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Abstract

The prion diseases are rare neurodegenerative conditions that cause complex and highly variable neuropsychiatric syndromes, often with remarkably rapid progression. Prominent behavioral and psychiatric symptoms have been recognized since these diseases were first described. While research on such symptoms in common dementias has led to major changes in the way these symptoms are managed, evidence to guide the care of patients with prion disease is scarce. The authors review the published research and draw on more than 10 years’ experience at the U.K. National Prion Clinic, including two large prospective clinical research studies in which more than 300 patients with prion disease have been followed up from diagnosis to death, with detailed observational data gathered on symptomatology and symptomatic treatments. The authors group behavioral and psychiatric symptoms into psychotic features, agitated features, and mood disorder and describe their natural history, showing that they spontaneously improve or resolve in many patients and are short-lived in many others because of rapid progression of global neurological disability. Diagnostic category, disease severity, age, gender, and genetic variation are or may be predictive factors. The authors review the observational data on pharmacological treatment of these symptoms in the U.K. clinical studies and make cautious recommendations for clinical practice. While nonpharmacological measures should be the first-line interventions for these symptoms, the authors conclude that there is a role for judicious use of pharmacological agents in some patients: antipsychotics for severe psychosis or agitation; benzodiazepines, particularly in the late stages of disease; and antidepressants for mood disorder.

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Published In

Go to American Journal of Psychiatry
Go to American Journal of Psychiatry
American Journal of Psychiatry
Pages: 265 - 274
PubMed: 24585329

History

Received: 23 November 2012
Revision received: 19 May 2013
Accepted: 28 May 2013
Published online: 1 March 2014
Published in print: March 2014

Authors

Details

Andrew Thompson, B.Sc., M.R.C.P.
From the National Health Service (NHS) National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London; the NHS Highland Mental Health Services, Argyll and Bute Hospital, Blarbuie Road, Lochgilphead, Scotland; and the Medical Research Council (MRC) Prion Unit, Department of Neurodegenerative Disease, University College London Institute of Neurology, London.
Angus MacKay, Ph.D., F.R.C.Psych.
From the National Health Service (NHS) National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London; the NHS Highland Mental Health Services, Argyll and Bute Hospital, Blarbuie Road, Lochgilphead, Scotland; and the Medical Research Council (MRC) Prion Unit, Department of Neurodegenerative Disease, University College London Institute of Neurology, London.
Peter Rudge, F.R.C.P.
From the National Health Service (NHS) National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London; the NHS Highland Mental Health Services, Argyll and Bute Hospital, Blarbuie Road, Lochgilphead, Scotland; and the Medical Research Council (MRC) Prion Unit, Department of Neurodegenerative Disease, University College London Institute of Neurology, London.
Ana Lukic, B.Sc., M.R.C.P.
From the National Health Service (NHS) National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London; the NHS Highland Mental Health Services, Argyll and Bute Hospital, Blarbuie Road, Lochgilphead, Scotland; and the Medical Research Council (MRC) Prion Unit, Department of Neurodegenerative Disease, University College London Institute of Neurology, London.
Marie-Claire Porter, B.Sc., M.R.C.P.
From the National Health Service (NHS) National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London; the NHS Highland Mental Health Services, Argyll and Bute Hospital, Blarbuie Road, Lochgilphead, Scotland; and the Medical Research Council (MRC) Prion Unit, Department of Neurodegenerative Disease, University College London Institute of Neurology, London.
Jessica Lowe, B.Sc.
From the National Health Service (NHS) National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London; the NHS Highland Mental Health Services, Argyll and Bute Hospital, Blarbuie Road, Lochgilphead, Scotland; and the Medical Research Council (MRC) Prion Unit, Department of Neurodegenerative Disease, University College London Institute of Neurology, London.
John Collinge, F.R.C.P., F.R.S.
From the National Health Service (NHS) National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London; the NHS Highland Mental Health Services, Argyll and Bute Hospital, Blarbuie Road, Lochgilphead, Scotland; and the Medical Research Council (MRC) Prion Unit, Department of Neurodegenerative Disease, University College London Institute of Neurology, London.
Simon Mead, Ph.D., F.R.C.P.
From the National Health Service (NHS) National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London; the NHS Highland Mental Health Services, Argyll and Bute Hospital, Blarbuie Road, Lochgilphead, Scotland; and the Medical Research Council (MRC) Prion Unit, Department of Neurodegenerative Disease, University College London Institute of Neurology, London.

Notes

Address correspondence to Dr. Mead ([email protected]).

Competing Interests

Prof. Collinge is a director and shareholder of D-Gen Limited, an academic spinout company working in the field of prion disease diagnosis, decontamination, and therapeutics. The other authors report no financial relationships with commercial interests.

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