Stern-Garcin variant of sporadic Creutzfeldt-Jakob disease

Last revised by Rohit Sharma on 27 Dec 2022

The Stern-Garcin variant of sporadic Creutzfeldt-Jakob disease (CJD) is a phenotypical variant characterized by prominent early extrapyramidal symptoms, such as parkinsonism 1.

Radiographic features

MRI

MRI shows early involvement of the basal ganglia (striatum) and thalamus, in addition to other typical radiographic features of Creutzfeldt-Jakob disease 1,2. Some authors dichotomise this variant depending on if predominantly involving the basal ganglia (Garcin variant) or if predominantly involving the thalamus (Stern variant) 2, although the clinical implications of this differentiation are uncertain.

History and etymology

The phenotype was first described by K Stern in 1939 and then again by R Garcin and colleagues in 1962 3,4.

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