Brownell-Oppenheimer variant of sporadic Creutzfeldt-Jakob disease

Last revised by Rohit Sharma on 25 Dec 2022

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.

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