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

Abstract

Objectives-To develop a scale sensitive for the neurological manifestations of CreutzfeldtJakob 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 to 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 of 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.

Role of magnetic resonance imaging, cerebrospinal fluid, and electroencephalogram in diagnosis of sporadic Creutzfeldt-Jakob disease

“…These results are in good agreement with a recent CJD neurological status scale developed to assist in distinguishing sCJD patients [25]. While sCJD may sometimes have a predilection for the cerebellum, MRI methods such as DWI may be insensitive to abnormalities in these regions [26].…”

CSF tau correlates with CJD disease severity and cognitive decline

“…In this study in a unique population of patients with fCJD, we showed a significant positive correlation between CSF tau levels and various measures of disease severity. The highest correlation was evident for our newly developed CJD‐NDS which although not specific to CJD is based on evaluation of the dysfunction in several neurological systems and reflects the versatility of the clinical symptoms in this disorder. In addition, we also found a significant negative correlation between tau levels in the CSF and the cognitive performance as reflected by the MMSE and FAB scales.…”

“…All patients were examined by a neurologist who performed several tests including the minimental state examination (MMSE) , the frontal assessment battery (FAB) , the expanded disability status scale (EDSS) , the NIH Stroke Scale (NIHSS) , and the CJD‐NS .…”

Unusual presentations in patients with E200K familial Creutzfeldt−Jakob disease

“…Sensation was intact and the gait was ataxic. She scored 27/30 on the Mini‐Mental State Examination (MMSE) (communicated in writing) and her score on the CJD neurological status scale (CJD‐NS) was 10 (range 0–52).…”