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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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Quantitative data illustrating median regional tau load in PSP-RS (black) and PSP-CBS (yellow) in 15 selected regions. Error bars represent 95% confidence interval. *Represents statistical significance, P < 0.0033 using the Mann–Whitney U-Test. PFG, posterior frontal grey matter; AFG, anterior frontal grey matter; AFWM, anterior frontal white matter; TG, temporal grey matter; TWM, temporal white matter; PG, parietal grey matter; PWM, parietal white matter; SN, substantia nigra; PONS, pons; CAUD, caudate; PUT, putamen; GP, globus pallidus; STN, subthalamic nucleus; DENT, dentate nucleus; CWM, cerebellar white matter.
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fig01: Quantitative data illustrating median regional tau load in PSP-RS (black) and PSP-CBS (yellow) in 15 selected regions. Error bars represent 95% confidence interval. *Represents statistical significance, P < 0.0033 using the Mann–Whitney U-Test. PFG, posterior frontal grey matter; AFG, anterior frontal grey matter; AFWM, anterior frontal white matter; TG, temporal grey matter; TWM, temporal white matter; PG, parietal grey matter; PWM, parietal white matter; SN, substantia nigra; PONS, pons; CAUD, caudate; PUT, putamen; GP, globus pallidus; STN, subthalamic nucleus; DENT, dentate nucleus; CWM, cerebellar white matter.

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)

Quantitative data illustrating median regional tau load in PSP-RS (black) and PSP-CBS (yellow) in 15 selected regions. Error bars represent 95% confidence interval. *Represents statistical significance, P < 0.0033 using the Mann–Whitney U-Test. PFG, posterior frontal grey matter; AFG, anterior frontal grey matter; AFWM, anterior frontal white matter; TG, temporal grey matter; TWM, temporal white matter; PG, parietal grey matter; PWM, parietal white matter; SN, substantia nigra; PONS, pons; CAUD, caudate; PUT, putamen; GP, globus pallidus; STN, subthalamic nucleus; DENT, dentate nucleus; CWM, cerebellar white matter.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig01: Quantitative data illustrating median regional tau load in PSP-RS (black) and PSP-CBS (yellow) in 15 selected regions. Error bars represent 95% confidence interval. *Represents statistical significance, P < 0.0033 using the Mann–Whitney U-Test. PFG, posterior frontal grey matter; AFG, anterior frontal grey matter; AFWM, anterior frontal white matter; TG, temporal grey matter; TWM, temporal white matter; PG, parietal grey matter; PWM, parietal white matter; SN, substantia nigra; PONS, pons; CAUD, caudate; PUT, putamen; GP, globus pallidus; STN, subthalamic nucleus; DENT, dentate nucleus; CWM, cerebellar white matter.
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