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Corticobasal syndrome due to sporadic Creutzfeldt-Jakob disease: a review and neuropsychological case report.
Clin Neuropsychol. 2017 Apr; 31(3):676-689.CN

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.

Authors+Show Affiliations

a Department of Neurology , University of Texas Health Science Center at San Antonio , San Antonio , TX , USA. b Psychology Service , South Texas Veterans Health Care System , San Antonio , TX.b Psychology Service , South Texas Veterans Health Care System , San Antonio , TX.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

27871202

Citation

González, David Andrés, and Jason R. Soble. "Corticobasal Syndrome Due to Sporadic Creutzfeldt-Jakob Disease: a Review and Neuropsychological Case Report." The Clinical Neuropsychologist, vol. 31, no. 3, 2017, pp. 676-689.
González DA, Soble JR. Corticobasal syndrome due to sporadic Creutzfeldt-Jakob disease: a review and neuropsychological case report. Clin Neuropsychol. 2017;31(3):676-689.
González, D. A., & Soble, J. R. (2017). Corticobasal syndrome due to sporadic Creutzfeldt-Jakob disease: a review and neuropsychological case report. The Clinical Neuropsychologist, 31(3), 676-689. https://doi.org/10.1080/13854046.2016.1259434
González DA, Soble JR. Corticobasal Syndrome Due to Sporadic Creutzfeldt-Jakob Disease: a Review and Neuropsychological Case Report. Clin Neuropsychol. 2017;31(3):676-689. PubMed PMID: 27871202.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Corticobasal syndrome due to sporadic Creutzfeldt-Jakob disease: a review and neuropsychological case report. AU - González,David Andrés, AU - Soble,Jason R, Y1 - 2016/11/22/ PY - 2016/11/23/pubmed PY - 2017/6/7/medline PY - 2016/11/23/entrez KW - CBS KW - CJD KW - Case report KW - Creutzfeldt–Jakob disease KW - corticobasal syndrome KW - review SP - 676 EP - 689 JF - The Clinical neuropsychologist JO - Clin Neuropsychol VL - 31 IS - 3 N2 - 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. SN - 1744-4144 UR - https://www.unboundmedicine.com/medline/citation/27871202/Corticobasal_syndrome_due_to_sporadic_Creutzfeldt-Jakob_disease:_a_review_and_neuropsychological_case_report L2 - http://www.tandfonline.com/doi/full/10.1080/13854046.2016.1259434 DB - PRIME DP - Unbound Medicine ER -