Globular Glial Tauopathy Type I Presenting as Atypical Progressive Aphasia, With Comorbid Limbic-Predominant Age-Related TDP-43 Encephalopathy

Rusina, Robert; Cséfalvay, Zsolt; Kovács, Gábor G.; Keller, Jiří; Javůrková, Alena; Matěj, Radoslav

doi:10.3389/fnagi.2019.00336

Cited by 8 publications

(10 citation statements)

References 18 publications

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“…The first case (previously published) was a 69‐year‐old man with a combination of two PPA subtypes (i.e., early nonfluent‐agrammatical and late semantic variant) and hippocampal amnesia [ 2 ]. He initially had mild anomia, without language comprehension impairment at the single word and sentence level.…”

Section: Methodsmentioning

confidence: 99%

“…

ME THODSThe first case (previously published) was a 69-year-old man with a combination of two PPA subtypes (i.e., early nonfluentagrammatical and late semantic variant) and hippocampal amnesia [2]. He initially had mild anomia, without language comprehension impairment at the single word and sentence level.

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confidence: 99%

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Corpus callosum hypersignals and focal atrophy: Neuroimaging findings in globular glial tauopathy type I

Keller

Kavkova

Matěj

et al. 2021

Euro J of Neurology

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Globular glial tauopathies (GGTs) have heterogeneous presentations [1]; little evidence regarding typical clinical and magnetic resonance imaging (MRI) presentations is available. We report MRI findings for three autopsy-confirmed GGT cases, two presenting clinically with atypical primary progressive aphasia (PPA) and one with corticobasal syndrome (CBS). ME THODSThe first case (previously published) was a 69-year-old man with a combination of two PPA subtypes (i.e., early nonfluentagrammatical and late semantic variant) and hippocampal amnesia [2]. He initially had mild anomia, without language comprehension impairment at the single word and sentence level. Progressively, he developed lexical retrieval problems, speech apraxia, and

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Section: Methodsmentioning

confidence: 99%

“…

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mentioning

confidence: 99%

Corpus callosum hypersignals and focal atrophy: Neuroimaging findings in globular glial tauopathy type I

Keller

Kavkova

Matěj

et al. 2021

Euro J of Neurology

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“…In addition, Parkinson-plus syndromes such as progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) as well as primary lateral sclerosis have been described particularly in GGT type II [1,4] whereas lower motor neuron disease [1] in combination with parkinsonism and dementia is associated with GGT type III. Comprehensive data remain scarce and there are only a handful of case reports and small series describing frontotemporal and occasionally parietal lobe atrophy on magnetic resonance imaging (MRI) [9][10][11][12][13]. To our knowledge there are only two cases in the literature showing frontotemporal hypometabolism on [ 18 F]-fluorodeoxyglucose-positron emission tomography (PET) [10,14] and a single case report describing changes on single-photon emission computed tomography (SPECT) [11] and molecular tau-PET in GGT [15].…”

Section: Introductionmentioning

confidence: 99%

The many faces of globular glial tauopathy: A clinical and imaging study

Buciuc

Koga

Pham

et al. 2022

Euro J of Neurology

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Background: Globular glial tauopathy (GGT) has been associated with frontotemporal dementia syndromes; little is known about the clinical and imaging characteristics of GGT and how they differ from other non-globular glial 4-repeat tauopathies (N4GT) such as progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD).Methods: For this case-control study the Mayo Clinic brain banks were queried for all cases with an autopsy-confirmed diagnosis of GGT between 1 January 2011 and 31 October 2021. Fifty patients with N4GT (30 PSP, 20 CBD) were prospectively recruited and followed by the Neurodegenerative Research Group at Mayo Clinic, Minnesota.Magnetic resonance imaging was used to characterize patterns of gray/white matter atrophy, MR-parkinsonism index, midbrain volume, and white matter hyperintensities. 18 F-Fluorodeoxyglucose-, 11 C Pittsburg compound-, and 18 F-flortaucipir-positron emission tomography scans were reviewed.Results: Twelve patients with GGT were identified: 83% were women compared to 42% in NG4T (p = 0.02) with median age at death 76.5 years (range: 55-87). The most frequent clinical features were eye movement abnormalities, parkinsonism, behavioral changes followed by pyramidal tract signs and motor speech abnormalities. Lower motor neuron involvement was present in 17% and distinguished GGT from NG4T (p = 0.035). Primary progressive apraxia of speech was the most frequent initial diagnosis (25%); 50% had a Parkinson-plus syndrome before death. Most GGT patients had asymmetric frontotemporal atrophy with matching hypometabolism. GGT patients had more gray matter atrophy in temporal lobes, normal MR-parkinsonism index, and larger midbrain volumes.Conclusions: Female sex, lower motor neuron involvement in the context of a frontotemporal dementia syndrome, and asymmetric brain atrophy with preserved midbrain might be suggestive of underlying GGT.

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“…Moreover, FLAIR hyperintensities, as well as cortical ischemic changes, were present as well (Fazekas 3); midbrain atrophy was also found. This case was previously published [ 9 , 42 ]; neuropathology revealed full-blown GGT type I combined with age-related neuropathology with deposits of TDP-43 protein, which met the diagnostic criteria for LATE.…”

Section: Case Descriptionmentioning

confidence: 88%

“…We hypothesize that in patients with a broader clinical presentation corresponding mainly to one of the archetypal PPA variants but also showing symptoms of another PPA variant, neurodegenerative comorbidities should be considered, as demonstrated by case 7, which was a combination of nfvPPA and svPPA attributable to fully developed GGT and LATE which were ultimately confirmed on autopsy [ 9 , 42 ]. A result of the clinical-neuropathological correlations in our cohort produced a very interesting finding, i.e., that in nfvPPA patients, ventral stream involvement was significantly linked to the ARTAG neuropathology.…”

Section: Discussionmentioning

confidence: 99%

Comorbid Neurodegeneration in Primary Progressive Aphasia: Clinicopathological Correlations in a Single-Center Study

Rusina

Bajtosova

Cséfalvay

et al. 2022

Behavioural Neurology

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Introduction. Primary progressive aphasia (PPA) is a clinically variable syndrome manifesting as slow progressive loss of speech and language with multiple underlying neurodegenerative pathologies. Materials and Methods. We included data from nine PPA patients with available autopsies. We then retrospectively reviewed all available medical records, neuropsychology, and MRI results to confirm the corresponding subtypes of PPA and compared them with postmortem neuropathological results. Results. Clinical presentations corresponded to the nonfluent/agrammatic variant in six cases, the semantic variant in one case, the logopenic variant in one case, and the mixed variant (concomitant nonfluent/agrammatic plus semantic variant) in one case. Patients with a broader clinical presentation, i.e., combining manifestations of one PPA subtype and symptoms of another PPA variant, had autopsy comorbidities showing multiple neurodegenerative disorders. Of the nine subjects enrolled in the study, Alzheimer’s disease (AD) was found in eight cases; however, in only one case, AD was detected as an isolated neuropathological substrate of PPA. In eight brain samples, different comorbid neuropathologies were detected: three cases with comorbid AD and dementia with Lewy bodies, two cases with comorbid AD and TDP-43 pathology, one case with comorbid AD and complex tauopathies, and one case with comorbid AD with both tau and TDP-43 deposits. Finally, one case had comorbid tau and TDP-43 pathology but without comorbid AD pathology. Conclusions. Our observation suggests that PPA cases could be more heterogeneous in their etiology than previously thought and underlying neurodegenerative comorbidities should be considered in routine practice, especially if the clinical presentation of PPA is atypical.

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Globular Glial Tauopathy Type I Presenting as Atypical Progressive Aphasia, With Comorbid Limbic-Predominant Age-Related TDP-43 Encephalopathy

Cited by 8 publications

References 18 publications

Corpus callosum hypersignals and focal atrophy: Neuroimaging findings in globular glial tauopathy type I

Corpus callosum hypersignals and focal atrophy: Neuroimaging findings in globular glial tauopathy type I

The many faces of globular glial tauopathy: A clinical and imaging study

Comorbid Neurodegeneration in Primary Progressive Aphasia: Clinicopathological Correlations in a Single-Center Study

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