Parkinson's disease with a typical clinical course of 17 years overlapped by Creutzfeldt-Jakob disease: an autopsy case report

BMC Neurol. 2021 Dec 10;21(1):480. doi: 10.1186/s12883-021-02504-1.

Abstract

Background: Late-stage Parkinson's disease (PD) often presents with neuropsychiatric symptoms such as dementia, psychosis, excessive daytime sleepiness, apathy, depression, and anxiety. However, neuropsychiatric symptoms are the cardinal features of Creutzfeldt-Jakob disease (CJD), raising the possibility that CJD may be an overlooked condition when it accompanies late-stage PD.

Case presentation: We describe a female autopsy case of PD with a typical clinical course of 17 years, in which CJD overlapped with PD during the final year of the patient's life. The patient died aged 85 years. Neuropathological features included widespread Lewy body-related α-synucleinopathy predominantly in the brainstem and limbic system, as well as the typical pathology of methionine/methionine type 1 CJD in the brain.

Conclusions: Our case demonstrates the clinicopathological co-occurrence of PD and CJD in a sporadic patient. The possibility of mixed pathology, including prion pathology, should be taken into account when neuropsychiatric symptoms are noted during the disease course of PD.

Keywords: Creutzfeldt–Jakob disease; Lewy bodies; Parkinson’s disease; alpha-synuclein; prion.

Publication types

  • Case Reports

MeSH terms

  • Autopsy
  • Brain / diagnostic imaging
  • Brain / metabolism
  • Creutzfeldt-Jakob Syndrome* / complications
  • Female
  • Humans
  • Parkinson Disease* / complications
  • Prions* / metabolism

Substances

  • Prions