title: 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.
  
  
  METHODS
  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.affiliation: 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.
authors.forename: David Andrés
authors.initials: DA
authors.lastname: González
authors.affiliation: b Psychology Service , South Texas Veterans Health Care System , San Antonio , TX.
authors.forename: Jason R
authors.initials: JR
authors.lastname: Soble
mesh_terms: D000368:Aged; D001288:Attention; D007562:Creutzfeldt-Jakob Syndrome / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000523:psychology; D038524:Diffusion Magnetic Resonance Imaging; D018450:Disease Progression; D056344:Executive Function; D017809:Fatal Outcome; D020233:Gait Disorders, Neurologic / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D008297:Male; D009069:Movement Disorders / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology; D009483:Neuropsychological Tests; D014792:Visual Acuity
keywords: CBS; CJD; Case report; Creutzfeldt–Jakob disease; corticobasal syndrome; review