Sporadic Creutzfeldt–Jakob disease (sCJD) is a rare transmissible disease. According to molecular classification, six clinical phenotypes of sCJD have been described: MM1, MM2, MV1, MV2, VV1 and VV2. MV2 subtype comprises 9% of sCJD cases. Atypical clinical course has been reported to be the main caveat for the diagnosis of the MV2 subtype. We hereby present a rare case of MV2 subtype of sCJD, highlighting the evolution of clinical, EEG and imaging attributes over a two-year period, thus underlining the atypical course of the disease.
ParchiPCastellaniRCapellariS, et al.Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol. 1996;39(6):767-778. doi: 10.1002/ana.410390613.
2.
WieserHGSchindlerKZumstegD. EEG in Creutzfeldt-Jakob disease. Clin Neurophysiol. 2006;117(5):935-951. doi: 10.1016/j.clinph.2005.12.007.
3.
CollinsSJSanchez-JuanPMastersCL, et al.Determinants of diagnostic investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease. Brain. 2006;129(9):2278-2287. doi: 10.1093/brain/awl159.
4.
CDC’s Diagnostic Criteria for Creutzfeldt-Jakob Disease (CJD). 2018. Centers for Disease Control and Prevention. 2019. Accessed February 15, 2021. cdc.gov/prions/ cjd/diagnostic-criteria.html.
5.
BinelliSAgazziPCanafogliaL, et al.Myoclonus in Creutzfeldt-Jakob disease: polygraphic and video-electroencephalography assessment of 109 patients. Mov Disord. 2010;25(16):2818-2827. doi: 10.1002/mds.23397.
6.
MeissnerBKallenbergKSanchez-JuanP, et al.MRI Lesion profiles in sporadic Creutzfeldt-Jakob disease. Neurology. 2009;72(23):1994-2001. doi: 10.1212/WNL.0b013e3181a96e5d.
7.
KrasnianskiASchulz-SchaefferWJKallenbergK, et al.Clinical findings and diagnostic tests in the MV2 subtype of sporadic CJD. Brain. 2006;129(9):2288-2296. doi: 10.1093/brain/awl123.
