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Neurol Neurochir Pol. 2017 Mar - Apr;51(2):197-200. doi: 10.1016/j.pjnns.2017.01.011. Epub 2017 Feb 10.

The Heidenhain variant of Creutzfeldt-Jakob disease and concomitant tau pathology: A case report.

Author information

1
Department of Neurology, Regional Hospital Pardubice, Czech Republic.
2
Faculty of Health Studies, University of Pardubice, Pardubice, Czech Republic; Department of Neurology, Regional Hospital Pardubice, Czech Republic.
3
Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine, Charles University in Prague and the General University Hospital, Prague, Czech Republic.
4
Department of Pathology and Molecular Medicine, Thomayer Hospital, Prague, Czech Republic; Department of Pathology, First Faculty of Medicine, Charles University in Prague and the General University Hospital, Prague, Czech Republic.
5
Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine, Charles University in Prague and the General University Hospital, Prague, Czech Republic; Department of Neurology, Thomayer Hospital, Prague, Czech Republic.

Abstract

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.

KEYWORDS:

Creutzfeldt–Jakob disease; Heidenhain variant; Prion; Visuospatial dysfunction

PMID:
28236445
DOI:
10.1016/j.pjnns.2017.01.011
[Indexed for MEDLINE]

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