Dr. Luís Fabiano Marin – Rua Maranhão 192 / 101 01240-000 São Paulo SP Brasil. E-mail: [email protected] Creutzfeldt-Jakob disease (CJD) is a transmissible spongiform encephalopathy or prionic disease characterized by accumulation of abnormal prionic protein (PrP) associated to spongiform degeneration of the brain. Although rare, CJD is the most common prionic disease in humans with an inc...

According to recent studies, the determination of cerebrospinal fluid (CSF) total tau (t-tau)/phosphorylated tau (p-tau) ratio and total prion protein (t-PrP) levels significantly improves the accuracy of the diagnosis of Alzheimer's disease (AD) in atypical cases with clinical or laboratory features mimicking Creutzfeldt-Jakob disease (CJD). However, this has neither been validated nor tested ...

Creutzfeldt-Jacob disease (CJD) is a rare and transmissible neurological disorder, which has been increasing in frequency in the United States over the past two decades [1]. CJD is a spongiform encephalopathy characterized by a conformational change of prion protein [2]. The death rate for CJD in the USA is 0.9 per million [1]. We report 7 cases of CJD from North Shore University Hospital, a co...

Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder in which accumulation of the pathogenic prion protein induces neuronal damage and results in distinct pathologic features. This abnormal prion is an infectious protein and resistant to methods of sterilization currently being used. Therefore, management of definite, or suspected CJD patients requires additional precautions. W...

Sporadic Creutzfeldt-Jakob disease (CJD) is the commonest human spongiform encephalopathy. The CJD Surveillance Project in the UK has recently extended the phenotype of this disorder by the identification of (1) a new variant of CJD in young patients, and (2) a subset of older sporadic CJD patients with accumulation of protease-resistant prion protein (PrPRES) in the brainstem and spinal cord. ...

Objective: We used permutation entropy (PE) to disclose abnormalities of cerebral activity in patients with early genetic or sporadic Creutzfeldt-Jakob disease (CJD), as compared to patients with steroid-sensitive subacute encephalopathies (SSE) and healthy volunteers (HV). Methods: We evaluated 16 EEG of CJD patients, 21 of SSE patients and 33 of HV. EEG of CJD patients were performed at an ea...

Creutzfeldt-Jakob disease (CJD) is an untreatable rare human prion disease characterized by rapidly progressive dementia along with various neurological features, including myoclonus and sometimes other movement disorders. The clinical course is typically insidious and rapid, leading to an early death. In general, the most common form is sporadic CJD; however, Slovakia is typical for a high per...