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Research Letter |

Globular Glial Tauopathy Presenting as Semantic Variant Primary Progressive Aphasia

Jonathan Graff-Radford, MD1; Keith A. Josephs, MD, MST, MSc1; Joseph E. Parisi, MD1,2 ; Dennis W. Dickson, MD3; Caterina Giannini, MD, PhD2 ; Bradley F. Boeve, MD1
[+] Author Affiliations
1Department of Neurology, Mayo Clinic, Rochester, Minnesota
2 Department of Laboratory Medicine and Pathology (Neuropathology), Mayo Clinic, Rochester, Minnesota
3Department of Neuroscience, Neuropathology Laboratory, Mayo Clinic, Jacksonville, Florida
JAMA Neurol. 2016;73(1):123-125. doi:10.1001/jamaneurol.2015.2711.
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This case report describes a woman in her 60s who presented with progressive anomia with loss of word knowledge, prosopagnosia, and surface dyslexia.

Semantic variant primary progressive aphasia (svPPA) most often is due to TAR DNA-binding protein 43 (TDP-43) pathology.1 Herein, we report a case of svPPA due to a globular glial tauopathy (GGT).

Article InformationCorresponding Author: Bradley F. Boeve, MD, Mayo Clinic, 200 1st St SW, Rochester, MN 55905 (bboeve@mayo.edu).

Published Online: November 16, 2015. doi:10.1001/jamaneurol.2015.2711.

Author Contributions: Dr Graff-Radford had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. All authors reviewed the manuscript and were in agreement with its content and provided final approval of the version to be published.

Study concept and design: Graff-Radford, Josephs, Parisi, Boeve.

Acquisition, analysis, or interpretation of data: Graff-Radford, Parisi, Dickson, Giannini, Boeve.

Drafting of the manuscript: Graff-Radford.

Critical revision of the manuscript for important intellectual content: All authors.

Administrative, technical, or material support: Parisi, Giannini, Boeve.

Study supervision: Josephs, Parisi, Giannini, Boeve.

Conflict of Interest Disclosures: None reported.

Funding/Support: This study was funded by grant P50 AG16574 from the National Institutes of Health.

Role of the Funder/Sponsor: The National Institutes of Health had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

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Figure 1.
Neuropsychologic Testing and Magnetic Resonance Imaging

A, All raw scores were converted to scaled scores based on Mayo Older American Normative Studies (MOANS) norms (mean [SD], 10 [3]). AVLT delay indicates retention on the Auditory Verbal Learning Test; BNT, Boston Naming Test; Categ, category; DRS, Dementia Rating Scale; Flu, Fluency; JLO, Judgment of Line Orientation; Rey-O, Rey-Osterrieth Complex Figure Test; TMT, Trail Making Test; WAIS-BD, Block Design subtest of the Wechsler Adult Intelligence Scale–Revised; WMS-LM-R, Logical Memory of the Wechsler Memory Scale–Revised; Stroop CW, Stroop color-word test. B, Coronal T1-weighted magnetic resonance images of the patient in her early 70s showing continued disproportional left anterior temporal lobe atrophy.

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Figure 2.
Histopathology Demonstrating Globular Glial Tauopathy

Globular glial tauopathy, type I, with 4R tau–positive globular glial inclusions, predominantly oligodendroglial and to a lesser extent astrocytic. The Gallyas stain was positive in oligodendroglial and negative in astrocytic lesions, as is typical of globular glial tauopathy. There were no TAR DNA-binding protein 43–positive inclusions. A, Temporal white matter (hematoxylin-eosin; original magnification ×200; inset ×750); B, temporal white matter (phospho-tau [AT8]; original magnification ×200); C, temporal gray matter (phospho-tau [AT8]; original magnification ×200; inset: 4R tau ×400); D, temporal white matter (Gallyas; original magnification ×200; inset ×750); E, temporal white matter (4R tau; original magnification ×200); and F, temporal gray matter (Gallyas; original magnification ×200).

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