The Heidenhain variant of Creutzfeldt-Jakob disease and concomitant tau pathology: A case report
Language English Country Poland Media print-electronic
Document type Case Reports, Journal Article
PubMed
28236445
DOI
10.1016/j.pjnns.2017.01.011
PII: S0028-3843(16)30115-3
Knihovny.cz E-resources
- Keywords
- Creutzfeldt–Jakob disease, Heidenhain variant, Prion, Visuospatial dysfunction,
- MeSH
- Creutzfeldt-Jakob Syndrome diagnosis pathology MeSH
- Diagnosis, Differential MeSH
- Diffusion Magnetic Resonance Imaging MeSH
- Electroencephalography MeSH
- Fatal Outcome MeSH
- Phosphorylation MeSH
- Comorbidity MeSH
- Humans MeSH
- Brain pathology MeSH
- Neurologic Examination MeSH
- Vision Disorders diagnosis pathology MeSH
- 14-3-3 Proteins cerebrospinal fluid MeSH
- tau Proteins chemical synthesis MeSH
- Aged MeSH
- Tauopathies diagnosis pathology MeSH
- Occipital Lobe pathology MeSH
- Check Tag
- Humans MeSH
- Male MeSH
- Aged MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
- Names of Substances
- 14-3-3 Proteins MeSH
- tau Proteins MeSH
The Heidenhain form of Creutzfeldt-Jakob disease (CJD) is a rare CJD variant with predominantly visual symptoms in the early stages. Clinical manifestations of metamorphopsia, hemianopia and Balint's syndrome correlate with the involvement of the posterior cortical regions. A 71-year old healthy and very active man was admitted because of impaired visual acuity, hemianopia, and gait disturbance progressing over one week. MRI found typical cortical hyperintensities in the occipital regions while rhythm slowing and sharp waves were seen in the occipital regions on EEG. Protein 14-3-3 was detected in the cerebrospinal fluid. Postmortem neuropathology revealed typical histopathological changes consistent with CJD. Moreover, we found deposits of phosphorylated tau protein in the limbic regions that met the criteria for primary age-related tauopathy (PART); representing an additional and interesting finding in our case.
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