Abstract
Introduction: Prion disease/Transmissible spongiform encephalopathies consists of a family of rare, progressive neurodegenerative disorders which have long incubation periods, are rapidly progressive and always fatal. CJD is caused by prions, which are abnormal pathogenic agents that induce abnormal folding of specific normal cellular proteins. This leads to brain damage and the characteristic signs and symptoms of the disease. There are 4 recognized forms – sporadic, genetic, iatrogenic and variant CJD.
Review of 4 cases admitted to our acute ward for the elderly who were all ultimately diagnosed with CJD.
Case 1: 56 M with 4 month history of dizziness and memory decline. Collateral History revealed development of slow speech, expressive dysphasia and rapid progression. MRI Head showed widespread FLAIR high signal and diffusion restriction.CSF analysis was diagnostic for CJD.
Case 2: 75 F with few weeks history of worsening confusion, short-term memory decline, change in personality, hallucinations and increasing dependence. MRI Head showed abnormal diffusion and cortical diffusion high signal within the insular cortices bilaterally. CSF sample was normal. A clinical diagnosis of CJD was made.
Case 3 : 68F with 2-4 week history of decorticate posturing, issues with lexical fluency, verbal apraxia, fluctuations in behaviour and visual hallucinations. MRI Head showed new restricted diffusion in left middle frontal gyrus cortex and the right cerebral hemisphere. Lumbar Puncture was negative. Diagnosed as Sporadic CJD.
Case 4: 79 F with 2-3 month history of struggling with personal care, with acute decline over 3 weeks. Collateral history noted rapid progression of symptoms which included increasing ataxia, emotional lability and word finding difficulty. CSF analysis was diagnostic for CJD.
Conclusion: These cases highlight the importance of obtaining a detailed collateral history and consideration of alternative diagnoses to dementia or delirium in patients who present with cognitive decline as part of rapidly progressive neurodegenerative disease.