Brownell-Oppenheimer variant of sporadic Creutzfeldt-Jakob disease
Citation, DOI & article data
- CJD (Oppenheimer-Brownell variant)
The Brownell-Oppenheimer phenotypic variant of sporadic Creutzfeldt-Jakob disease (CJD) is a distinct clinical entity with predominant gait ataxia, linked to molecular subtype VV2 1,2.
The EEG findings in patients with Brownell-Oppenheimer phenotype usually reveal the absence of periodic sharp-wave complexes (PSWCs) 1,2. The protein 14-3-3 is usually elevated in CSF analysis in patients with VV2 variant of sCJD 2.
MRI shows cerebellar atrophy and selective signal abnormalities in the cerebellar cortex 1.
References
- 1. Fragoso D, Gonçalves Filho A, Pacheco F et al. Imaging of Creutzfeldt-Jakob Disease: Imaging Patterns and Their Differential Diagnosis. RadioGraphics. 2017;37(1):234-57. doi:10.1148/rg.2017160075
- 2. Appleby B, Appleby K, Crain B, Onyike C, Wallin M, Rabins P. Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants. Arch Neurol. 2009;66(2). doi:10.1001/archneurol.2008.533
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