The Creutzfeldt-Jakob disease (CJD) neurological status scale: a new tool for evaluation of disease severity and progression

Abstract

Cohen OS, Prohovnik I, Korczyn AD, Ephraty L, Nitsan Z, Tsabari R, Appel S, Rosenmann H, Kahana E, Chapman J. The Creutzfeldt–Jakob disease (CJD) neurological status scale: a new tool for evaluation of disease severity and progression.Acta Neurol Scand: 2011: 124: 368–374.© 2011 John Wiley & Sons A/S. Objectives – To develop a scale sensitive for the neurological manifestations of Creutzfeldt–Jakob disease (CJD). Methods – A 26-item CJD neurological status scale (CJD-NS) was created based on characteristic disease manifestations. Each sign was assigned to one of eight neurological systems to calculate a total scale score (TSS) and a system involvement score (SIS). The scale was administered to 37 CJD patients, 101 healthy first-degree relatives of the patients and 14 elderly patients with Parkinson’s disease (PD). Results – The mean TSS (±SD) was significantly higher in patients with CJD (13.19 ± 5.63) compared with normal controls (0.41 ± 0.78) and PD patients (9.71 ± 3.05). The mean SIS was also significantly different between the CJD (5.19 ± 1.22) and PD (2.78 ± 1.18 P ≤ 0.01) groups reflecting the disseminated nature of neurological involvement in CJD. Using a cutoff of TSS > 4 yielded a sensitivity of 97% for CJD, and specificity of 100% against healthy controls. All individual items showed excellent specificity against healthy subjects, but sensitivity was highly variable. Repeat assessments of CJD patients over 3–9 months revealed a time-dependent increase in both the TSS and the SIS reflecting the scale’s ability to track disease progression. Conclusions – The CJD-NS scale is sensitive to neurological signs and their progression in CJD patients.