Brownell-Oppenheimer variant of sporadic Creutzfeldt-Jakob disease
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The Brownell-Oppenheimer 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.
History and etymology
This phenotype was first described by Betty Brownell and David R Oppenheimer, British neuropathologists, in 1965 3.
- 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
- 3. Brownell B & Oppenheimer D. An Ataxic Form of Subacute Presenile Polioencephalopathy (Creutzfeldt-Jakob Disease). J Neurol Neurosurg Psychiatry. 1965;28(4):350-61. doi:10.1136/jnnp.28.4.350 - Pubmed