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'.

Patterns of Striatal Degeneration in Frontotemporal Dementia

“…Although previous neuroimaging 7, 9–12, 17, 18, 40, 41 and histopathological studies 21–23 have noted general striatal involvement in FTD, this study provides a systematic investigation of striatal subregion atrophy in early bvFTD, SD, and AD. By focusing on specific striatal subregions and using manual volumetric methods better suited to delineate and quantify these structures, we identified pan-striatal atrophy in bvFTD and more selective putamen and NAcc atrophy in SD.…”

“…Abundant striatal tau pathology, manifesting as NCIs, DNs, GCIs, and white matter threads, has been widely reported in patients with FTLD-tau 23 . In this study, we found severe NAcc atrophy in SD, which rarely results from FTLD-tau 31, 45, 46 , and we explored this finding in available neuropathological materials.…”

Patterns of Striatal Degeneration in Frontotemporal Dementia

“…Although previous neuroimaging 7, 9–12, 17, 18, 40, 41 and histopathological studies 21–23 have noted general striatal involvement in FTD, this study provides a systematic investigation of striatal subregion atrophy in early bvFTD, SD, and AD. By focusing on specific striatal subregions and using manual volumetric methods better suited to delineate and quantify these structures, we identified pan-striatal atrophy in bvFTD and more selective putamen and NAcc atrophy in SD.…”

“…Abundant striatal tau pathology, manifesting as NCIs, DNs, GCIs, and white matter threads, has been widely reported in patients with FTLD-tau 23 . In this study, we found severe NAcc atrophy in SD, which rarely results from FTLD-tau 31, 45, 46 , and we explored this finding in available neuropathological materials.…”

K369I Tau Mice Demonstrate a Shift Towards Striatal Neuron Burst Firing and Goal-directed Behaviour

“…The resulting phenotype includes motor symptoms such as tremor, bradykinesia, muscle rigidity and postural instability that resemble parkinsonism, which were amendable to pharmacological intervention (Ittner et al, 2008;Van Eersel et al, 2010). Such extrapyramidal symptoms are common in FTLD, along with striatal atrophy and neuronal loss, fibrillary gliosis and reduced dopamine transporter binding in caudate/putamen (Rinne et al, 2002;Tsuchiya and Ikeda, 2002;Josephs et al, 2008;Looi et al, 2009;Whitwell et al, 2009;Halabi et al, 2013). These striatalbased changes correlate with the degree of extrapyramidal motor symptoms, as well as with compulsive and repetitive behavioural symptoms in bvFTLD (Rinne et al, 2002;Josephs et al, 2008;Halabi et al, 2013).…”

Patterns of Striatal Degeneration in Frontotemporal Dementia

“…Although previous neuroimaging 7, 9–12, 17, 18, 40, 41 and histopathological studies 21–23 have noted general striatal involvement in FTD, this study provides a systematic investigation of striatal subregion atrophy in early bvFTD, SD, and AD. By focusing on specific striatal subregions and using manual volumetric methods better suited to delineate and quantify these structures, we identified pan-striatal atrophy in bvFTD and more selective putamen and NAcc atrophy in SD.…”

“…Abundant striatal tau pathology, manifesting as NCIs, DNs, GCIs, and white matter threads, has been widely reported in patients with FTLD-tau 23 . In this study, we found severe NAcc atrophy in SD, which rarely results from FTLD-tau 31, 45, 46 , and we explored this finding in available neuropathological materials.…”

K369I Tau Mice Demonstrate a Shift Towards Striatal Neuron Burst Firing and Goal-directed Behaviour

“…The resulting phenotype includes motor symptoms such as tremor, bradykinesia, muscle rigidity and postural instability that resemble parkinsonism, which were amendable to pharmacological intervention (Ittner et al, 2008;Van Eersel et al, 2010). Such extrapyramidal symptoms are common in FTLD, along with striatal atrophy and neuronal loss, fibrillary gliosis and reduced dopamine transporter binding in caudate/putamen (Rinne et al, 2002;Tsuchiya and Ikeda, 2002;Josephs et al, 2008;Looi et al, 2009;Whitwell et al, 2009;Halabi et al, 2013). These striatalbased changes correlate with the degree of extrapyramidal motor symptoms, as well as with compulsive and repetitive behavioural symptoms in bvFTLD (Rinne et al, 2002;Josephs et al, 2008;Halabi et al, 2013).…”

Patterns of Striatal Degeneration in Frontotemporal Dementia

“…Although previous neuroimaging 7, 9–12, 17, 18, 40, 41 and histopathological studies 21–23 have noted general striatal involvement in FTD, this study provides a systematic investigation of striatal subregion atrophy in early bvFTD, SD, and AD. By focusing on specific striatal subregions and using manual volumetric methods better suited to delineate and quantify these structures, we identified pan-striatal atrophy in bvFTD and more selective putamen and NAcc atrophy in SD.…”

“…Abundant striatal tau pathology, manifesting as NCIs, DNs, GCIs, and white matter threads, has been widely reported in patients with FTLD-tau 23 . In this study, we found severe NAcc atrophy in SD, which rarely results from FTLD-tau 31, 45, 46 , and we explored this finding in available neuropathological materials.…”

K369I Tau Mice Demonstrate a Shift Towards Striatal Neuron Burst Firing and Goal-directed Behaviour

“…The resulting phenotype includes motor symptoms such as tremor, bradykinesia, muscle rigidity and postural instability that resemble parkinsonism, which were amendable to pharmacological intervention (Ittner et al, 2008;Van Eersel et al, 2010). Such extrapyramidal symptoms are common in FTLD, along with striatal atrophy and neuronal loss, fibrillary gliosis and reduced dopamine transporter binding in caudate/putamen (Rinne et al, 2002;Tsuchiya and Ikeda, 2002;Josephs et al, 2008;Looi et al, 2009;Whitwell et al, 2009;Halabi et al, 2013). These striatalbased changes correlate with the degree of extrapyramidal motor symptoms, as well as with compulsive and repetitive behavioural symptoms in bvFTLD (Rinne et al, 2002;Josephs et al, 2008;Halabi et al, 2013).…”