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Different tau pathology pattern in two clinical phenotypes of progressive supranuclear palsy.
Neurodegener Dis. 2008; 5(6):339-46.ND

Abstract

BACKGROUND

The clinical and pathological heterogeneity of progressive supranuclear palsy (PSP) is well established. Recent clinicopathological studies showed much more severe and more widespread tau pathology in Richardson's syndrome (RS), clinically manifest by early onset, falls, supranuclear gaze palsy, dementia and shorter disease duration than in atypical PSP-parkinsonism (PSP-P) often mimicking Parkinson's disease, in which tau pathology is relatively restricted to substantia nigra, subthalamic nucleus and internal globus pallidus.

OBJECTIVE

To perform a comparative clinicopathological study of 30 autopsy-proven cases of PSP.

METHODS

Retrospective assessment of major clinical signs in 18 patients referred to as RS and 12 PSP-P, and semiquantitative assessment of the severity and distribution pattern of tau pathology in both phenotypes using routine stains and immunohistochemistry.

RESULTS

RS (61% males) and PSP-P (33% males) showed significant differences in clinical symptomatology and course (RS mean duration 4.2 years, PSP-P 13.8 years) and significant differences in histopathology: widespread tau pathology and related multisystem degeneration in RS and more restricted lesions in PSP-P, which, however, were not only involving predominantly the subthalamo-nigral-pallidal system. Cortical tau pathology in both groups was usually restricted to limbic areas, and neocortical Alzheimer-type pathology was only seen in very old or demented PSP patients.

CONCLUSIONS

The present study confirmed the recently reported existence of two distinct clinical phenotypes in patients with pathologically proven PSP-P and RS, showing significant differences in severity and distribution of tau pathology, the latter more severe and more widely distributed than in PSP-P.

Authors+Show Affiliations

Institute of Clinical Neurobiology, Vienna, Austria. kurt.jellinger@univie.ac.at

Pub Type(s)

Journal Article

Language

eng

PubMed ID

18349518

Citation

Jellinger, Kurt A.. "Different Tau Pathology Pattern in Two Clinical Phenotypes of Progressive Supranuclear Palsy." Neuro-degenerative Diseases, vol. 5, no. 6, 2008, pp. 339-46.
Jellinger KA. Different tau pathology pattern in two clinical phenotypes of progressive supranuclear palsy. Neurodegener Dis. 2008;5(6):339-46.
Jellinger, K. A. (2008). Different tau pathology pattern in two clinical phenotypes of progressive supranuclear palsy. Neuro-degenerative Diseases, 5(6), 339-46. https://doi.org/10.1159/000121388
Jellinger KA. Different Tau Pathology Pattern in Two Clinical Phenotypes of Progressive Supranuclear Palsy. Neurodegener Dis. 2008;5(6):339-46. PubMed PMID: 18349518.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Different tau pathology pattern in two clinical phenotypes of progressive supranuclear palsy. A1 - Jellinger,Kurt A, Y1 - 2008/03/18/ PY - 2007/07/17/received PY - 2007/09/26/accepted PY - 2008/3/20/pubmed PY - 2008/12/17/medline PY - 2008/3/20/entrez SP - 339 EP - 46 JF - Neuro-degenerative diseases JO - Neurodegener Dis VL - 5 IS - 6 N2 - BACKGROUND: The clinical and pathological heterogeneity of progressive supranuclear palsy (PSP) is well established. Recent clinicopathological studies showed much more severe and more widespread tau pathology in Richardson's syndrome (RS), clinically manifest by early onset, falls, supranuclear gaze palsy, dementia and shorter disease duration than in atypical PSP-parkinsonism (PSP-P) often mimicking Parkinson's disease, in which tau pathology is relatively restricted to substantia nigra, subthalamic nucleus and internal globus pallidus. OBJECTIVE: To perform a comparative clinicopathological study of 30 autopsy-proven cases of PSP. METHODS: Retrospective assessment of major clinical signs in 18 patients referred to as RS and 12 PSP-P, and semiquantitative assessment of the severity and distribution pattern of tau pathology in both phenotypes using routine stains and immunohistochemistry. RESULTS: RS (61% males) and PSP-P (33% males) showed significant differences in clinical symptomatology and course (RS mean duration 4.2 years, PSP-P 13.8 years) and significant differences in histopathology: widespread tau pathology and related multisystem degeneration in RS and more restricted lesions in PSP-P, which, however, were not only involving predominantly the subthalamo-nigral-pallidal system. Cortical tau pathology in both groups was usually restricted to limbic areas, and neocortical Alzheimer-type pathology was only seen in very old or demented PSP patients. CONCLUSIONS: The present study confirmed the recently reported existence of two distinct clinical phenotypes in patients with pathologically proven PSP-P and RS, showing significant differences in severity and distribution of tau pathology, the latter more severe and more widely distributed than in PSP-P. SN - 1660-2862 UR - https://www.unboundmedicine.com/medline/citation/18349518/Different_tau_pathology_pattern_in_two_clinical_phenotypes_of_progressive_supranuclear_palsy_ DB - PRIME DP - Unbound Medicine ER -