Basal ganglia lesions in ‘Pick complex’: A topographic neuropathological study of 19 autopsy cases

Abstract

The distribution of basal ganglia lesions, including the amygdala, striatum (caudate nucleus, putamen), pallidum, and substantia nigra, were reinvestigated in 19 Japanese autopsy cases of 'Pick complex', consisting of five patients with corticobasal degeneration (CBD), 10 patients with Pick's disease with Pick bodies (PDPB), and four patients with generalized variant of Pick's disease (gvPD). The lesions in the amygdala, striatum, and pallidum were classified into three categories (severe, moderate, and slight). The lesions in the substantia nigra were qualitatively judged, compared with normal controls. In CBD, basal ganglia lesions in all five cases were uniform: the pallidum showed severe lesions, the striatum moderate lesions, the amygdala slight lesions, and obvious neuronal loss of the substantia nigra was verified in all five cases. Basal ganglia lesions in 10 cases of PDPB were also uniform: the amygdala disclosed severe to moderate lesions, the striatum moderate to slight lesions, the pallidum slight lesions, while obvious neuronal loss of the substantia nigra was found in only two of nine cases in which this structure was examined. Furthermore, basal ganglia lesions in all four cases of gvPD were uniform: the caudate nucleus showed severe lesions, the putamen and amygdala severe to moderate lesions, the pallidum moderate to slight lesions, and obvious neuronal loss of the substantia nigra was confirmed in all four cases. This study, using conventional staining such as hematoxylin-eosin and Holzer, clarified that there were prominent lesions in the pallidum in CBD, in the amygdala in PDPB, and in the caudate nucleus in gvPD, respectively. In addition, nigral involvement was usually found in CBD and gvPD, but was rarely seen in PDPB. These neuropathological findings may help to elucidate the pathological heterogeneity of basal ganglia lesions in 'Pick complex'.