Preservation of nigral neurons in Pick's disease with Pick bodies: a clinicopathological and morphometric study of five autopsy cases

Abstract 

Many reports have described loss of neurons in the substantia nigra in Pick's disease (PiD). In those reports, however, “Pick's disease” includes PiD without Pick bodies (PB), and there is only limited data available on regional nigral pathology in PiD with PB. To elucidate the pathological changes of the substantia nigra in PiD with PB, we examined five cases and 12 age-matched controls by morphometry. The number and size of pigmented and nonpigmented neurons, as well as the area of the substantia nigra were examined. The area of the substantia nigra was significantly reduced in PiD with PB. The pigmented and nonpigmented neuron counts in PiD with PB were not statistically different from those in controls. There was a significant reduction in the size of pigmented neurons in PiD with PB to 82% with that in the controls. In addition, after reviewing 48 cases of PiD with PB reported in the literature, we found that none of the cases with typical frontotemporal lobe symptoms exhibited parkinsonism until the terminal stage. These data are useful for discriminating PiD with PB from other diseases showing frontotemporal characteristics, including the frontal lobe degeneration type and the motor neuron disease type of frontotemporal dementia.

Keywords:  Pick's disease, Pick body, Frontotemporal dementia, Substantia nigra, Parkinsonism, Morphometric study