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Characteristics of progressive supranuclear palsy presenting with corticobasal syndrome: a cortical variant.

Ling H, Ling H, de Silva R, Massey LA, Courtney R, Hondhamuni G, Bajaj N, Lowe J, Holton JL, Lees A, Revesz T - Neuropathol. Appl. Neurobiol. (2014)

Bottom Line: We identified that there was a shift of tau burden towards the cortical regions away from the basal ganglia; supporting the notion that PSP-CBS is a 'cortical' PSP variant.PSP-CBS also had less severe neuronal loss in the dorsolateral and ventrolateral subregions of the substantia nigra and more severe microglial response in the corticospinal tract than in PSP-RS; however, neuronal loss in subthalamic nucleus was equally severe in both groups.A better understanding of the factors that influence the selective pathological vulnerability in different PSP variants will provide further insights into the neurodegenerative process underlying tauopathies.

View Article: PubMed Central - PubMed

Affiliation: Reta Lila Weston Institute of Neurological Studies, Institute of Neurology, University College London, London, UK; Queen Square Brain Bank for Neurological Disorders, Institute of Neurology, University College London, London, UK; Sara Koe PSP Research Centre, Institute of Neurology, University College London, London, UK.

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Tau immunohistochemistry in selected brain regions of the two most representative cases. PSP-CBS has significantly greater tau load in the posterior frontal (A) and anterior frontal grey matter (C) when compared with PSP-RS (B, D). Regional tau load in the caudate and subthalamic nucleus are greater in PSP-RS (F, H) than in PSP-CBS (E, G). Median tau load values. Posterior frontal grey matter: (A) PSP-CBS: 1.09; (B) PSP-RS: 0.05; Anterior frontal grey matter: (C) PSP-CBS: 0.18; (D) PSP-RS: 0.04; Caudate: (E) PSP-CBS: 0.11; (F) PSP-RS: 1.06; Subthalamic nucleus: (G) PSP-CBS: 0.05; (H) PSP-RS: 0.29. Tau load for each region, that is, ‘regional’ tau load was expressed as percentage (areal fraction × 100%); ‘areal fraction’, which was computed by Image Pro, was defined by a ratio of the tau-positive immunoreactive pixels to the total number of pixels of the whole field. AT8 immunohistochemistry, bar in panel B represents 225 microns in all the panels.
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fig02: Tau immunohistochemistry in selected brain regions of the two most representative cases. PSP-CBS has significantly greater tau load in the posterior frontal (A) and anterior frontal grey matter (C) when compared with PSP-RS (B, D). Regional tau load in the caudate and subthalamic nucleus are greater in PSP-RS (F, H) than in PSP-CBS (E, G). Median tau load values. Posterior frontal grey matter: (A) PSP-CBS: 1.09; (B) PSP-RS: 0.05; Anterior frontal grey matter: (C) PSP-CBS: 0.18; (D) PSP-RS: 0.04; Caudate: (E) PSP-CBS: 0.11; (F) PSP-RS: 1.06; Subthalamic nucleus: (G) PSP-CBS: 0.05; (H) PSP-RS: 0.29. Tau load for each region, that is, ‘regional’ tau load was expressed as percentage (areal fraction × 100%); ‘areal fraction’, which was computed by Image Pro, was defined by a ratio of the tau-positive immunoreactive pixels to the total number of pixels of the whole field. AT8 immunohistochemistry, bar in panel B represents 225 microns in all the panels.

Mentions: The median ‘regional’ tau load in the posterior frontal cortical grey matter (PSP-CBS: 0.59; PSP-RS: 0.05), anterior frontal cortical grey matter (PSP-CBS: 0.06; PSP-RS: 0.03) and parietal subcortical white matter (PSP-CBS: 0.06; PSP-RS: 0.01) was significantly greater in PSP-CBS than in PSP-RS (P < 0.0033 in all). The median ‘regional’ tau load in the caudate (PSP-CBS: 0.14; PSP-RS: 0.49; P < 0.001), STN (PSP-CBS: 0.21; PSP-RS: 0.40; P < 0.001) and cerebellar white matter (PSP-CBS: 0.02; PSP-RS: 0.06; P = 0.007 with borderline significance) was greater in the PSP-RS than in PSP-CBS (Figures 1 and 2).


Characteristics of progressive supranuclear palsy presenting with corticobasal syndrome: a cortical variant.

Ling H, Ling H, de Silva R, Massey LA, Courtney R, Hondhamuni G, Bajaj N, Lowe J, Holton JL, Lees A, Revesz T - Neuropathol. Appl. Neurobiol. (2014)

Tau immunohistochemistry in selected brain regions of the two most representative cases. PSP-CBS has significantly greater tau load in the posterior frontal (A) and anterior frontal grey matter (C) when compared with PSP-RS (B, D). Regional tau load in the caudate and subthalamic nucleus are greater in PSP-RS (F, H) than in PSP-CBS (E, G). Median tau load values. Posterior frontal grey matter: (A) PSP-CBS: 1.09; (B) PSP-RS: 0.05; Anterior frontal grey matter: (C) PSP-CBS: 0.18; (D) PSP-RS: 0.04; Caudate: (E) PSP-CBS: 0.11; (F) PSP-RS: 1.06; Subthalamic nucleus: (G) PSP-CBS: 0.05; (H) PSP-RS: 0.29. Tau load for each region, that is, ‘regional’ tau load was expressed as percentage (areal fraction × 100%); ‘areal fraction’, which was computed by Image Pro, was defined by a ratio of the tau-positive immunoreactive pixels to the total number of pixels of the whole field. AT8 immunohistochemistry, bar in panel B represents 225 microns in all the panels.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4260147&req=5

fig02: Tau immunohistochemistry in selected brain regions of the two most representative cases. PSP-CBS has significantly greater tau load in the posterior frontal (A) and anterior frontal grey matter (C) when compared with PSP-RS (B, D). Regional tau load in the caudate and subthalamic nucleus are greater in PSP-RS (F, H) than in PSP-CBS (E, G). Median tau load values. Posterior frontal grey matter: (A) PSP-CBS: 1.09; (B) PSP-RS: 0.05; Anterior frontal grey matter: (C) PSP-CBS: 0.18; (D) PSP-RS: 0.04; Caudate: (E) PSP-CBS: 0.11; (F) PSP-RS: 1.06; Subthalamic nucleus: (G) PSP-CBS: 0.05; (H) PSP-RS: 0.29. Tau load for each region, that is, ‘regional’ tau load was expressed as percentage (areal fraction × 100%); ‘areal fraction’, which was computed by Image Pro, was defined by a ratio of the tau-positive immunoreactive pixels to the total number of pixels of the whole field. AT8 immunohistochemistry, bar in panel B represents 225 microns in all the panels.
Mentions: The median ‘regional’ tau load in the posterior frontal cortical grey matter (PSP-CBS: 0.59; PSP-RS: 0.05), anterior frontal cortical grey matter (PSP-CBS: 0.06; PSP-RS: 0.03) and parietal subcortical white matter (PSP-CBS: 0.06; PSP-RS: 0.01) was significantly greater in PSP-CBS than in PSP-RS (P < 0.0033 in all). The median ‘regional’ tau load in the caudate (PSP-CBS: 0.14; PSP-RS: 0.49; P < 0.001), STN (PSP-CBS: 0.21; PSP-RS: 0.40; P < 0.001) and cerebellar white matter (PSP-CBS: 0.02; PSP-RS: 0.06; P = 0.007 with borderline significance) was greater in the PSP-RS than in PSP-CBS (Figures 1 and 2).

Bottom Line: We identified that there was a shift of tau burden towards the cortical regions away from the basal ganglia; supporting the notion that PSP-CBS is a 'cortical' PSP variant.PSP-CBS also had less severe neuronal loss in the dorsolateral and ventrolateral subregions of the substantia nigra and more severe microglial response in the corticospinal tract than in PSP-RS; however, neuronal loss in subthalamic nucleus was equally severe in both groups.A better understanding of the factors that influence the selective pathological vulnerability in different PSP variants will provide further insights into the neurodegenerative process underlying tauopathies.

View Article: PubMed Central - PubMed

Affiliation: Reta Lila Weston Institute of Neurological Studies, Institute of Neurology, University College London, London, UK; Queen Square Brain Bank for Neurological Disorders, Institute of Neurology, University College London, London, UK; Sara Koe PSP Research Centre, Institute of Neurology, University College London, London, UK.

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Related in: MedlinePlus