Atypical progressive supranuclear palsy with corticospinal tract degeneration

Keith Anthony Josephs, Omi Katsuse, Dayne A. Beccano-Kelly, Wen Lang Lin, Ryan J. Uitti, Yasuhiro Fujino, Bradley F Boeve, Michael L. Hutton, Matthew C. Baker, Dennis W Dickson

Research output: Contribution to journalArticle

90 Citations (Scopus)

Abstract

Progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), sporadic multisystem tauopathy, and some forms of frontotemporal dementia with Parkinsonism linked to chromosome 17 are characterized by neuronal and glial lesions accumulating tau protein containing 4 conserved repeats in microtubule-binding domain (4R tau). Corticospinal tract degeneration is not a common feature of 4R tauopathies. Our objective was to describe 12 cases with pathologic features similar to those of PSP but with prominent corticospinal tract degeneration. We reviewed the historical records and neuropathologic evaluation using standardized sampling, immunohistochemistry, semiquantitative analysis, image analysis, and electron microscopy. The mean age at onset and illness duration was 71 and 5.7 years, respectively. Eight cases were female. Eleven cases had clinical evidence of prominent upper motor neuron disease plus extrapyramidal features. There was focal parasagittal cortical atrophy involving motor cortex and degeneration of corticospinal tract with sparing of lower motor neurons like in primary lateral sclerosis. Prominent tau pathology was found in oligodendrocytes in motor cortex, subjacent white matter, and corticospinal tract characterized by globular cytoplasmic filamentous inclusions that were immunoreactive for 4R tau. The clinicopathologic features of these 12 cases expand the spectrum of 4R tauopathies.

Original languageEnglish (US)
Pages (from-to)396-405
Number of pages10
JournalJournal of Neuropathology and Experimental Neurology
Volume65
Issue number4
DOIs
StatePublished - Apr 2006

Fingerprint

Pyramidal Tracts
Tauopathies
Progressive Supranuclear Palsy
Motor Neuron Disease
Motor Cortex
tau Proteins
Frontotemporal Dementia
Chromosomes, Human, Pair 17
Inclusion Bodies
Oligodendroglia
Motor Neurons
Age of Onset
Microtubules
Neuroglia
Atrophy
Electron Microscopy
Immunohistochemistry
Pathology
Progressive supranuclear palsy atypical

Keywords

  • Corticospinal tract
  • Electron microscopy
  • Immunohistochemistry
  • Microglia
  • Oligodendroglia
  • Progressive supranuclear palsy
  • Tauopathy

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neuroscience(all)

Cite this

Atypical progressive supranuclear palsy with corticospinal tract degeneration. / Josephs, Keith Anthony; Katsuse, Omi; Beccano-Kelly, Dayne A.; Lin, Wen Lang; Uitti, Ryan J.; Fujino, Yasuhiro; Boeve, Bradley F; Hutton, Michael L.; Baker, Matthew C.; Dickson, Dennis W.

In: Journal of Neuropathology and Experimental Neurology, Vol. 65, No. 4, 04.2006, p. 396-405.

Research output: Contribution to journalArticle

Josephs, Keith Anthony ; Katsuse, Omi ; Beccano-Kelly, Dayne A. ; Lin, Wen Lang ; Uitti, Ryan J. ; Fujino, Yasuhiro ; Boeve, Bradley F ; Hutton, Michael L. ; Baker, Matthew C. ; Dickson, Dennis W. / Atypical progressive supranuclear palsy with corticospinal tract degeneration. In: Journal of Neuropathology and Experimental Neurology. 2006 ; Vol. 65, No. 4. pp. 396-405.
@article{13a662554ff5476e964d191b508147e7,
title = "Atypical progressive supranuclear palsy with corticospinal tract degeneration",
abstract = "Progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), sporadic multisystem tauopathy, and some forms of frontotemporal dementia with Parkinsonism linked to chromosome 17 are characterized by neuronal and glial lesions accumulating tau protein containing 4 conserved repeats in microtubule-binding domain (4R tau). Corticospinal tract degeneration is not a common feature of 4R tauopathies. Our objective was to describe 12 cases with pathologic features similar to those of PSP but with prominent corticospinal tract degeneration. We reviewed the historical records and neuropathologic evaluation using standardized sampling, immunohistochemistry, semiquantitative analysis, image analysis, and electron microscopy. The mean age at onset and illness duration was 71 and 5.7 years, respectively. Eight cases were female. Eleven cases had clinical evidence of prominent upper motor neuron disease plus extrapyramidal features. There was focal parasagittal cortical atrophy involving motor cortex and degeneration of corticospinal tract with sparing of lower motor neurons like in primary lateral sclerosis. Prominent tau pathology was found in oligodendrocytes in motor cortex, subjacent white matter, and corticospinal tract characterized by globular cytoplasmic filamentous inclusions that were immunoreactive for 4R tau. The clinicopathologic features of these 12 cases expand the spectrum of 4R tauopathies.",
keywords = "Corticospinal tract, Electron microscopy, Immunohistochemistry, Microglia, Oligodendroglia, Progressive supranuclear palsy, Tauopathy",
author = "Josephs, {Keith Anthony} and Omi Katsuse and Beccano-Kelly, {Dayne A.} and Lin, {Wen Lang} and Uitti, {Ryan J.} and Yasuhiro Fujino and Boeve, {Bradley F} and Hutton, {Michael L.} and Baker, {Matthew C.} and Dickson, {Dennis W}",
year = "2006",
month = "4",
doi = "10.1097/01.jnen.0000218446.38158.61",
language = "English (US)",
volume = "65",
pages = "396--405",
journal = "American Journal of Psychotherapy",
issn = "0002-9564",
publisher = "Lippincott Williams and Wilkins",
number = "4",

}

TY - JOUR

T1 - Atypical progressive supranuclear palsy with corticospinal tract degeneration

AU - Josephs, Keith Anthony

AU - Katsuse, Omi

AU - Beccano-Kelly, Dayne A.

AU - Lin, Wen Lang

AU - Uitti, Ryan J.

AU - Fujino, Yasuhiro

AU - Boeve, Bradley F

AU - Hutton, Michael L.

AU - Baker, Matthew C.

AU - Dickson, Dennis W

PY - 2006/4

Y1 - 2006/4

N2 - Progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), sporadic multisystem tauopathy, and some forms of frontotemporal dementia with Parkinsonism linked to chromosome 17 are characterized by neuronal and glial lesions accumulating tau protein containing 4 conserved repeats in microtubule-binding domain (4R tau). Corticospinal tract degeneration is not a common feature of 4R tauopathies. Our objective was to describe 12 cases with pathologic features similar to those of PSP but with prominent corticospinal tract degeneration. We reviewed the historical records and neuropathologic evaluation using standardized sampling, immunohistochemistry, semiquantitative analysis, image analysis, and electron microscopy. The mean age at onset and illness duration was 71 and 5.7 years, respectively. Eight cases were female. Eleven cases had clinical evidence of prominent upper motor neuron disease plus extrapyramidal features. There was focal parasagittal cortical atrophy involving motor cortex and degeneration of corticospinal tract with sparing of lower motor neurons like in primary lateral sclerosis. Prominent tau pathology was found in oligodendrocytes in motor cortex, subjacent white matter, and corticospinal tract characterized by globular cytoplasmic filamentous inclusions that were immunoreactive for 4R tau. The clinicopathologic features of these 12 cases expand the spectrum of 4R tauopathies.

AB - Progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), sporadic multisystem tauopathy, and some forms of frontotemporal dementia with Parkinsonism linked to chromosome 17 are characterized by neuronal and glial lesions accumulating tau protein containing 4 conserved repeats in microtubule-binding domain (4R tau). Corticospinal tract degeneration is not a common feature of 4R tauopathies. Our objective was to describe 12 cases with pathologic features similar to those of PSP but with prominent corticospinal tract degeneration. We reviewed the historical records and neuropathologic evaluation using standardized sampling, immunohistochemistry, semiquantitative analysis, image analysis, and electron microscopy. The mean age at onset and illness duration was 71 and 5.7 years, respectively. Eight cases were female. Eleven cases had clinical evidence of prominent upper motor neuron disease plus extrapyramidal features. There was focal parasagittal cortical atrophy involving motor cortex and degeneration of corticospinal tract with sparing of lower motor neurons like in primary lateral sclerosis. Prominent tau pathology was found in oligodendrocytes in motor cortex, subjacent white matter, and corticospinal tract characterized by globular cytoplasmic filamentous inclusions that were immunoreactive for 4R tau. The clinicopathologic features of these 12 cases expand the spectrum of 4R tauopathies.

KW - Corticospinal tract

KW - Electron microscopy

KW - Immunohistochemistry

KW - Microglia

KW - Oligodendroglia

KW - Progressive supranuclear palsy

KW - Tauopathy

UR - http://www.scopus.com/inward/record.url?scp=33745007339&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=33745007339&partnerID=8YFLogxK

U2 - 10.1097/01.jnen.0000218446.38158.61

DO - 10.1097/01.jnen.0000218446.38158.61

M3 - Article

C2 - 16691120

AN - SCOPUS:33745007339

VL - 65

SP - 396

EP - 405

JO - American Journal of Psychotherapy

JF - American Journal of Psychotherapy

SN - 0002-9564

IS - 4

ER -