Abstract
Neuropathologically, Alzheimer-type abnormalities are demonstrated in patients with
Down syndrome (DS), both demented and nondemented and more than a half of patients
with DS above 50 years develop Alzheimer's disease (AD). The apolipoprotein E epsilon4
allele, oestrogen deficiency, high levels of Aβ1–42 peptide, elevated expression of
BACE2, and valine polymorphism of prion protein gene are associated with earlier onset
of dementia in DS individuals. Advanced AD alone may be an important risk factor for
new-onset seizures in older adults and age above 60 years is a recognized risk factor
for poor outcome from convulsive and nonconvulsive status epilepticus. DS patients
aged over 45 years are significantly more likely to develop Alzheimer's disease than
those less than 45 years and up to 84% demented individuals with DS develop seizures.
Late-onset epilepsy in DS is associated with AD, while early-onset epilepsy is associated
with an absence of dementia. In AD patients with a younger age of dementia onset are
particularly susceptible to seizures. DS adults with epilepsy score significantly
higher overall on the adaptive behaviour profile. Language function declined significantly
more rapidly in AD patients with seizures and there is a good correlation between
the severity of EEG abnormalities and cognitive impairment whereas in DS slowing of
the dominant occipital rhythm is related to AD and the frequency of the dominant occipital
activity decreases at the onset of cognitive deterioration.
Keywords
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Article info
Publication history
Accepted:
July 21,
2004
Received in revised form:
July 20,
2004
Received:
January 6,
2004
Identification
Copyright
© 2004 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
