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Corticobasal syndrome is an atypical parkinsonian syndrome consisting of a constellation of clinical findings that can be the result of various etiologies. While most cases are a result of a tauopathy, such as corticobasal degeneration, other etiologies must be considered in the evaluation of patients presenting with corticobasal syndrome. We present a case of a patient presenting with clinical features of corticobasal syndrome due to a prion disease, Creutzfeldt-Jakob disease (CJD), who was initially misdiagnosed due to known pitfalls in the CJD diagnostic criteria. We further discuss this unusual manifestation of CJD presenting as corticobasal syndrome and relevant diagnostic consideration in the evaluation of this entity.
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