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A prospective 14‐year longitudinal follow‐up of dementia in persons with D own syndrome
Author(s) -
McCarron M.,
McCallion P.,
Reilly E.,
Mulryan N.
Publication year - 2014
Publication title -
journal of intellectual disability research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.941
H-Index - 104
eISSN - 1365-2788
pISSN - 0964-2633
DOI - 10.1111/jir.12074
Subject(s) - dementia , medicine , prospective cohort study , intellectual disability , pediatrics , gerontology , longitudinal study , psychiatry , psychology , disease , pathology
Background To examine dementia characteristics, age at onset and associated comorbidities in persons with D own syndrome. Method Seventy‐seven people with D own syndrome aged 35 years and older were followed longitudinally. The diagnosis of dementia was established using the modified I nternational C lassification of D iseases, T enth R evision ( ICD ‐10) criteria and a combination of objective and informant‐based tests. Cognitive tests included the T est for S evere I mpairment and the D own S yndrome M ental S tatus E xamination; adaptive behaviour was measured using the D aily L iving S kills Q uestionnaire. The D ementia Q uestionnaire for M ental R etarded P ersons ( DMR ) was added to the test battery in 2005 and this study includes follow‐up data for this instrument. Results Over the 14‐year period the average age of diagnosis at 55.41 years ( SD = 7.14) was in the higher range of previously reported estimates (51–56 years) and a median survival of 7 years after diagnosis. Persons with dementia in the sample were significantly older than persons without dementia. The presence of dementia was also associated with epilepsy and sensory impairments. Among instruments the DMR appeared most sensitive to tracking change in symptoms over time before diagnosis. Conclusion The previously reported high risk levels for dementia among people with D own syndrome was confirmed in these data as was the value of the instruments utilised in tracking decline and helping to confirm diagnosis even in persons with severe intellectual disability.