Pearls and Oy-sters: Typical Atypical Creutzfeld-Jakob Disease: A Case Presenting as a Rapidly Progressive Corticobasal Syndrome Without Dementia
| Autor: | Yitao Ma, Timothy R Malone, Jason S. Hawley, Zoe Olga Marinides |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Pediatrics
medicine.medical_specialty Cognitive Symptoms business.industry Early disease Cognition Disease Progressive neurodegeneration medicine.disease nervous system diseases Corticobasal Degeneration mental disorders Early dementia medicine Humans Dementia Neurology (clinical) business Rare disease |
| Zdroj: | Neurology. 97:1045-1048 |
| ISSN: | 1526-632X 0028-3878 |
| DOI: | 10.1212/wnl.0000000000012613 |
| Popis: | Creutzfeld-Jakob Disease (CJD) is a rare disease but a common cause of rapidly progressive neurodegeneration. Although the "classic" presentation involves early dementia or behavioral changes, there are well-described atypical variants with less prominent cognitive symptoms at onset. One such variant is Corticobasal Syndrome (CBS), which may be seen with other underlying neurodegenerative processes, but when due to prion disease pathology involves much more rapid progression and certain characteristic imaging findings. This report presents a case presenting as CBS without cognitive or behavioral changes at onset, which rapidly progressed and was determined ultimately to be due to underlying CJD. This case illustrates the need for a high index of suspicion for CJD in order to drive appropriate diagnostic testing and careful review of imaging and EEG findings, which may be subtle in early disease. |
| Databáze: | OpenAIRE |
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