Brownell-Oppenheimer variant of sporadic Creutzfeldt-Jakob disease
Citation, DOI, disclosures and article data
At the time the article was created Antonio Rodrigues de Aguiar Neto had no recorded disclosures.
View Antonio Rodrigues de Aguiar Neto's current disclosuresAt the time the article was last revised Rohit Sharma had no financial relationships to ineligible companies to disclose.
View Rohit Sharma's current disclosures- CJD (Oppenheimer-Brownell variant)
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.
Radiographic features
MRI
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.
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
- 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
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