Breaking the news in a rapidly progressive dementia — CASC communication station

FRANZCP/MRCPsych-style station: staged disclosure in probable prion disease — correcting a premature diagnosis, explaining diagnostic tiers, answering transmission and inheritance questions honestly, and moving to a concrete plan without false reassurance.

Prompt

You are the consultation-liaison psychiatrist. Mrs A is the wife of a 63-year-old man admitted ten weeks after the onset of rapid cognitive decline, now with ataxia and myoclonus. A junior doctor told her yesterday that the CSF result 'means Creutzfeldt-Jakob disease'. She has since read about the condition online and is frightened, angry, and asking whether she and their two adult children are at risk. The MRI has now been repeated with diffusion-weighted sequences and shows cortical and striatal restricted diffusion; RT-QuIC is pending. Meet her, correct the misinformation without undermining the team, and agree the next steps.

Candidate instructions (7 minutes)

Meet Mrs A. Establish what she understands, correct the premature certainty, answer her questions about risk to the family, and agree next steps. Do not promise a diagnosis you cannot make, and do not retract hope you have no right to remove.^[1]^[2]

References20Show

[1]Zerr I, Kallenberg K, Summers DM, et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease Brain, 2009.PMID 19773352
[2]Hermann P, Appleby B, Brandel JP, et al. Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease Lancet Neurol, 2021.PMID 33609480
[3]Watson N, Brandel JP, Green A, et al. The importance of ongoing international surveillance for Creutzfeldt-Jakob disease Nat Rev Neurol, 2021.PMID 33972773
[4]Ladogana A, Puopolo M, Croes EA, et al. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada Neurology, 2005.PMID 15883321
[5]Appleby BS, Rincon-Beardsley TD, Appleby KK, et al. Initial diagnoses of patients ultimately diagnosed with prion disease J Alzheimers Dis, 2014.PMID 24934543
[6]Zeidler M, Johnstone EC, Bamber RW, et al. New variant Creutzfeldt-Jakob disease: psychiatric features Lancet, 1997.PMID 9314868
[7]Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-Jakob disease in the UK Lancet, 1996.PMID 8598754
[8]Zeidler M, Sellar RJ, Collie DA, et al. The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease Lancet, 2000.PMID 10791525
[9]Brown P, Brandel JP, Sato T, et al. Iatrogenic Creutzfeldt-Jakob disease, final assessment Emerg Infect Dis, 2012.PMID 22607808
[10]Mead S, Hermann P, Mok TH, et al. Genetic causes and modifiers of prion diseases Lancet Neurol, 2026.PMID 41579904
[11]Day GS Rapidly Progressive Dementia Continuum (Minneap Minn), 2022.PMID 35678409
[12]Chitravas N, Jung RS, Kofskey DM, et al. Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease Ann Neurol, 2011.PMID 21674591
[13]Graus F, Titulaer MJ, Balu R, et al. A clinical approach to diagnosis of autoimmune encephalitis Lancet Neurol, 2016.PMID 26906964
[14]Vitali P, Maccagnano E, Caverzasi E, et al. Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias Neurology, 2011.PMID 21471469
[15]Steinhoff BJ, Zerr I, Glatting M, et al. Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease Ann Neurol, 2004.PMID 15449324
[16]Muayqil T, Gronseth G, Camicioli R Evidence-based guideline: diagnostic accuracy of CSF 14-3-3 protein in sporadic Creutzfeldt-Jakob disease: report of the guideline development subcommittee of the American Academy of Neurology Neurology, 2012.PMID 22993290
[17]Foutz A, Appleby BS, Hamlin C, et al. Diagnostic and prognostic value of human prion detection in cerebrospinal fluid Ann Neurol, 2017.PMID 27893164
[18]Pocchiari M, Puopolo M, Croes EA, et al. Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies Brain, 2004.PMID 15361416
[19]Williams RT, White N, Kearns J, et al. Palliative care symptoms of people living with rapidly progressive prion diseases: a systematic review BMC Palliat Care, 2026.PMID 42046045
[20]Stevenson M, Uttley L, Oakley JE, et al. Interventions to reduce the risk of surgically transmitted Creutzfeldt-Jakob disease: a cost-effective modelling review Health Technol Assess, 2020.PMID 32122460

Breaking the news in a rapidly progressive dementia — CASC communication station

Candidate instructions (7 minutes)

References20Show

[1]Zerr I, Kallenberg K, Summers DM, et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease Brain, 2009.PMID 19773352
[2]Hermann P, Appleby B, Brandel JP, et al. Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease Lancet Neurol, 2021.PMID 33609480
[3]Watson N, Brandel JP, Green A, et al. The importance of ongoing international surveillance for Creutzfeldt-Jakob disease Nat Rev Neurol, 2021.PMID 33972773
[4]Ladogana A, Puopolo M, Croes EA, et al. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada Neurology, 2005.PMID 15883321
[5]Appleby BS, Rincon-Beardsley TD, Appleby KK, et al. Initial diagnoses of patients ultimately diagnosed with prion disease J Alzheimers Dis, 2014.PMID 24934543
[6]Zeidler M, Johnstone EC, Bamber RW, et al. New variant Creutzfeldt-Jakob disease: psychiatric features Lancet, 1997.PMID 9314868
[7]Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-Jakob disease in the UK Lancet, 1996.PMID 8598754
[8]Zeidler M, Sellar RJ, Collie DA, et al. The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease Lancet, 2000.PMID 10791525
[9]Brown P, Brandel JP, Sato T, et al. Iatrogenic Creutzfeldt-Jakob disease, final assessment Emerg Infect Dis, 2012.PMID 22607808
[10]Mead S, Hermann P, Mok TH, et al. Genetic causes and modifiers of prion diseases Lancet Neurol, 2026.PMID 41579904
[11]Day GS Rapidly Progressive Dementia Continuum (Minneap Minn), 2022.PMID 35678409
[12]Chitravas N, Jung RS, Kofskey DM, et al. Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease Ann Neurol, 2011.PMID 21674591
[13]Graus F, Titulaer MJ, Balu R, et al. A clinical approach to diagnosis of autoimmune encephalitis Lancet Neurol, 2016.PMID 26906964
[14]Vitali P, Maccagnano E, Caverzasi E, et al. Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias Neurology, 2011.PMID 21471469
[15]Steinhoff BJ, Zerr I, Glatting M, et al. Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease Ann Neurol, 2004.PMID 15449324
[16]Muayqil T, Gronseth G, Camicioli R Evidence-based guideline: diagnostic accuracy of CSF 14-3-3 protein in sporadic Creutzfeldt-Jakob disease: report of the guideline development subcommittee of the American Academy of Neurology Neurology, 2012.PMID 22993290
[17]Foutz A, Appleby BS, Hamlin C, et al. Diagnostic and prognostic value of human prion detection in cerebrospinal fluid Ann Neurol, 2017.PMID 27893164
[18]Pocchiari M, Puopolo M, Croes EA, et al. Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies Brain, 2004.PMID 15361416
[19]Williams RT, White N, Kearns J, et al. Palliative care symptoms of people living with rapidly progressive prion diseases: a systematic review BMC Palliat Care, 2026.PMID 42046045
[20]Stevenson M, Uttley L, Oakley JE, et al. Interventions to reduce the risk of surgically transmitted Creutzfeldt-Jakob disease: a cost-effective modelling review Health Technol Assess, 2020.PMID 32122460