TY - JOUR
T1 - Ballooned neurons in progressive supranuclear palsy are usually due to concurrent argyrophilic grain disease
AU - Togo, Takashi
AU - Dickson, Dennis W.
N1 - Funding Information:
Acknowledgements The authors thank Virginia Phillips and Linda Rousseau for their expert technical assistance and Dr. Peter Davies for CP13. Most of the cases used in this study were from the Society for Progressive Supranuclear Palsy brain bank and generous donations of family members in this endeavor are greatly appreciated. This study was supported by NIH AG 16574, AG 17216, AG 14449, AG03949 and the Mayo Foundation.
PY - 2002
Y1 - 2002
N2 - Progressive supranuclear palsy (PSP) is a sporadic multisystem neurodegenerative disorder that is one of the major causes of parkinsonism, which shares common biochemical and genetic features with corticobasal degeneration (CBD). Ballooned neurons (BN) are one of the histopathologic hallmarks of CBD and their presence is a neuropathologic feature that helps differentiate PSP from CBD, since BN are uncommon in PSP. There are, however, several reports in the literature of BN in PSP. BN are also a consistent finding in argyrophilic grain disease (AGD), where they are relatively confined to limbic structures, in particular the amygdala. Since AGD has been found with increased frequency in PSP, it is possible that cases of PSP with BN may represent co-existing AGD. In the present study, we investigated this possibility by studying the distribution and the density of BN with αB-crystallin immunostaining in 20 cases of PSP, including equal numbers of cases with and without co-existing AGD. In PSP cases with concurrent AGD, BN were consistently found in limbic areas, but in pure PSP cases, BN were rare, supporting the hypothesis that concurrent AGD may account for most cases of PSP with BN.
AB - Progressive supranuclear palsy (PSP) is a sporadic multisystem neurodegenerative disorder that is one of the major causes of parkinsonism, which shares common biochemical and genetic features with corticobasal degeneration (CBD). Ballooned neurons (BN) are one of the histopathologic hallmarks of CBD and their presence is a neuropathologic feature that helps differentiate PSP from CBD, since BN are uncommon in PSP. There are, however, several reports in the literature of BN in PSP. BN are also a consistent finding in argyrophilic grain disease (AGD), where they are relatively confined to limbic structures, in particular the amygdala. Since AGD has been found with increased frequency in PSP, it is possible that cases of PSP with BN may represent co-existing AGD. In the present study, we investigated this possibility by studying the distribution and the density of BN with αB-crystallin immunostaining in 20 cases of PSP, including equal numbers of cases with and without co-existing AGD. In PSP cases with concurrent AGD, BN were consistently found in limbic areas, but in pure PSP cases, BN were rare, supporting the hypothesis that concurrent AGD may account for most cases of PSP with BN.
KW - Argyrophilic grain disease
KW - Ballooned neurons
KW - Corticobasal degeneration
KW - Progressive supranuclear palsy
KW - βB-crystallin
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U2 - 10.1007/s00401-002-0520-1
DO - 10.1007/s00401-002-0520-1
M3 - Article
C2 - 12070664
AN - SCOPUS:0036944428
SN - 0001-6322
VL - 104
SP - 53
EP - 56
JO - Acta neuropathologica
JF - Acta neuropathologica
IS - 1
ER -