RESUMO
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.
Assuntos
Síndrome de Creutzfeldt-Jakob/epidemiologia , Síndrome de Creutzfeldt-Jakob/patologia , Adulto , Idade de Início , Idoso , Síndrome de Creutzfeldt-Jakob/mortalidade , Feminino , Geografia , Humanos , Masculino , México/epidemiologia , Pessoa de Meia-Idade , Análise de Sobrevida , Fatores de TempoRESUMO
En Chile no hay casos de la nueva variante de la ECJ. Se presentan alrededor de 30 casos de muertes anuales por la forma clásica de la enfermedad. Las muertes no son confirmadas por diagnóstico anatomo-patológico, sino que solamente clínico, lo que disminuye la confiabilidad del diagnóstico. La reciente obligatoriedad de la notificacción de los casos de ECJ, nos dará información más confiable al respecto. El pequeño número de casos de muertes anuales por ECJ hace especialmente difícil el análisis y es necesario recurrir a agrupaciones de casos temporales (quinquenios y decenios) y geográficas para poder obtener algún resultado significativo. A partir del análisis realizado, la mortalidad en Chile se ha mantenido estable en los últimos 20 años, aunque se observa una leve tendencia al aumento, a pesar de no ser significativa estadísticamente. Afecta principalmente a los mayores de 60 años y se observa una mayor mortalidad femenina. Al efectuar el análisis por macroregiones, utilizando la RME para dos decenios, se puede concluir que la macroregión norte (I a IV regiones), presenta un menor riesgo de mortalidad por ECJ que la población estándar del país.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Notificação de Abuso , Síndrome de Creutzfeldt-Jakob/mortalidade , ChileAssuntos
Hormônio do Crescimento Humano/efeitos adversos , Hipopituitarismo/tratamento farmacológico , Criança , Síndrome de Creutzfeldt-Jakob/induzido quimicamente , Síndrome de Creutzfeldt-Jakob/mortalidade , Humanos , Hipopituitarismo/etiologia , Neoplasias/etiologia , Neoplasias/mortalidade , Fatores de Risco , Estados Unidos/epidemiologiaRESUMO
OBJECTIVE: Patients who received pituitary-derived growth hormone (GH) are at excess risk of mortality from Creutzfeldt-Jakob disease. We investigated whether they were at increased risk of death from other conditions, particularly preventable conditions. STUDY DESIGN: A cohort (N=6107) from known US pituitary-derived GH recipients (treated 1963-1985) was studied. Deaths were identified by reports from physicians and parents and the National Death Index. Rates were compared with the expected rates for the US population standardized for race, age, and sex. RESULTS: There were 433 deaths versus 114 expected (relative risk [RR], 3.8; 95% confidence interval [CI], 3.4-4.2; P<.0001) from 1963 through 1996. Risk was increased in subjects with GH deficiency caused by any tumor (RR, 10.4; 95% CI, 9.1-12.0; P<.0001). Surprisingly, subjects with hypoglycemia treated within the first 6 months of life were at extremely high risk (RR, 18.3; 95% CI, 9.2-32.8; P<.0001), as were all subjects with adrenal insufficiency (RR, 7.1; 95% CI, 6.2-8.2; P<.0001). A quarter of all deaths were sudden and unexpected. Of the 26 cases of Creutzfeldt-Jakob disease, four cases have died since 2000. CONCLUSIONS: The death rate in pituitary-derived GH recipients was almost four times the expected rate. Replacing pituitary-derived GH with recombinant GH has eliminated only the risk of Creutzfeldt-Jakob disease. Hypoglycemia and adrenal insufficiency accounted for far more mortality than Creutzfeldt-Jakob disease. The large number of potentially preventable deaths in patients with adrenal insufficiency and hypoglycemia underscores the importance of early intervention when infection occurs in patients with adrenal insufficiency, and aggressive treatment of panhypopituitarism.
Assuntos
Insuficiência Adrenal/mortalidade , Síndrome de Creutzfeldt-Jakob/mortalidade , Hormônio do Crescimento Humano/efeitos adversos , Hormônio do Crescimento Humano/deficiência , Hipoglicemia/mortalidade , Insuficiência Adrenal/tratamento farmacológico , Insuficiência Adrenal/etiologia , Distribuição por Idade , Fatores Etários , Criança , Pré-Escolar , Estudos de Coortes , Síndrome de Creutzfeldt-Jakob/induzido quimicamente , Atestado de Óbito , Morte Súbita/epidemiologia , Epilepsia/mortalidade , Feminino , Humanos , Hipoglicemia/tratamento farmacológico , Hipoglicemia/etiologia , Masculino , Neoplasias/complicações , Neoplasias/mortalidade , Fatores de Risco , Taxa de Sobrevida , Estados Unidos/epidemiologiaRESUMO
Descriptive epidemiological data are presented from a survey of 35 cases of Creutzfeldt-Jakob disease (CJD) that occurred in Chile in the period 1955 to 1977. The average mortality in Chile (0.31 deaths per 1 million) and in urban Santiago (0.73 deaths per 1 million) is compared with data reported from other countries. An increasing incidence in recent years is probably related to a greater awareness of the disease. The familial occurrence of CJD in nine patients from five affected families is described, and its analysis suggests a genetically determined susceptibility with incubation periods of more than 30 years. Horizontal transmission of the disease may have occurred in one patient in whom the disease developed 13 years after the patient married into a family with seven other affected members.