DOI: http://dx.doi.org/10.18203/2349-3933.ijam20210276

A rare case of sporadic Creutzfeldt-Jakob disease in an 83 years old female

Medo M. Kuotsu, Arjun Bal Kallupurakkal, Nyamnyei Konyak, Keisham Jaya Chanu, Vikie-o Khruomo, Dinesh Kumar Perumal

Abstract


Sporadic Creutzfeldt-Jakob disease is a rare invariably fatal neurodegenerative prion disease. Prion disease are associated with the conversion of alpha-helix rich cellular prion protein (PrPC) into a beta-structure rich insoluble conformer scrapie isoform (PrPSc) thought to be infectious isoform. Here we present a case of an 83 years old woman with findings of rapidly progressive dementia, cognitive disturbance, myoclonic jerks and extrapyramidal signs (cogwheel rigidity). Following a series of clinical and diagnostic (diffusion-weighted magnetic resonance imaging (MRI) brain, electroencephalogram (EEG)) examination she was diagnosed with sporadic Creutzfeldt-Jakob disease based on Centers for disease control and prevention (CDC) criteria.


Keywords


Creutzfeldt-Jakob Disease, Prions, Dementia, Myoclonus, Prognosis

Full Text:

PDF

References


Prusiner SB. Prions. Proc Natl Acad Sci. 1998;95:13363-383.

Prusiner SB, Miller BL. Prion Diseases. In: Kasper, Hauser (20thEd.), Harrison’s Principles of Internal Medicine. McGraw Hill, New York.

P Brown, F Cathala, P Castaigne, Gajdusek DC. Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases. Ann Neurol. 1986;20(5):597-602.

Geschwind MD. Prion Diseases. Continuum (Minneap Minn). Neuro infec dis. 2015;21(6):1612-638.

Kuotsu MM et al. Int J Adv Med. 2021 Feb;8(2):279-282

International Journal of Advances in Medicine | February 2021 | Vol 8 | Issue 2 Page 282

Vitali P, Migliaccio R, Agosta F, Rosen HJ, Geschwind MD. Neuroimaging in dementia. Semin Neurol. 2008;28(4):467-83.

Zerr I, Kallenberg K, Summers DM, Romero C, Taratuto A, Heinemann U, Breithaupt U et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain. 2009;132:2659-668.