Study on the characteristics of patients with Creutzfeld-Jakob disease in Shaanxi Province，2006-2010

Objective To describe the epidemiological and clinical characteristics of Creutzfeldt-Jakob disease (CJD) in Shaanxi Province. Methods Clinical and epidemical data on 42 suspected CJD patients from clinical hospitals in Shaanxi from 2006 to 2010 was analyzed. 42 blood samples, 41 cerebral spinal fluid (CSF) specimens and 1 brain tissue from these patients were collected. Western blot assay was used to detect PrP^Sc in brain tissue and 14-3-3 protein in CSF. PCR and sequencing were used for analyzing the polymorphism of 129 amino acids and mutation of PRNP gene. Results A total number of 18 probable and three possible sporadic CJD patients, two familial CJD cases were identified. No geographic- or occupational-related events were observed among these cases. The mean age of onset was 55.9 years old, the fender ratio was 1.25:1. Rapid progressive dementia was the main symptom, presenting in 34.78 percent of the CJD patients. Conclusions This report indicates that the main type of CJD in Shaanxi Province is sporadic CJD with its distinctive characteristics including geography distribution, occupation, gender ratio and the average of onset. Follow-up visits to probable and possible CJD patients may contribute to a proper diagnosis.