Analysis of Chinese patients with sporadic Creutzfeldt-Jakob disease

Abstract

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, incurable, and fatal neurodegenerative disorder. The objective of this study was to describe the clinical features and survival time of Chinese sCJD patients, and to explore the associations between clinical data and survival. In this study, we analysed the clinical data of 21 sCJD patients in a tertiary care hospital and used all Chinese case material available from 152 patients with sCJD in literatures between 2008 and 2018. The mean age of onset of all 173 deceased patients was 61.44 year-olds (y), with the highest incidence in the population of 60 to 69 y. The most common manifestation at disease onset was progressive dementia. With the progression of the disease, the four main clinical symptoms and signs were developed, including myoclonus, visual or cerebella disturbance, pyramidal or extrapyramidal dysfunction, and akinetic mutism. Extrapyramidal symptoms were more frequently observed. The mean survival time was 7.34 months, and 82.10% of cases died within 1 year after disease onset. The follow-up showed that the survival time was longer and the myoclonus sign was more frequently presented in younger-onset sCJD patients. Patients with abnormalities only in cortical regions had a higher frequency of pyramidal dysfunction than patients having lesions in both cortex and basal ganglia. The findings of this study might provide some insight into the clinical characteristics of sCJD patients in China, but further studies could examine the presences of clinical features and survival time in patients with early age of onset in a prospective manner.

Epidemiological and Clinical Characteristics of Sporadic Creutzfeldt–Jakob Disease: A Retrospective Study in Eastern China

“…In this study, we found that there were more female than male sCJD patients, and the female-to-male ratio was 1.39. Two studies of Chinese patients were consistent with our findings, in which the number of females predominated (10,12), but two other studies had reported a higher proportion of males, where the female-to-male ratio was 0.82 and 0.79, respectively (8,14). Because of small sample size and various nationalities in China, there might be biases in those results.…”

“…Besides, the fear and limited understanding of prion disease, as well as the restriction on the conditions of medical institutions, also affect autopsies on patients with this contagious neurodegenerative disease in China. Biopsy could not be performed smoothly and generally in China, either, which was confirmed by previous domestic literature (8,(10)(11)(12)(13). Thus, their vast majority of molecular pathological subtypes remained unknown.…”

“…Besides, clinical features such as gender ratio and survival time are not consistent in several Chinese studies. Data on the prognosis of Chinese sCJD patients is even more deficient (8)(9)(10)(11)(12)(13)(14), and only one study had analyzed the factors affecting it (11). It is urgent to understand the current situation of sCJD in China.…”

Creutzfeldt-Jakob disease with neuroleptic malignant syndrome

“…According to Yang et al study of 173 sCJD cases, extrapyramidal symptoms were noticed in over 77% of patients and were the second most frequent symptom after progressive dementia. [19].…”

Survival Patterns of Human Prion Diseases in Spain, 1998–2018: Clinical Phenotypes and Etiological Clues

“…Knowledge of factors relating to survival might prove helpful in predicting the course of the disease, guiding clinical management and decision-making, and assessing the effectiveness of possible future treatments. To our knowledge, there are few studies on TSE survival ( Puopolo et al, 2003 ; Pocchiari et al, 2004 ; Sanchez-Valle et al, 2004 ; Heinemann et al, 2007 ; Nagoshi et al, 2011 ; Iwasaki et al, 2015 ; Sun et al, 2020 ; Yang et al, 2020 ).…”