Pathologic Features

Grossly, the brain is small and of below average weight. The shape is round with flattened occipital lobes. The

figure 13.38

Down syndrome with Alzheimer's disease. A well-adjusted, neat woman with Down syndrome was easy to get along with and able to perform simple housework until about age 38, when her behavior gradually changed. She neglected her appearance; became irritable with screaming and cursing; and was unable to help with housework. She developed seizures, and her physical and mental condition progressively deteriorated. During the final stage of her illness, she was mute and totally helpless, dying at age 56 years. The cerebral cortex shows numerous neuritic plaques and neurofibrillary tangles (Bodian stain).

figure 13.38

Down syndrome with Alzheimer's disease. A well-adjusted, neat woman with Down syndrome was easy to get along with and able to perform simple housework until about age 38, when her behavior gradually changed. She neglected her appearance; became irritable with screaming and cursing; and was unable to help with housework. She developed seizures, and her physical and mental condition progressively deteriorated. During the final stage of her illness, she was mute and totally helpless, dying at age 56 years. The cerebral cortex shows numerous neuritic plaques and neurofibrillary tangles (Bodian stain).

convolutional pattern is simple, and the first temporal gyri are narrow (Fig. 13.37).

Histologic findings include minor cortical anomalies, diminished dendritic arborization of the neurons, reduced synaptic density, and calcifications in the basal ganglia. The histology of the dementing process is identical to that of Alzheimer's disease (see Fig. 13.38). The gene of amyloid precursor protein (APP), a basic component of the neuritic plaques in both Down syndrome and Alzheimer's disease, maps to chromosome 21.

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