Palliative and hospice care: more detail

How were the recommendations made?

Through a hybrid on-site and remote workshop of UK specialists, not a formal Delphi process. The panel included prion disease physicians, nurses and researchers, palliative care doctors, psychiatrists, a general practitioner, a clinical neuropsychologist and a lived experience representative. International specialists were asked to comment on practice in Japan, Germany and the United States. A PubMed search run on 26 January 2026 covered symptom management in human prion disease, excluding single case reports and diagnostic, genetic and disease-modifying studies.

The paper does not report a number of statements, voting rounds or a consensus threshold. The authors describe the output as expert consensus informed by their collective experience, and say they plan to reconvene in five years.

What do they recommend about timing and planning?

Early involvement of palliative care is encouraged to help guide conversations about preferences, and preferences should be sought ideally at the time of diagnosis. The reason given is that capacity may already be lost during the time taken to reach a diagnosis.

Future care planning is distinguished from advance care planning, which requires capacity. It can include appointing proxies, advance decisions and escalation plans. Where no family is available in the UK, the authors recommend involving an Independent Mental Capacity Advocate. Clinicians may complete an SR1 form and add the patient to the Gold Standards Framework register.

Who should be on the team?

The GP, occupational therapy, physiotherapy, speech and language therapy, district nurses, geriatrics and old age psychiatry, palliative care, social work, and the National Prion Clinic and National CJD NHS Nursing Service. The National Prion Clinic staff page lists a consultant in neuropalliative care from 2026 alongside the Director, John Collinge, and the Clinical Lead, Simon Mead.

What does the paper say about symptoms?

Myoclonus is treated only when it interferes with function or care, or causes distress. Levetiracetam is first line at a starting dose of 250 to 500 mg twice daily, and the authors recommend reserving clonazepam for more advanced stages when people are no longer walking. At the end of life, levetiracetam or midazolam can be given by subcutaneous infusion.

Agitation and aggression are approached by treating unmet needs first, and a period of watch and wait is often justified because symptoms can resolve quickly. Risperidone is listed first line with olanzapine as an alternative. Parenterally, lorazepam or midazolam are preferred to haloperidol because of extrapyramidal risk.

Pain is assessed with collateral history and observational tools, none of which are validated in prion disease. Regular paracetamol is suggested first line. The authors note that opioids have adverse effects on cognition and falls risk and should be used with caution. Neuropathic agents are used for dysaesthesia.

For swallowing and feeding, the benefit of oral intake is weighed against aspiration risk with speech and language therapy input, decisions are reassessed at every review, and clinically assisted nutrition and hydration should not routinely be used in people living with severe dementia unless a reversible comorbidity is present.

Sleep is managed first with non-drug measures, with melatonin, trazodone, low-dose mirtazapine, zopiclone or a benzodiazepine considered when needed, weighing falls risk.

Where do people die?

National Prion Monitoring Cohort data cited in the paper give 28 percent of deaths at home, 28 percent in a hospice, 23 percent in a residential or nursing home and 21 percent in hospital. For comparison the paper gives 12.1 percent of dementia deaths in hospital and 3.2 percent in hospice, and reads the difference as a marker of complex care needs.

What about the last days and afterwards?

Future care plans should be reviewed and respected, and medicines and interventions stopped unless essential, with a documented plan including escalation. Symptom assessment is difficult when someone cannot communicate and the illness is moving fast; proxy tools such as IPOS-Dem may help, though their evidence comes from other conditions. Nursing attention goes to skin and corneal integrity, mouth care, continence and upper airway secretions. Anticipatory medicines are usually prescribed subcutaneously or intravenously, since swallowing is often unsafe.

After death, the paper notes that MRI and CSF RT-QuIC have improved diagnostic confidence and reduced the need for post-mortem examination. A consented examination may still be sought to confirm or exclude the diagnosis, classify the disease, obtain tissue for PRNP testing where blood was not taken, honour family wishes, or donate tissue to research. Most consented examinations are limited to the brain; sampling from a fresh brain allows the remainder to return to the body without delaying the funeral, while prolonged fixation improves diagnostic yield. Samples are handled in a containment level 3 laboratory, with derogations, until fixed and formic acid treated, and investigations can take up to three months. Families who wish to see or touch the body should not be discouraged, ideally before the examination.

Infection control guidance in England is published by the Department of Health and Social Care for the ACDP TSE risk management subgroup and covers decontamination, instruments, endoscopy, pathology, care after death and an information sheet for funeral directors and relatives.

How does practice differ between countries?

In Japan there is national prion guidance with an update scheduled for 2026; family preferences and social norms can make declining nutrition difficult when swallowing becomes unsafe, tube feeding is common including in the terminal phase, and most deaths occur in hospital. In Germany, care broadly follows the same principles, but home nursing through statutory insurance can be slow administratively, coverage of specialist palliative services varies, reimbursement for genetic testing can delay diagnosis, gastrostomy is now rare, and most people die in palliative institutions. In the United States, fragmented care and insurance-dependent eligibility can delay diagnosis and palliative referral; the paper gives an average of about 7.9 months from symptom onset to death, with many deaths within two months of diagnosis, and about two thirds of patients accessing palliative care, often late. The authors read these differences as reflecting health systems and cultural norms rather than patient need.

What the paper does not establish

The evidence base is observational cohorts, case reports, case series and expert opinion. There are no randomised trials of symptom management in prion disease. Medication guidance is extrapolated from other dementias, and several agents rest on small numbers or case reports. Pain and tremor evidence is described as very sparse. The resource table is UK-focused, with the CJD International Support Alliance as the international pointer.

Support for families and relatives

National CJD specialists give advice before and after a death, with local support from GPs, palliative care teams, hospital bereavement services and third sector organisations such as the CJD Support Network. For inherited prion disease, the National Prion Clinic can provide follow-on care for at-risk family members. In Spain, the Fundacion Espanola de Enfermedades Prionicas offers free individual psychological support and a grief group alongside legal guidance pages.

The plain version of this page is palliative and hospice care, and the formal choices are covered at end of life decisions.