Corticobasal syndrome due to sporadic Creutzfeldt–Jakob disease: a review and neuropsychological case report

Abstract

Objective: Creutzfeldt–Jakob disease (CJD) is a rare, rapidly progressive, and fatal neurodegenerative disease with neuropsychological sequelae. This study highlighted a rare presentation of CJD (e.g. corticobasal syndrome [CBS]), reviewed updated diagnostic criteria and procedures for CJD (e.g. diffusion weighted imaging [DWI], real-time quaking-induced conversion [RT-QuIC]), and discussed differential diagnoses. Method: Case report methodology focused on a 68-year-old, Hispanic, right-handed man with 11 years of education. He presented with a 1–2-month history of gait and motor difficulties (e.g. rigidity, myoclonus). Results: After evaluation, a ‘cortical ribboning’ pattern on DWI and positive RT-QuIC was integrated with performance on neurobehavioral exam (i.e. alien limb phenomenon, unilateral ideomotor apraxia) and neuropsychological testing (i.e. frontal-parietal dysfunction pattern) to reach a diagnosis of sCJD-CBS. The patient expired 3 months after onset of symptoms. Conclusions: This literature review and case report highlighted the importance of staying abreast of developments in neurological literature and the added value of neuropsychology, when integrated with newer procedures, for confirming and excluding diagnostic considerations.

Keywords:

• Case report
• review
• corticobasal syndrome
• Creutzfeldt–Jakob disease
• CBS
• CJD

Acknowledgments

The views expressed herein are those of the authors and do not necessarily reflect the views or the official policy of the Department of Veterans Affairs or U.S. Government. This case study received IRB approval despite informed consent not being obtained from the patient secondary to rapid mortality. We would like to acknowledge Justin O’Rourke, PhD, ABPP, for reviewing an initial draft of this manuscript.

Notes

1. Some authors (i.e. Takada & Geschwind, 2013) recently have begun referring to CJD as Jakob–Creutzfeldt disease since Jakob’s cases were pathologically confirmed. For this case study, we retain the more commonly used diagnostic label of CJD.