Development of novel clinical examination scales for the measurement of disease severity in Creutzfeldt-Jakob disease
Journal of Neurology, Neurosurgery & Psychiatry — Nihat 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.