Forma esporádica de enfermedad de Creutzfeldt-Jakob: reporte de dos casos
| Autor: | Guillermo Gonzalez M, Cindy Beltrán E, Efrain Amaya V, Nicolas Vargas P, Ángel Galván Marín |
|---|---|
| Rok vydání: | 2015 |
| Předmět: |
Pathology
medicine.medical_specialty Pediatrics Ataxia biology Vascular disease business.industry Tau protein General Engineering Disease Creutzfeldt-Jakob Syndrome medicine.disease nervous system diseases mental disorders medicine biology.protein Dementia Differential diagnosis medicine.symptom business Myoclonus |
| Zdroj: | Acta Neurológica Colombiana. 31:291-298 |
| ISSN: | 2422-4022 0120-8748 |
| DOI: | 10.22379/2422402244 |
| Popis: | Creutzfeldt-Jakob is the most common spongiform encephalopathy in humans and the prototype of prions diseases. Astrogliosis and degeneration of the gray matter are the histological features. Typically starts with nonspecific prodromal symptoms that progressing to dementia with myoclonus and ataxia. We present two cases of women in middle age with progressive cognitive impairment, motor difficulties, language disorders and myoclonus that lead to death. EEG slow periodic triphasic waves and elevated protein tau and CSF14-3-3 support for The National Prion Disease Pathology Surveillance Center - Cleveland, all these findings define the conditions for the clinical diagnosis of prion disease. The differential diagnosis in the context of rapidly progressive dementia is broad including infections, poisoning, metabolic disorders, autoimmunity, vascular disease and neoplasms that could explain a possible underreporting in regional statistics. There is a possible risk association between disease and Medical Pathologists that although discussed could limit the study of histological specimens that are key to definitive diagnosis. Despite the public health importance of these conditions the current model of health limits the comprehensive management of patients. |
| Databáze: | OpenAIRE |
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