부산대학교 도서관

We herein introduce two sisters with young-onset dementia and demonstrate their clinical course, radiographic findings, psychiatric abnormalities and neuropathological findings. They suffered from frontal lobe dementia with decreased motivation, impaired attention, amnestic syndrome and inappropriate affect. Visuospatial disorientation was developed two years later together with fecal and urinary incontinence two to three years later. They finally showed hypokinetic mutism and required nasal tube nutrition in the bed-ridden state five to six years later. Tonic-clonic seizures were observed three years later in the younger sister and ten years later in the elder sister after the onset of their illness. Laboratory tests were unremarkable. Computed tomography of the two cases demonstrated diffuse cerebral atrophy with ventricular enlargement and calcifications in bilateral basal ganglia. Brain magnetic resonance imaging revealed cerebral atrophy with diffuse changes in the white matter on T2-wighted sequences. Pathological bone fractures were not noted in either of their clinical courses. The genetic analysis was not performed because their family did not give a consent. Duration of clinical course of their illness was 22 years in the elder sister and 12 years in the younger sister, respectively.