ArticlePrion2017
An autopsy-verified case of steroid-responsive encephalopathy with convulsion and a false-positive result from the real-time quaking-induced conversion assay.
Article in Prion, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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13 citing papers in PubMed, 18 citations in OpenAlex.
- Diagnostic Odyssey: Primary CNS Lymphoma (Lymphoma Cerebri) Presenting as Rapidly Progressive Dementia.Case reports in hematology · 2025Article
- Application of real-time quaking-induced conversion in Creutzfeldt-Jakob disease surveillance.Journal of neurology · 2023Article
- Experimental Bovine Spongiform Encephalopathy in Squirrel Monkeys: The Same Complex Proteinopathy Appearing after Very Different Incubation Times.Pathogens (Basel, Switzerland) · 2022Article
- Validation of Revised International Creutzfeldt-Jakob Disease Surveillance Network Diagnostic Criteria for Sporadic Creutzfeldt-Jakob Disease.JAMA network open · 2022Article
- Sporadic Creutzfeldt-Jakob disease: Real-Time Quaking Induced Conversion (RT-QuIC) assay represents a major diagnostic advance.European journal of histochemistry : EJH · 2021Review
- Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease.The Lancet. Neurology · 2021Review
- Towards an improved early diagnosis of neurodegenerative diseases: the emerging role of in vitro conversion assays for protein amyloids.Acta neuropathologica communications · 2020Review
- Second-Generation RT-QuIC Assay for the Diagnosis of Creutzfeldt-Jakob Disease Patients in Brazil.Frontiers in bioengineering and biotechnology · 2020Article
- RT-QuIC: a new test for sporadic CJD.Practical neurology · 2019Article
- Steroid-responsive encephalopathy with a peculiar CSF biomarker profile in an 89-year-old man.Oxford medical case reports · 2018Article
- Design, implementation, and interpretation of amplification studies for prion detection.Prion · 2018Article
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Authors and funding
10 authors at 4 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
We report an autopsy-verified case of steroid-responsive encephalopathy with convulsion and a false-positive result from the real-time quaking-induced conversion (RT-QUIC) assay. A 61-year-old Japanese man presented with acute onset of consciousness disturbance, and convulsions, but without a past medical or family history of progressive dementia, epilepsy, or prion disease. Brain diffusion and fluid-attenuated inverted recovery MR images revealed edematous cortical hyper-intensity, which diminished after the acute phase. Steroid pulse therapy was partially effective, although he continued to have dementia with myoclonus and psychiatric symptoms, despite resolution of the consciousness disturbance. Cerebrospinal fluid (CSF) analysis revealed a normal cell count, with significantly elevated levels of 14-3-3 protein and total tau protein. In addition, prion protein in the CSF was slowly amplified by the RT-QUIC assay. PRNP gene analysis revealed methionine homozygosity at codon 129 without mutation. The patient died of sudden cardiac arrest at 3 months after the onset of symptoms. The positive result from the RT-QUIC assay led us to suspect involvement of prion disease, although a postmortem assessment revealed that he had pathological changes after convulsion, and no prion disease. This case indicates that convulsion may cause false-positive RT-QUIC results, and that a postmortem evaluation remains the gold standard for diagnosing similar cases.
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