Objective: Alzheimer’s disease occurs at a higher prevalence and an earlier age in individuals with Down syndrome (DS) than typically developing individuals. However, diagnosing dementia in individuals with intellectual disability remains a challenge due to pre-existing cognitive deficits. The aim of this study was to investigate the validity and reliability of the Brazilian version of the Cambridge Examination for Mental Disorders of Older People with Down’s syndrome and Others with Intellectual Disabilities (CAMDEX-DS) for individuals with DS. Methods: Two psychiatrists, working independently, evaluated 92 adults with DS ≥ 30 years of age. The concurrent validity of the CAMDEX-DS was analyzed in relation to the gold standard of established international criteria. In a subgroup of 20 subjects, the concurrent validity of the CAMDEX-DS was analyzed in relation to an independent objective assessment of cognitive decline over three years. We analyzed the inter-rater reliability of cognitive assessment. Results: The diagnostic accuracy of the CAMDEX-DS compared to the gold standard was 96.7%. CAMDEX-DS-based diagnosis was considered consistent with cognitive decline. The probability of a participant with dementia having cognitive decline was 83%. Inter-rater reliability for the participant assessment was good, with a kappa of > 0.8 for 93% of the CAMDEX-DS items. Conclusion: The CAMDEX-DS can be considered the first valid and reliable instrument for evaluating dementia in adults with DS in Brazil. Its use in such individuals could improve clinical practice and research.

Os autores apresentam o caso de criança de 10 anos, portadora de síndrome nefrótica, que, durante a fase de diurese rápida conseqüente à administração de ACTH, apresentou crises convulsivas, coma, alterações do electrencefalograma, do electrocardiograma, hiponatremia, hipocalemia, hipocloremia e alcalose. Como principais seqüelas, o paciente apresentava, ao ter alta hospitalar, síndrome convulsiva e hemianopsia homônima lateral direita. Os autores comentam o possível mecanismo dessas alterações.

Report of a case of a 10 year old nephrotic boy that developed convulsions progressing to a coma, severe electrocardiographic and electroencephalographic changes, hyponatremia, hypokalemia, hypochloremia and alkalosis following massive diuresis secondary to the administration of ACTH. The patient remained with a convulsive disorder and a right lateral homonymous hemianopsia. The mechanism of those severe disturbances is commented on.