Dementia in Down's syndrome: observations from a neurology clinic

Clinical manifestations of dementia were reviewed in 15 Down's syndrome (DS) patients referred to a neurological clinic over a 24-month period for mental deterioration. The ages ranged from 32-64 years. One hundred percent showed personality changes and loss of independent daily living skills, the presenting symptoms in two-thirds of the cases. Other manifestations included seizures (53%), gait deterioration (73%), sphincteric incontinence (40%), and pathological release reflexes (67%). All 7 patients with CT-scans showed moderate or severe central and peripheral cortical atrophy. Detailed clinical information is presented for two patients, one of whom showed a temporary remission with imipramine. A characteristic dementia syndrome appears to be present in a subpopulation of aging DA patients with radiographic findings of Alzheimer's disease.