Clinical, laboratory, and morphological diagnosis of human subacute spongiform encephalopathies in byelorussia

Protas, I I; Kolomietz, N. D.; Nedzvedz, M. K.; Milkamanovich, E. K.

doi:10.1007/bf00143132

Cited by 1 publication

(2 citation statements)

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“…This stage is characterized by nonspecific neuropsychiatric symptoms [16, 17], depression [18], fatigue, sleep and autonomic dysfunctions [19], and behavioral disturbances [20, 21]. Mental disturbances, including aggressive behavior, psychosis and depression [14], as well as other non specific prodromal symptoms including headache, loss of appetite and loss of weight have also been reported in patients with familial CJD carrying the E200K mutation [22].…”

Section: Discussionmentioning

confidence: 99%

“…In contrast to the relatively stereotyped phenomenology of variant CJD, which has a known progression from behavioral changes to involuntary movements and dementia, [23] and Gerstmann-Sträussler-Scheinker disease [24] in which cerebellar signs predominate over many years, sporadic CJD [19,21] and familial CJD associated with the E200K mutation [22, 25] are heterogeneous in both presentation and progression. There are well known phenotypic variants of CJD including the Heidenhain variant [26], which presents with visual disturbances and the Oppenheimer–Brownell variant [27], which presents with ataxia.…”

Section: Discussionmentioning

confidence: 99%

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

Cohen

Prohovnik

Korczyn

et al. 2011

Acta Neurologica Scandinavica

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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.

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