Dement Neurocogn Disord.  2012 Jun;11(2):74-77. 10.12779/dnd.2012.11.2.74.

A Case of Familial Creutzfeldt-Jacob Disease (V180I) Initially Presenting with Depression

Affiliations
  • 1Department of Neurology, Veterans hospital, Seoul Medical Center, Seoul, Korea. astro76@naver.com

Abstract

Creutzfeldt-Jakob disease (CJD) is a degenerative neurological disorder that is incurable and invariably fatal. It is characterized by rapidly progressive dementia presenting with memory loss, personality changes and hallucinations. The symptoms of CJD are caused by progressive death of neurons in the central nervous system, which is associated with build-up of the abnormal prion proteins forming amyloids. In human, CJD can be acquired genetically through a mutation of the gene encoding for the prion protein (PRNP). This occurs in only 5-10% of all CJD cases. We report a 64-year old woman with CJD carrying a V180I mutation that features late onset, rapid progression, no periodic sharp wave complexes on electroencephalography, and cortical signal change and edema in bilateral frontotemporoparietal lobes and basal ganglia on MRI.

Keyword

Creutzfeldt-Jakob disease; Prion protein; V180I mutation

MeSH Terms

Amyloid
Basal Ganglia
Central Nervous System
Creutzfeldt-Jakob Syndrome
Dementia
Depression
Edema
Electroencephalography
Female
Hallucinations
Humans
Lifting
Memory Disorders
Nervous System Diseases
Neurons
Prions
Amyloid
Prions
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