant medications in both motor and neuropsychiatric symptoms in prion disease. These approaches and associated challenges were reflected in international expert opinion that was gathered via online survey.

Conclusion There continues to be a paucity of good-quality evidence and we suggest a need for longitudinal, population-based and standardised research to allow a robust evidence base, which in turn will guide excellent symptom control and end of life care for this group of complex patients.

  • Palliative Care
  • Neurological conditions
  • Pharmacology
  • Psychological care
  • Symptoms and symptom management
  • Supportive care

Data availability statement

Data are available upon request.

https://doi.org/10.1136/spcare-2024-005027

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    Data availability statement

    Data are available upon request.

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    Footnotes

    • X @acemcdermott, @dr_mary_miller, @drchlo

    • Contributors The original review question and search strategy was developed and conducted by JC. Abstract and full-text screening was conducted by JC, AG, FM, RH and VB. Data extraction and thematic synthesis conducted by VB and RH. Case vignettes were written by EAM and TA. Patient and relatives’ feedback was obtained and compiled by TA. Expert opinion was gathered by an online survey designed, distributed and analysed by JC. Scoping review drafted by VB and RH. Paper reviewed and edited by SP, MM, MC, TB, RQ and CAC in addition to the other authors listed above. All authors are part of a group currently working towards developing an evidence base and clinical guideline for symptom management in prion diseases. VB and RH act as guarantors for the work.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; internally peer-reviewed.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.