title: The Creutzfeldt-Jakob disease (CJD) neurological status scale: a new tool for evaluation of disease severity and progression.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Neurology and the Sagol Neuroscience Center, Tel-Hashomer, Israel. oren.cohen@sheba.health.gov.il
authors.forename: O S
authors.initials: OS
authors.lastname: Cohen
authors.forename: I
authors.initials: I
authors.lastname: Prohovnik
authors.forename: A D
authors.initials: AD
authors.lastname: Korczyn
authors.forename: L
authors.initials: L
authors.lastname: Ephraty
authors.forename: Z
authors.initials: Z
authors.lastname: Nitsan
authors.forename: R
authors.initials: R
authors.lastname: Tsabari
authors.forename: S
authors.initials: S
authors.lastname: Appel
authors.forename: H
authors.initials: H
authors.lastname: Rosenmann
authors.forename: E
authors.initials: E
authors.lastname: Kahana
authors.forename: J
authors.initials: J
authors.lastname: Chapman
mesh_terms: D000368:Aged; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis*; D003943:Diagnostic Techniques, Neurological; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D012680:Sensitivity and Specificity