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Lewy bodies in progressive supranuclear palsy represent an independent disease process.
J Neuropathol Exp Neurol. 2006 Apr; 65(4):387-95.JN

Abstract

Progressive supranuclear palsy (PSP) is a neurodegenerative tauopathy characterized by Parkinsonism, vertical gaze palsy, and early falls. Lewy bodies (LBs) are detected in approximately 10% of PSP cases, but there is little information on the relationship of LBs to tau pathology. We determined the frequency of LBs in a large series of autopsy-confirmed cases of PSP and studied the density and distribution of LBs, including Parkinson disease stage, in cases with LBs (PSP/LBD). PSP/LBD was compared with pure LB disease (LBD), including assessment of neuronal loss in key brainstem nuclei. Immunohistochemistry for alpha-synuclein revealed LBs in 31 of 290 PSP cases (11%). One case had multiple system atrophy in addition to PSP and was excluded from further study along with 2 PSP/LBD cases with concurrent Alzheimer disease. The 29 cases of PSP/LBD were compared with 30 cases of PSP and 24 cases of LBD. The age, sex, brain weight, Braak neurofibrillary tangle (NFT) stage, as well as counts of NFTs and senile plaques were not different among PSP, LBD, and PSP/LBD, but disease duration was longer in LBD. The Parkinson disease stage was similar, but the density of LBs in most subcortical nuclei tended to be greater in LBD than in PSP/LBD. In contrast, substantia nigra neuronal loss was greater in PSP/LBD than both PSP and LBD. Double immunostaining demonstrated alpha-synuclein and tau in different neurons with few exceptions. The findings suggest that LBs in PSP are similar in distribution to those in LBD and independent of tau pathology. The greater density of LBs in LBD compared with PSP/LBD may be the result of longer disease duration in LBD, whereas greater neuronal loss in the substantia nigra in PSP/LBD may be the result of vulnerability of this brain region to both disease processes.

Authors+Show Affiliations

Department of Neuroscience, Mayo Clinic, Jacksonville, Florida 32224, USA.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

16691119

Citation

Uchikado, Hirotake, et al. "Lewy Bodies in Progressive Supranuclear Palsy Represent an Independent Disease Process." Journal of Neuropathology and Experimental Neurology, vol. 65, no. 4, 2006, pp. 387-95.
Uchikado H, DelleDonne A, Ahmed Z, et al. Lewy bodies in progressive supranuclear palsy represent an independent disease process. J Neuropathol Exp Neurol. 2006;65(4):387-95.
Uchikado, H., DelleDonne, A., Ahmed, Z., & Dickson, D. W. (2006). Lewy bodies in progressive supranuclear palsy represent an independent disease process. Journal of Neuropathology and Experimental Neurology, 65(4), 387-95.
Uchikado H, et al. Lewy Bodies in Progressive Supranuclear Palsy Represent an Independent Disease Process. J Neuropathol Exp Neurol. 2006;65(4):387-95. PubMed PMID: 16691119.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Lewy bodies in progressive supranuclear palsy represent an independent disease process. AU - Uchikado,Hirotake, AU - DelleDonne,Anthony, AU - Ahmed,Zeshan, AU - Dickson,Dennis W, PY - 2006/5/13/pubmed PY - 2006/6/10/medline PY - 2006/5/13/entrez SP - 387 EP - 95 JF - Journal of neuropathology and experimental neurology JO - J Neuropathol Exp Neurol VL - 65 IS - 4 N2 - Progressive supranuclear palsy (PSP) is a neurodegenerative tauopathy characterized by Parkinsonism, vertical gaze palsy, and early falls. Lewy bodies (LBs) are detected in approximately 10% of PSP cases, but there is little information on the relationship of LBs to tau pathology. We determined the frequency of LBs in a large series of autopsy-confirmed cases of PSP and studied the density and distribution of LBs, including Parkinson disease stage, in cases with LBs (PSP/LBD). PSP/LBD was compared with pure LB disease (LBD), including assessment of neuronal loss in key brainstem nuclei. Immunohistochemistry for alpha-synuclein revealed LBs in 31 of 290 PSP cases (11%). One case had multiple system atrophy in addition to PSP and was excluded from further study along with 2 PSP/LBD cases with concurrent Alzheimer disease. The 29 cases of PSP/LBD were compared with 30 cases of PSP and 24 cases of LBD. The age, sex, brain weight, Braak neurofibrillary tangle (NFT) stage, as well as counts of NFTs and senile plaques were not different among PSP, LBD, and PSP/LBD, but disease duration was longer in LBD. The Parkinson disease stage was similar, but the density of LBs in most subcortical nuclei tended to be greater in LBD than in PSP/LBD. In contrast, substantia nigra neuronal loss was greater in PSP/LBD than both PSP and LBD. Double immunostaining demonstrated alpha-synuclein and tau in different neurons with few exceptions. The findings suggest that LBs in PSP are similar in distribution to those in LBD and independent of tau pathology. The greater density of LBs in LBD compared with PSP/LBD may be the result of longer disease duration in LBD, whereas greater neuronal loss in the substantia nigra in PSP/LBD may be the result of vulnerability of this brain region to both disease processes. SN - 0022-3069 UR - https://www.unboundmedicine.com/medline/citation/16691119/Lewy_bodies_in_progressive_supranuclear_palsy_represent_an_independent_disease_process_ L2 - https://academic.oup.com/jnen/article-lookup/doi/10.1097/01.jnen.0000218449.17073.43 DB - PRIME DP - Unbound Medicine ER -