TY - JOUR
T1 - Cerebrovascular pathology presenting as corticobasal syndrome
T2 - An autopsy case series of “vascular CBS”
AU - Koga, Shunsuke
AU - Roemer, Shanu F.
AU - Kasanuki, Koji
AU - Dickson, Dennis W.
N1 - Funding Information:
Dr. Dickson receives support from the NIH (RF1 AG051504, R01 AG057181, P30 AG062677, and the Rainwater Charitable Foundation). Dr. Dickson is an editorial board member of Acta Neuropathologica, Annals of Neurology, Brain, Brain Pathology, and Neuropathology, and he is editor of American Journal of Neurodegenerative Disease.We would like to thank the patients and their families who donated brains to help further the scientific understanding of neurodegeneration. The authors would also like to acknowledge Ariston L. Librero and Virginia Phillips for histologic support, and Monica Castanedes-Casey (Mayo Clinic, Jacksonville) for immunohistochemistry support. This work is supported by NIH grant P50 NS072187, U54 NS100693, a Jaye F. and Betty F. Dyer Foundation Fellowship in progressive supranuclear palsy research, and CBD Solutions Research Grant.
Funding Information:
Dr. Dickson receives support from the NIH ( RF1 AG051504 , R01 AG057181 , P30 AG062677 , and the Rainwater Charitable Foundation ). Dr. Dickson is an editorial board member of Acta Neuropathologica, Annals of Neurology , Brain , Brain Pathology, and Neuropathology, and he is editor of American Journal of Neurodegenerative Disease .
Funding Information:
We would like to thank the patients and their families who donated brains to help further the scientific understanding of neurodegeneration. The authors would also like to acknowledge Ariston L. Librero and Virginia Phillips for histologic support, and Monica Castanedes-Casey (Mayo Clinic, Jacksonville) for immunohistochemistry support. This work is supported by NIH grant P50 NS072187 , U54 NS100693 , a Jaye F. and Betty F. Dyer Foundation Fellowship in progressive supranuclear palsy research, and CBD Solutions Research Grant.
Publisher Copyright:
© 2019 Elsevier Ltd
PY - 2019/11
Y1 - 2019/11
N2 - Background: The corticobasal syndrome (CBS) is heterogeneous in terms of postmortem neuropathology. While it has been previously studied with antemortem neuroimaging, clinicopathologic features of corticobasal syndrome associated with cerebrovascular pathology (vascular CBS) have yet to be reported. Methods: To identify vascular CBS, we searched the database of the CurePSP Brain Bank for patients with a clinical diagnosis of CBS who failed to meet neuropathologic criteria for corticobasal degeneration (CBD) or other neurodegenerative disease processes, but who had significant cerebrovascular pathology. Hemibrains were assessed macroscopically and processed for histological assessment. Medical records were reviewed to characterize clinical features of vascular CBS. Results: Of 217 patients with an antemortem diagnosis of CBS, we identified three patients with vascular CBS. Multiple infarcts in the watershed regions (frontal lobe and motor cortex), periventricular white matter, thalamus, and basal ganglia were observed in two patients. One patient had no cortical infarcts, but had multiple white matter infarcts and corticospinal tract degeneration. All were clinically thought to have CBS based on progressive asymmetric motor symptoms, including rigidity and apraxia, as well as cognitive impairment. Antemortem imaging studies showed findings of chronic cerebrovascular disease, with infarcts or white matter pathology. Conclusions: This autopsy study of vascular CBS shows that, while rare, cerebrovascular pathology involving the frontal lobe, white matter tracts, basal ganglia, thalamus, and corticospinal tract can underlie clinical features suggestive of CBS. When neuroimaging suggests an alternative explanation, including chronic infarcts in critical regions, caution is merited in considering CBD as the underlying pathology.
AB - Background: The corticobasal syndrome (CBS) is heterogeneous in terms of postmortem neuropathology. While it has been previously studied with antemortem neuroimaging, clinicopathologic features of corticobasal syndrome associated with cerebrovascular pathology (vascular CBS) have yet to be reported. Methods: To identify vascular CBS, we searched the database of the CurePSP Brain Bank for patients with a clinical diagnosis of CBS who failed to meet neuropathologic criteria for corticobasal degeneration (CBD) or other neurodegenerative disease processes, but who had significant cerebrovascular pathology. Hemibrains were assessed macroscopically and processed for histological assessment. Medical records were reviewed to characterize clinical features of vascular CBS. Results: Of 217 patients with an antemortem diagnosis of CBS, we identified three patients with vascular CBS. Multiple infarcts in the watershed regions (frontal lobe and motor cortex), periventricular white matter, thalamus, and basal ganglia were observed in two patients. One patient had no cortical infarcts, but had multiple white matter infarcts and corticospinal tract degeneration. All were clinically thought to have CBS based on progressive asymmetric motor symptoms, including rigidity and apraxia, as well as cognitive impairment. Antemortem imaging studies showed findings of chronic cerebrovascular disease, with infarcts or white matter pathology. Conclusions: This autopsy study of vascular CBS shows that, while rare, cerebrovascular pathology involving the frontal lobe, white matter tracts, basal ganglia, thalamus, and corticospinal tract can underlie clinical features suggestive of CBS. When neuroimaging suggests an alternative explanation, including chronic infarcts in critical regions, caution is merited in considering CBD as the underlying pathology.
KW - Cerebrovascular pathology
KW - Corticobasal syndrome (CBS)
KW - Corticospinal tract degeneration
KW - Vascular parkinsonism
KW - Wallerian degeneration
UR - http://www.scopus.com/inward/record.url?scp=85069270107&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85069270107&partnerID=8YFLogxK
U2 - 10.1016/j.parkreldis.2019.09.001
DO - 10.1016/j.parkreldis.2019.09.001
M3 - Article
C2 - 31621626
AN - SCOPUS:85069270107
VL - 68
SP - 79
EP - 84
JO - Parkinsonism and Related Disorders
JF - Parkinsonism and Related Disorders
SN - 1353-8020
ER -