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

Authors

  • Medo M. Kuotsu Department of Medicine, Regional Institute of Medical Sciences, Imphal, Manipur, India
  • Arjun Bal Kallupurakkal Department of Medicine, Regional Institute of Medical Sciences, Imphal, Manipur, India
  • Nyamnyei Konyak Department of Medicine, Regional Institute of Medical Sciences, Imphal, Manipur, India
  • Keisham Jaya Chanu Department of Medicine, Regional Institute of Medical Sciences, Imphal, Manipur, India
  • Vikie-o Khruomo Department of Medicine, Regional Institute of Medical Sciences, Imphal, Manipur, India
  • Dinesh Kumar Perumal Department of Medicine, Regional Institute of Medical Sciences, Imphal, Manipur, India

DOI:

https://doi.org/10.18203/2349-3933.ijam20210276

Keywords:

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

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.

Author Biographies

Medo M. Kuotsu, Department of Medicine, Regional Institute of Medical Sciences, Imphal, Manipur, India

Post Graduate Trainee, Department of Medicine, Regional Institute of Medical Sciences, Imphal

Arjun Bal Kallupurakkal, Department of Medicine, Regional Institute of Medical Sciences, Imphal, Manipur, India

Post Graduate Trainee, Department of Medicine, Regional Institute of Medical Sciences, Imphal

Nyamnyei Konyak, Department of Medicine, Regional Institute of Medical Sciences, Imphal, Manipur, India

Post Graduate Trainee, Department of Medicine, Regional Institute of Medical Sciences, Imphal

Keisham Jaya Chanu, Department of Medicine, Regional Institute of Medical Sciences, Imphal, Manipur, India

Senior Resident, Department of Neurology, Regional Institute of Medical Sciences, Imphal

Vikie-o Khruomo, Department of Medicine, Regional Institute of Medical Sciences, Imphal, Manipur, India

Post Graduate Trainee, Department of Medicine, Regional Institute of Medical Sciences, Imphal

Dinesh Kumar Perumal, Department of Medicine, Regional Institute of Medical Sciences, Imphal, Manipur, India

Post Graduate Trainee, Department of Medicine, Regional Institute of Medical Sciences, Imphal

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

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Published

2021-01-27

Issue

Vol. 8 No. 2 (2021): February 2021

Section

Case Reports