TY - JOUR
T1 - Coexistence of PSP and MSA
T2 - A case report and review of the literature
AU - Uchikado, Hirotake
AU - DelleDonne, Anthony
AU - Uitti, Ryan
AU - Dickson, Dennis W.
N1 - Funding Information:
Acknowledgements This study was supported by NIH grants: P50-AG16574, P01-AG17216, P01-AG14449, P01-AG03949, and P50-NS40256; Mayo Foundation; State of Florida Alzheimer Disease Initiative; and the Society for Progressive Supranuclear Palsy.
PY - 2006/2
Y1 - 2006/2
N2 - Progressive supranuclear palsy (PSP) is a neurodegenerative tauopathy characterized by Parkinsonism, vertical gaze palsy, and early falls. The neuropathology is characterized by neurofibrillary tangles, tufted astrocytes, and coiled bodies, but some brains show other pathologic processes. To investigate the frequency of α-synuclein pathology in PSP with immunohistochemistry and to report the clinical and pathological features of a case of PSP with concomitant Multiple system atrophy (MSA) (PSP/MSA), 290 cases of PSP were screened for α-synuclein pathology with immunohistochemistry. Double-labeling immunohistochemistry was performed on a case of PSP/MSA. Among the PSP cases screened for α-synuclein pathology, a single case of PSP/MSA was detected. The patient was an 86-year-old woman with clinical features consistent with PSP. She had no documented dysautonomia or cerebellar signs, and imaging studies were not diagnostic of MSA. Pathological examination showed τ-immunoreactive neuronal and glial lesions consistent with PSP as well as α-synuclein immunoreactive glial cytoplasmic inclusions diagnostic of MSA. Double-immunolabeling studies showed no co-localization of α-synuclein and τ in most neuronal and glial lesions. Based upon the findings in this case, the neuropathologic changes of PSP and MSA are distinct and independent processes, but they can occasionally coexist.
AB - Progressive supranuclear palsy (PSP) is a neurodegenerative tauopathy characterized by Parkinsonism, vertical gaze palsy, and early falls. The neuropathology is characterized by neurofibrillary tangles, tufted astrocytes, and coiled bodies, but some brains show other pathologic processes. To investigate the frequency of α-synuclein pathology in PSP with immunohistochemistry and to report the clinical and pathological features of a case of PSP with concomitant Multiple system atrophy (MSA) (PSP/MSA), 290 cases of PSP were screened for α-synuclein pathology with immunohistochemistry. Double-labeling immunohistochemistry was performed on a case of PSP/MSA. Among the PSP cases screened for α-synuclein pathology, a single case of PSP/MSA was detected. The patient was an 86-year-old woman with clinical features consistent with PSP. She had no documented dysautonomia or cerebellar signs, and imaging studies were not diagnostic of MSA. Pathological examination showed τ-immunoreactive neuronal and glial lesions consistent with PSP as well as α-synuclein immunoreactive glial cytoplasmic inclusions diagnostic of MSA. Double-immunolabeling studies showed no co-localization of α-synuclein and τ in most neuronal and glial lesions. Based upon the findings in this case, the neuropathologic changes of PSP and MSA are distinct and independent processes, but they can occasionally coexist.
KW - Multiple system atrophy
KW - Progressive supranuclear palsy
KW - Tau
KW - α-Synuclein
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U2 - 10.1007/s00401-005-0022-z
DO - 10.1007/s00401-005-0022-z
M3 - Article
C2 - 16456665
AN - SCOPUS:33646197123
SN - 0001-6322
VL - 111
SP - 186
EP - 192
JO - Acta neuropathologica
JF - Acta neuropathologica
IS - 2
ER -