Neurol. praxi. 2016;17(3):134 | DOI: 10.36290/neu.2016.041
The heidenhain variant of sporadic Creutzfeldt-Jakob disease
- Neurologické oddělení, Krajská zdravotní a.s. &ndash, Nemocnice Chomutov
Prion diseases are rare neurodegenerative conditions with a lethal course. These diseases are caused by deposition in the brain of a pathologically altered prion protein, leading to gradual neuronal degeneration and irreversible brain damage. Creutzfeldt-Jakob disease (CJD) is the best-known and most common human prion disease. It is clinically manifested by a wide range of symptoms, rapid progression, and poor prognosis. Early-onset rapidly progressive dementia is typical. We report a case of a 49-year-old man with presumed sporadic CJD that was confirmed by neuropathological investigation. It was shown to be a rare Heidenhain variant dominated by disorders of visual-spatial functions in the prodromal phase. In the Czech Republic, the diagnosis of CJD and other prion diseases is dealt with by the National Reference Laboratory for Human Prion Diseases.
Keywords: prion diseases, Creutzfeldt-Jakob disease, Heidenhain variant
Published: August 1, 2016 Show citation
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