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.