TranslationBACKGROUND/OBJECTIVE: Progressive encephalopathy (PE) is a heterogeneous group of individually rare diseases, many with an autosomal recessive mode of inheritance. We estimated the increased risk of PE associated with consanguinity. PATIENTS AND METHODS: Using a historic cohort study design, the exposures were country of origin (Pakistan versus Norway) and consanguinity. We included children living in Oslo, born between 1985 and 2003. PE cases were retrieved from an electronic registry of diagnoses coded according to the International Classification of Diseases. Incidence rates were calculated for country of origin. We also estimated population attributable risks caused by consanguinity. RESULTS: We identified 30 …
