An atypical case of sporadic Creutzfeldt-Jakob disease with Parkinson's disease

Takashi Iida, Katsumi Doh-Ura, Toshiro Kawashima, Hirofumi Abe, Toru Iwaki

Research output: Contribution to journalArticle

25 Citations (Scopus)

Abstract

We report here an autopsy case of a 64-year-old female with slowly progressive dementia and parkinsonism in a 4-year-long clinical course. Post-mortem examination revealed a severely atrophic brain with spongiform degeneration, neuronal loss and gliosis in the gray matter. Many prion protein plaque deposits were present in the occipital lobe, amygdala and cerebellum. Additionally, Lewy bodies were observed in the brainstem. Prion protein gene analysis of the patient revealed polymorphism at the codon-129 valine heterozygote. This genotype is known to sometimes accompany a missense mutation of the gene in uncommon hereditary prion diseases, but no mutation was found in the open reading frame. Thus, it might be suggested that this case showed simultaneously the features of both sporadic Creutzfeldt-Jakob disease (CJD) with codon-129 valine and Parkinson's disease. However, the predisposing factors for contracting both diseases simultaneously remain to be determined, because the incidence of Parkinson's disease accompanied by CJD is very low.

Original languageEnglish
Pages (from-to)294-297
Number of pages4
JournalNeuropathology
Volume21
Issue number4
DOIs
Publication statusPublished - Dec 1 2001

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Valine
Codon
Parkinson Disease
Autopsy
Lewy Bodies
Occipital Lobe
Creutzfeldt-Jakob Syndrome
Inborn Genetic Diseases
Prion Diseases
Gliosis
Parkinsonian Disorders
Missense Mutation
Heterozygote
Amygdala
Causality
Cerebellum
Open Reading Frames
Genes
Brain Stem
Dementia

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Clinical Neurology

Cite this

An atypical case of sporadic Creutzfeldt-Jakob disease with Parkinson's disease. / Iida, Takashi; Doh-Ura, Katsumi; Kawashima, Toshiro; Abe, Hirofumi; Iwaki, Toru.

In: Neuropathology, Vol. 21, No. 4, 01.12.2001, p. 294-297.

Research output: Contribution to journalArticle

Iida, Takashi ; Doh-Ura, Katsumi ; Kawashima, Toshiro ; Abe, Hirofumi ; Iwaki, Toru. / An atypical case of sporadic Creutzfeldt-Jakob disease with Parkinson's disease. In: Neuropathology. 2001 ; Vol. 21, No. 4. pp. 294-297.
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