Creutzfeldt-Jakob disease in Mexico

Abstract

Creutzfeldt-Jakob disease (CJD) is classified within the group of transmissible spongiform encephalopathies (TSE). It is a rapidly progressive illness that affects mental functions. The average age of onset is 50 years. Various tests can help orient the clinical diagnosis, but the confirmatory test is still the post mortem analysis. The aim of this study was to describe the epidemiological, clinical and histopathological characteristics of patients diagnosed as suffering from CJD, at the National Institute of Neurology and Neurosurgery of Mexico (NINN). An observational, descriptive and transversal study was conducted. We collected information concerning these cases from the Departments of Epidemiology and Pathology, as well as the clinical charts of the patients with a diagnosis of CJD. Fifteen cases were registered of which three CJD cases were definite, five probable cases were identified, and seven were possible. The average age of the patients was 49 years. Two definite cases were female and one was male. It is important to improve the systems for surveillance of this type of disease and, furthermore, to permit greater accessibility to laboratories where the procedures necessary for supporting diagnosis can be followed.

Can prion disease suspicion be supported earlier? Clinical, radiological and laboratory findings in a series of cases

“…It has been recognized worldwide, at rates of 0.25 to 2 cases per million per year. In Mexico, there are only three previous reports of CJD cases [3][4][5] among other reasons, due to limited knowledge concerning this disease on the part of the medical staff, which causes a lack of notification of cases, and an under-registration of the disease. 1 On the other hand, there are no centers or laboratories of microbiology and genetics where tests to support the diagnosis of the disease can be conducted.…”

“…On the other hand, the familial form features a dominant inheritance pattern. 1,3 This form is caused by somatic mutations that occur in the prionic gene (PRNP) located on the short arm of chromosome 20 at codon 102. 1 Lastly, iatrogenic transmission has also been described.…”

“…[1][2][3] No treatment for CJD exists at the moment, only management of symptoms. 3 The sporadic CJD (sCJD) form is responsible for 85-90% of CJD cases. Several hypothetical mechanism for the origin and…”

“…The main clinical symptoms consist of mood changes, psychoaffective and sleep disorders, rapidly progressive dementia, hyperesthesia, hyperreflexia, muscular fasciculations, tremors, myoclonus (abrupt jerking movements of muscle groups or the entire limbs), visual ailments, and other pyramidal and extrapyramidal signs. 1,3 Tests used to orient the clinical diagnosis of CJD are the 14-3-3 protein in the cerebrospinal fluid (CSF 14-3-3), electroencephalogram (EEG) and magnetic resonance imaging (MRI). [1][2][3] No treatment for CJD exists at the moment, only management of symptoms.…”

“…1,3 Tests used to orient the clinical diagnosis of CJD are the 14-3-3 protein in the cerebrospinal fluid (CSF 14-3-3), electroencephalogram (EEG) and magnetic resonance imaging (MRI). [1][2][3] No treatment for CJD exists at the moment, only management of symptoms. 3 The sporadic CJD (sCJD) form is responsible for 85-90% of CJD cases.…”