Development of novel clinical examination scales for the measurement of disease severity in Creutzfeldt-Jakob disease

Journal of Neurology, Neurosurgery & PsychiatryNihat A, Mok TH, Odd H, et al. | January 13, 2022


By using a robust statistical methodology, researchers herein constructed clinical rating scales that would aid in quantifying longitudinal motor and cognitive dysfunction in sporadic Creutzfeldt-Jakob disease (sCJD) at the bedside.

  • To demonstrate the utility of the scale, researchers used a longitudinal clinical examination dataset constructed from 528 patients with sCJD, comprising 1,030 Motor Scale and 757 Cognitive Scale scores over 130 patient-years of study.

  • Eight items, including assessments reliant on pyramidal, extrapyramidal and cerebellar systems, are included in the Rasch-derived Motor Scale.

  • Six items, including measures of executive function, language, visual perception and memory, are included in the Cognitive Scale.

  • By averaging both scores, derivation of a composite Examination Scale can be done.

  • The scales are suggested as valuable for objectively measuring phenotypic and clinical change in future clinical trials and for patient stratification.

  • This statistical approach can aid in overcoming challenges encountered in determining clinical change in rapidly progressive, multisystem conditions with limited longitudinal follow-up.

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