Brownell-Oppenheimer variant of sporadic Creutzfeldt-Jakob disease

Last revised by Assoc Prof Frank Gaillard on 23 Sep 2021

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.

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