The Creutzfeldt-Jakob disease (CJD) neurological status scale: a new tool for evaluation of disease severity and progression |
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Authors: | Cohen O S Prohovnik I Korczyn A D Ephraty L Nitsan Z Tsabari R Appel S Rosenmann H Kahana E Chapman J |
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Affiliation: | Department of Neurology and the Sagol Neuroscience Center, Tel-Hashomer, Israel. oren.cohen@sheba.health.gov.il |
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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. |
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Keywords: | Creutzfeldt–Jakob disease prion clinical rating scale progression disability |
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