MULTICYSTIC ENCEPHALOPATHY – CONSIDERING TWO CASE REPORTS

• Main Authors: Bárbara Pereira, Clara Alves Pereira, Joana Pinto, Manuela Santos
• Format: Article
• Language: English
• Published: Centro Hospitalar do Porto 2017-01-01
• Series: Nascer e Crescer
• Subjects: Encefalopatia multiquística; epilepsia; lactente
• Online Access: http://revistas.rcaap.pt/nascercrescer/article/view/10768
• ISSN: 0872-0754; 2183-9417

Introduction: Multicystic encephalopathy is a rare disease where the brain parenchyma is replaced by cysts of different sizes. It has been associated to multiple causes: foetal transfusion in monozygotic twin pregnancy, neonatal encephalopathy, central nervous system infections, stroke, trauma, severe maternal hypotension, among others. The common factor linking all of these conditions is the occurrence of severe cerebral hypoxia. The diagnosis is based on neuroimaging and cranial ultrasonography is considered the first-line exam. It is a disease with poor prognosis and in most cases it develops into severe progressive neurological dysfunction to death. Cases Report: The authors report two cases of multicystic encephalopathy, both with early neonatal presentation but distinct. Discussion/Conclusion: The knowledge of this entity, its risk factors and its important morbidity and mortality in children are essential for the early diagnosis and appropriate approach.