J Korean Radiol Soc.  2005 Nov;53(5):331-335. 10.3348/jkrs.2005.53.5.331.

Rapidly Aggravated Creutzfeldt-Jacob Disease: Autopsy-Proven Case

• Park SH
• Kang HK
• Yu H
• Lee SC

• KMID: 1582622
• DOI: http://doi.org/10.3348/jkrs.2005.53.5.331

Abstract

Creutzfeldt-Jakob disease (CJD) is one of the transmissible spongiform encephalopathies, which is mediated by what has been known as "prion". It is a rare and fatal progressive neurodegenerative disease that affects the middle and old aged. There are a number of subtypes of CJD, one of which is the sporadic type characterized by rapidly progressing clinical symptoms, including progressive dementia, myoclonic jerk, and pyramidal or extrapyramidal syndrome. Patients usually end up dying within 1 to 2 years of contacting the disease. We report an autopsy-proven case of sporadic CJD with clinical symptoms that progressed within several days, along with dramatic changes on diffusion weighted magnetic resonance images.

Keyword

Brain; Brain, MRI; Brain, infection

MeSH Terms

Brain
Creutzfeldt-Jakob Syndrome
Dementia
Diffusion
Humans
Myoclonus
Neurodegenerative Diseases
Prion Diseases