Palliative care symptoms of people living with rapidly progressive prion diseases: a systematic review.

backgroundCreutzfeldt-Jakob disease (CJD) is a rapidly progressive, fatal dementia with an average prognosis of four to six months. Palliative care, as a holistic person-centred approach to supporting people and families affected by life-limiting illness, may be beneficial. However, despite the rapid disease trajectory, relatively little is known about the palliative care symptoms and concerns of this population. This systematic review identifies and reports the palliative care symptoms of people living with sporadic, iatrogenic, and acquired CJD.

methodStudies were included if they reported the symptoms of patients with a diagnosis of CJD (excluding inherited prion disease), using quantitative methodology. No date range was used to narrow the search, non-English language and non-peer reviewed papers were excluded. Searches for relevant studies published before December 2023 were conducted across five databases (including EMBASE, CINAHL, and PubMed) by eight independent reviewers. Methodological quality appraisal was undertaken using the Hawker Quality appraisal tool. Data on study characteristics and reported symptoms, were extracted. Identified symptoms associated with CJD were collated, and Artificial Intelligence assisted semantic clustering was performed to group symptoms into categories, the reported symptoms were tallied to identify those most commonly reported across the literature. A palliative care lens was then applied to classify symptoms as physical, psychological, social, or spiritual. The review protocol was registered with PROSPERO (CRD42020194294).

resultsThe search yielded 8,503 unique citations; following title, abstract, and full-text screening, 30 papers were included in the final analysis, describing 4,434 participants. Across the included literature, 109 unique symptoms were identified, 374 in total when accounting for duplicates. Symptoms were categorised into 11 subsets: motor impairments (17.6%, 66/374 proportion of total number of symptoms); coordination, balance and vestibular issues (17.6%, 66/374); behavioural, psychiatric and emotional symptoms (23.5%, 88/374); speech and language disorders (10.1%, 38/374); visual and sensory disturbances (7.7%, 29/374); cognitive disorders (6.9%, 26/374); sleep disorders (6.6%, 25/374); other neurological symptoms (4.2%, 16/374); swallowing and nutrition issues (2.9%, 11/374); autonomic and sphincter dysfunction (1.3%, 5/374); and social symptoms (1.0%, 4/374).

conclusionFindings align with established knowledge regarding the diverse and complex symptom burden in CJD, highlighting the potential challenges of symptom management. Motor and coordination impairments were most frequently reported, alongside behavioural and psychiatric disturbance. Limited evidence was identified regarding social or spiritual symptoms, quality of life, or symptom progression, as many studies focused primarily on improving early diagnosis. Further primary research is required to better understand the palliative care needs of people living with CJD to ensure comprehensive end-of-life care.