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Heidenhain Variant of Creutzfeldt-Jakob Disease: A Case Report.
Cureus
August 2024
Neurology, Advocate Lutheran General Hospital, Park Ridge, USA.
Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, fatal neurodegenerative disorder caused by an accumulation of protein-containing particles called prions in the central nervous system. The Heidenhain variant (HvCJD) is a rare subtype of CJD that presents with predominantly visual symptoms at onset. The patient presented in this case had several weeks of visual symptoms prior to hospital admission.
View Article and Find Full Text PDFHeidenhain variant of Creutzfeldt-Jakob disease masquerading as neuromyelitis optica spectrum disorder: recognizing when apheresis is not the answer.
Lab Med
July 2024
Department of Laboratory Medicine, Yale School of Medicine, New Haven, CT, US.
Article Synopsis
- The Heidenhain variant of Creutzfeld-Jakob disease (CJD) is a rare and severe neurological condition that starts with visual problems, often mistaken for other disorders like NMOSD.
- A case study of a 66-year-old man illustrates this, as he experienced vision loss and was treated for NMOSD with immunosuppressive therapy and plasma exchange, but his condition worsened.
- Ultimately, after further investigation, he was diagnosed with CJD, but unfortunately his health had declined significantly by that point, leading to hospice care and his eventual passing.
Heidenhain Variant of Sporadic Creutzfeldt-Jakob Disease with a Variety of Visual Symptoms: A Case Report with Autopsy Study.
Am J Case Rep
March 2023
Department of General Medicine, Saga University Hospital, Saga, Japan.
BACKGROUND Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal disease caused by the change of prion protein (PrP). Affected patients present with rapidly progressive cognitive dysfunction, myoclonus, or akinetic mutism. Diagnosing the Heidenhain variant of sCJD, which initially causes various visual symptoms, can be particularly difficult.
View Article and Find Full Text PDFReduplicative paramnesia as an atypical form of presentation of a Heidenhain variant of Creutzfeldt-Jakob disease: A case report.
J Int Neuropsychol Soc
July 2023
Movement Disorders Unit, Neurology Department, Sant Pau Hospital, Barcelona, Spain.
A previously healthy woman began to present recurrent episodes of reduplicative paramnesia within her home and later structured visual hallucinations. The case was initially oriented as an incipient vascular dementia. Detailed anamnesis and neuropsychological examination suggested a rapidly progressive pattern of neuropsychological deficits mostly attributable to parieto-occipital disturbances with some component of fronto-temporal involvement.
View Article and Find Full Text PDFCase Report: A Case of Creutzfeldt-Jakob Heidenhain Variant Simulating PRES.
Diagnostics (Basel)
June 2022
Neurology Unit, Department of Neurosciences and Rehabilitation, S. Anna University Hospital, 44124 Ferrara, Italy.
The Heidenhain Variant of Creutzfeldt-Jakob disease (CJD) is an uncommon early clinical syndrome of the otherwise regular sporadic CJD, which belongs to the group of prion diseases caused by a transmissible agent, the misfolded form of the prion protein. The most characteristic symptoms of CJD are rapidly progressive cognitive impairment, typical motor manifestations and mental and behavioural changes. Conversely, in the Heidenhain Variant, different kinds of visual disturbances are observed at onset due to microvacuolar spongiform degeneration or, less frequently, confluent spongiform changes in the parieto-occipital area, detectable through brain MRI with hyperintensity in T2-FLAIR or DWI in the same areas.
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