The development of Alzheimer's disease in Down's syndrome assessed by auditory event-related potentials

Several studies have reported changes in auditory event-related potentials in patients with Alzheimer's type dementia. These include an increase in latency and a reduction in amplitude of the P300 (P3) response, a late positive component generated about 300 ms after an unexpected stimulus. Alzheimer's type dementia is an almost invariable acompaniment of ageing in Down's syndrome. This study was designed to assess the usefulness of the auditory P300 response as a measure of the onset of dementia in Down's subjects, who because of poor language development may be difficult to assess by psychological tests. Auditory event-related potentials were recorded from 89 Down's subjects, aged 16-66 years. A control group of 29 mentally retarded subjects with fragile-X syndrome and 83 normal volunteer controls were also tested. Clinical psychological testing found evidence of dementia in 16 Down's subjects and none with fragile-X. Furthermore, in the Down's population but not the fragile-X or control groups, there was a marked increase in P300 latency with age starting around 37 years. In controls, the effect of age on P300 latency became significant some 17 years later around the age of 54 years. The premature effect of age on P300 in Down's syndrome was due to the prolonged P300 latency in the 16 subjects showing signs of dementia. It was confirmed that P300 latency increase reflects the development of Alzheimer's dementia in Down's subjects.