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

Acta Neurol Scand. 2011 Dec;124(6):368-74. doi: 10.1111/j.1600-0404.2011.01489.x. Epub 2011 Feb 8.


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.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Aged
  • Creutzfeldt-Jakob Syndrome / diagnosis*
  • Diagnostic Techniques, Neurological
  • Disease Progression
  • Female
  • Humans
  • Male
  • Middle Aged
  • Neurologic Examination
  • Neuropsychological Tests*
  • Sensitivity and Specificity