FTLDs with Tau Pathology

Pick's Disease

Clinical features. Pick's disease (PD) was recognized first as a distinct dementia caused by frontotemporal degeneration; it became a model for lobar dementias. The disease usually begins between the ages of 50 and 60 years and affects both men and women equally. It presents with frontal lobe behavioral changes, nonfluent aphasia, cognitive decline, and sometimes extrapyramidal features. It is sporadic, but familial examples with suspected autosomal dominant inheritance are known. The clinical course averages from 4 to 14 years.

Pathology. The characteristic gross feature is a sharply defined knife-blade atrophy of the frontal and temporal lobes, sparing the posterior superior temporal convolutions. The cortical ribbon is thin, and the white matter atrophic and firm. The frontal and temporal horns are considerably enlarged. The basal ganglia, in some cases, are also atrophic (Figs. 5.12 and 5.13).

Histology. The Pick cells, hallmarks of the disease, are swollen neurons that contain a Pick body— an argyro-philic inclusion that immunoreacts for tau protein (Fig. 5.14; Table 5.6). Pick cells are present throughout the atrophic cortex and occasionally in the subcortical gray structures, hypothalamus, and midbrain. The diseased neurons gradually die and, ultimately, the cortex

FIGURE 5.12

Pick's disease. A 50-year-old man began to deteriorate mentally and, by age 55, was diagnosed with dementia. By age 62, he was mute, bedridden, and tube fed. Following a 15-year clinical course, he died at age 65. A. The 800-g brain shows the utmost degree of frontal and temporal lobe atrophy. B. The walls of the frontal lobes are reduced to a thickness of only 3 to 4 mm, and the anterior horns are enormously enlarged. C. The temporal lobes are severely atrophic, the cortex is spongy, and the temporal horns are markedly enlarged.

FIGURE 5.12

Pick's disease. A 50-year-old man began to deteriorate mentally and, by age 55, was diagnosed with dementia. By age 62, he was mute, bedridden, and tube fed. Following a 15-year clinical course, he died at age 65. A. The 800-g brain shows the utmost degree of frontal and temporal lobe atrophy. B. The walls of the frontal lobes are reduced to a thickness of only 3 to 4 mm, and the anterior horns are enormously enlarged. C. The temporal lobes are severely atrophic, the cortex is spongy, and the temporal horns are markedly enlarged.

FIGURE 5.13

Pick's disease. A 60-year-old man became irritable and his memory began to decline. His cognitive skills slowly deteriorated and, by age 66, his short- and long-term memory, object naming, counting, and simple motor activities were greatly impaired. During the following years, he gradually became mute, physically abusive, and wheelchair bound. Following a 16-year clinical course, he died at age 76. Transverse section of the 1,100-g brain shows severe circumscribed symmetrical atrophy of the temporal lobes (knife-blade atrophy), sparing the posterior superior temporal convolutions. The temporal horns are markedly enlarged.

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