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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., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ Group.)

Background: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly progressive and fatal prion disease with significant public health implications. Survival is heterogenous, posing challenges for prognostication and care planning. We developed a survival model using diagnostic data from comprehensive UK sCJD surveillance., Methods: Using national CJD surveillance data from the United Kingdom (UK), we included 655 cases of probable or definite sCJD according to 2017 international consensus diagnostic criteria between 01/2017 and 01/2022. Data included symptoms at diagnosis, CSF RT-QuIC and 14-3-3, MRI and EEG findings, as well as sex, age, PRNP codon 129 polymorphism, CSF total protein and S100b. An artificial neural network based multitask logistic regression was used for survival analysis. Model-agnostic interpretation methods was used to assess the contribution of individual features on model outcome., Results: Our algorithm had a c-index of 0.732, IBS of 0.079, and AUC at 5 and 10 months of 0.866 and 0.872, respectively. This modestly improved on Cox proportional hazard model (c-index 0.730, IBS 0.083, AUC 0.852 and 0863) but was not statistically significant. Both models identified codon 129 polymorphism and CSF 14-3-3 to be significant predictive features., Conclusions: sCJD survival can be predicted using routinely collected clinical data at diagnosis. Our analysis pipeline has similar levels of performance to classical methods and provide clinically meaningful interpretation which help deepen clinical understanding of the condition. Further development and clinical validation will facilitate improvements in prognostication, care planning, and stratification to clinical trials., Competing Interests: Declarations. Conflicts of interest: No competing interests to declare., (© 2024. The Author(s).)

Introduction: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly progressive neurodegenerative disease with public health implications. Mean age of onset is 68 years. Age-specific incidence declines after 80 years. This may arise from under-ascertainment or other biological features of the disease. Accurate characterisation of late-onset sCJD is important for early diagnosis, avoiding unnecessary investigations and improving ascertainment for public health purposes., Objective: To phenotype the clinical features and investigation profile of sCJD in adults >80 years., Methods: We analysed all probable and definite sCJD cases identified by the UK National CJD Research & Surveillance Unit over a 10-year period (2011-2021). Individuals were grouped by age of onset. Clinical features and investigation profiles were compared., Results: 10.3% (123/1196) had an age of onset over 80. Median survival was shorter (3.2 vs 4.3 months; P < 0.001). Pyramidal signs (48.3% vs 34.2%; P = 0.008) and akinetic mutism (55.1% vs 33.2%; P < 0.001) were more frequent. Psychiatric symptoms (26.3% vs 39.6%; P = 0.01) and cerebellar signs (65.4% vs 78.6%, P = 0.007) were less frequent. Cognitive impairment and myoclonus were highly prevalent regardless of age. Between age groups, the diagnostic sensitivity of cerebrospinal fluid real-time quaking-induced conversion (CSF RT-QuIC) (92.9% vs 91.9%, P = 0.74) was comparable, electroencephalography was superior (41.5% vs 25.4%; P = 0.006) and MRI was inferior (67.8% vs 91.4%; P < 0.001)., Conclusions: Late-onset sCJD has distinct clinical features, shorter survival and a different profile of investigation sensitivity. CSF RT-QuIC, MRI brain and specialist CJD review is recommended in older adults with a rapidly progressive neurological disorder. Autopsy is valuable when the cause remains elusive., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Geriatrics Society.)

Introduction: Sporadic Creutzfeldt-Jakob Disease (sCJD) is the commonest human prion disease, with a median age of onset of 68 years. We characterise the clinical, investigation, and neuropathological features in young individuals with sCJD using data from UK national CJD surveillance., Methods: Referrals between 2011 and 2021 were examined, with definite (post-mortem confirmed) or probable sCJD cases included. Clinical features, MRI, EEG, CSF RT-QuIC, 14-3-3, PRNP sequencing and neuropathological findings were examined. We compared younger (≤ 50 years age of onset) with older individuals. Records of Non-sCJD referrals were also reviewed., Results: 46 (4%) young individuals were identified (age at onset 25-50) from 1178 cases. 15 (33%) were autopsy confirmed. Psychiatric disturbance (37% vs 22%, p = 0.02) and headache (11% vs 3%, p = 0.01) at presentation, and longer disease duration (by 1.45 months, 95% CI 0.43-2.79, logrank p = 0.007) were commoner. CSF RT-QuIC showed lower sensitivity (82% vs 93%, p = 0.02). There was no difference in sensitivity of MR brain or CSF 14-3-3. There were no significant co-pathologies in autopsy-confirmed cases. For non-sCJD referrals, 41 cases were of other CJD subtypes, and 7 non-prion diagnoses., Conclusions: Young-onset sCJD is more likely to present with neuropsychiatric symptoms and headache, longer disease duration, and lower sensitivity of RT-QuIC. These findings may be driven by the underlying molecular subtypes. Our results guide the evaluation of younger individuals presenting with rapidly progressive cognitive, neuropsychiatric, and motor decline, and emphasise the need for additional vigilance for atypical features by clinicians and CJD surveillance programmes worldwide., (© 2022. The Author(s).)

Objective: To determine if a high compliance rate for wearing external hip protectors could be achieved and sustained in a long-term care population., Study Design: A 13-month prospective study of daytime use of external hip protectors in an at-risk long-term care population., Setting: One hundred-bed not-for-profit long-term care facility., Participants: Thirty-eight ambulatory residents having at least 1 of 4 risk factors (osteoporosis, recent fall, positive fall screen, previous fracture)., Intervention: The rehabilitation department coordinated an implementation program. Members of the rehabilitation team met with eligible participants, primary caregivers, families, and other support staff for educational instruction and a description of the program. The rehabilitation team assumed overall responsibility for measuring and ordering hip protectors and monitoring compliance., Results: By the end of the third month, hip protector compliance averaged greater than 90% daily wear. The average number of falls per month in the hip protector group was 3.9 versus 1.3 in nonparticipants. Estimated total indirect staff time was 7.75 hours. The total cost of the study (hip protectors and indirect staff time) was 6,300 US dollars., Conclusions: High hip protector compliance is both feasible and sustainable in an at-risk long-term care population. Achieving high compliance requires an interdisciplinary approach with one department acting as a champion. The cost of protectors could be a barrier to widespread use. Facilities might be unable to cover the cost until the product is paid for by third-party payers.