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Rare histotype of sporadic Creutzfeldt-Jakob disease, clinically suspected as corticobasal degeneration.
BMJ Case Rep 2019; 12(3)BC

Abstract

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disease that can mimic other neurological disorders. We present a case of sCJD in a 64-year-old man that presented with corticobasal syndrome and survived for 3 years. He presented initially with dementia, hemiparkinsonism and alien limb phenomenon and was diagnosed with corticobasal degeneration, ultimately progressing to immobility and akinetic mutism. With a normal MRI 1 year before onset, his neuroimaging 1 year later revealed abnormal DaTscan, cortical and hippocampal atrophy with ventricular dilatation on MRI, and diffusion-weighted cortical ribboning and thalamic hyperintensity. Postmortem, the patient's brain was collected by the Parkinson's UK Tissue Bank. Prion protein immunohistochemistry revealed widespread diffuse microvacuolar staining without kuru-type plaques. Hyperphosphorylated tau was only found in the entorhinal cortex and hippocampus. This case highlights the clinical heterogeneity of sCJD presentation and the important inclusion of CJD in the differential diagnosis of atypical presentations of neurodegenerative disease.

Authors+Show Affiliations

Department of Medicine, Imperial College London, London, UK.National Creutzfeldt-Jakob Disease Research and Surveillance Unit, University of Edinburgh, Edinburgh, UK.Institute of Neuroscience, Newcastle University, Newcastle Upon Tyne, UK.Institute of Neuroscience, Newcastle University, Newcastle Upon Tyne, UK.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

30850568

Citation

Tilley, Bension Shlomo, et al. "Rare Histotype of Sporadic Creutzfeldt-Jakob Disease, Clinically Suspected as Corticobasal Degeneration." BMJ Case Reports, vol. 12, no. 3, 2019.
Tilley BS, Smith C, Pavese N, et al. Rare histotype of sporadic Creutzfeldt-Jakob disease, clinically suspected as corticobasal degeneration. BMJ Case Rep. 2019;12(3).
Tilley, B. S., Smith, C., Pavese, N., & Attems, J. (2019). Rare histotype of sporadic Creutzfeldt-Jakob disease, clinically suspected as corticobasal degeneration. BMJ Case Reports, 12(3), doi:10.1136/bcr-2018-228305.
Tilley BS, et al. Rare Histotype of Sporadic Creutzfeldt-Jakob Disease, Clinically Suspected as Corticobasal Degeneration. BMJ Case Rep. 2019 Mar 7;12(3) PubMed PMID: 30850568.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Rare histotype of sporadic Creutzfeldt-Jakob disease, clinically suspected as corticobasal degeneration. AU - Tilley,Bension Shlomo, AU - Smith,Colin, AU - Pavese,Nicola, AU - Attems,Johannes, Y1 - 2019/03/07/ PY - 2019/3/10/entrez PY - 2019/3/10/pubmed PY - 2019/6/25/medline KW - memory disorders KW - movement disorders (other than parkinsons) KW - pathology JF - BMJ case reports JO - BMJ Case Rep VL - 12 IS - 3 N2 - Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disease that can mimic other neurological disorders. We present a case of sCJD in a 64-year-old man that presented with corticobasal syndrome and survived for 3 years. He presented initially with dementia, hemiparkinsonism and alien limb phenomenon and was diagnosed with corticobasal degeneration, ultimately progressing to immobility and akinetic mutism. With a normal MRI 1 year before onset, his neuroimaging 1 year later revealed abnormal DaTscan, cortical and hippocampal atrophy with ventricular dilatation on MRI, and diffusion-weighted cortical ribboning and thalamic hyperintensity. Postmortem, the patient's brain was collected by the Parkinson's UK Tissue Bank. Prion protein immunohistochemistry revealed widespread diffuse microvacuolar staining without kuru-type plaques. Hyperphosphorylated tau was only found in the entorhinal cortex and hippocampus. This case highlights the clinical heterogeneity of sCJD presentation and the important inclusion of CJD in the differential diagnosis of atypical presentations of neurodegenerative disease. SN - 1757-790X UR - https://www.unboundmedicine.com/medline/citation/30850568/Rare_histotype_of_sporadic_Creutzfeldt-Jakob_disease,_clinically_suspected_as_corticobasal_degeneration L2 - https://casereports.bmj.com/cgi/pmidlookup?view=long&pmid=30850568 DB - PRIME DP - Unbound Medicine ER -