scholarly journals Anterior temporal laterality in primary progressive aphasia shifts to the right

2005 ◽  
Vol 58 (3) ◽  
pp. 362-370 ◽  
Author(s):  
Mathieu Vandenbulcke ◽  
Ronald Peeters ◽  
Paul Van Hecke ◽  
Rik Vandenberghe
Keyword(s):  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
The Right

scholarly journals Asymmetric TDP pathology in primary progressive aphasia with right hemisphere language dominance

Neurology ◽  
2018 ◽  
Vol 90 (5) ◽  
pp. e396-e403 ◽  
Author(s):  
Garam Kim ◽  
Shahrooz Vahedi ◽  
Tamar Gefen ◽  
Sandra Weintraub ◽  
Eileen H. Bigio ◽  
...  
Keyword(s):  
Right Hemisphere ◽  
Primary Progressive Aphasia ◽  
Cortical Atrophy ◽  
Progressive Aphasia ◽  
Language Dominance ◽  
Primary Progressive ◽  
Activated Microglia ◽  
Unbiased Stereology ◽  
Cortical Regions ◽  
The Right

ObjectiveTo quantitatively examine the regional densities and hemispheric distribution of the 43-kDa transactive response DNA-binding protein (TDP-43) inclusions, neurons, and activated microglia in a left-handed patient with right hemisphere language dominance and logopenic-variant primary progressive aphasia (PPA).MethodsPhosphorylated TDP-43 inclusions, neurons, and activated microglia were visualized with immunohistochemical and histologic methods. Markers were quantified bilaterally with unbiased stereology in language- and memory-related cortical regions.ResultsClinical MRI indicated cortical atrophy in the right hemisphere, mostly in the temporal lobe. Significantly higher densities of TDP-43 inclusions were present in right language-related temporal regions compared to the left or to other right hemisphere regions. The memory-related entorhinal cortex (ERC) and language regions without significant atrophy showed no asymmetry. Activated microglia displayed extensive asymmetry (R > L). A substantial density of neurons remained in all areas and showed no hemispheric asymmetry. However, perikaryal size was significantly smaller in the right hemisphere across all regions except the ERC. To demonstrate the specificity of this finding, sizes of residual neurons were measured in a right-handed case with PPA and were found to be smaller in the language-dominant left hemisphere.ConclusionsThe distribution of TDP-43 inclusions and microglial activation in right temporal language regions showed concordance with anatomic distribution of cortical atrophy and clinical presentation. The results revealed no direct relationship between density of TDP-43 inclusions and activated microglia. Reduced size of the remaining neurons is likely to contribute to cortical atrophy detected by MRI. These findings support the conclusion that there is no obligatory relationship between logopenic PPA and Alzheimer pathology.

scholarly journals Neuronavigated Repetitive Transcranial Magnetic Stimulation as Novel Mapping Technique Provides Insights Into Language Function in Primary Progressive Aphasia

2021 ◽  
Author(s):  
Felix Mueller-Sarnowski ◽  
Nico Sollmann ◽  
Axel Schröder ◽  
Leen Houri ◽  
Sebastian Ille ◽  
...  
Keyword(s):  
Transcranial Magnetic Stimulation ◽  
Magnetic Stimulation ◽  
Right Hemisphere ◽  
Repetitive Transcranial Magnetic Stimulation ◽  
Primary Progressive Aphasia ◽  
Language Function ◽  
Language Mapping ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
The Right

Abstract Neuronavigated repetitive transcranial magnetic stimulation (nrTMS) is an innovative technique that provides insight into language function with high accuracy in time and space. So far, nrTMS has mainly been applied in presurgical language mapping of patients with cranial neoplasms. For the present study nrTMS was used for language mapping in primary progressive aphasia Seven patients (median age: 70 years, 4 males) with the non-fluent variant of primary progressive aphasia were included in this pilot study. Inhibitory nrTMS trains (5 Hz, 40 % resting motor threshold) caused virtual lesions at 46 standardized cortical stimulation targets per hemisphere. Patients’ errors in a naming task during stimulation were counted. The majority of errors induced occurred during frontal lobe stimulation (34.3 %). Timing errors and non-responses were most frequent. More errors were induced in the right hemisphere (58%) than in the left hemisphere (42%). Mapping was tolerated by all patients, however, discomfort or pain was reported for stimulation of frontal areas. The elevated right-hemispheric error rate in our study supports the hypothesis of a partial shift of language function to the right hemisphere in neurodegenerative aphasia during the course of disease and therefore points to the existence of significant neuronal plasticity in primary progressive aphasia. While this is an interesting finding for neurodegenerative disorders per se, its promotion might also harbor future therapeutic targets.

scholarly journals The Right Temporal Variant of Frontotemporal Dementia is Not Genetically Sporadic: A Case Series

2021 ◽  
pp. 1-7
Author(s):  
Hulya Ulugut Erkoyun ◽  
Sven J. van der Lee ◽  
Bas Nijmeijer ◽  
Rosalina van Spaendonk ◽  
Anne Nelissen ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Genetic Variant ◽  
Autosomal Dominant ◽  
Case Series ◽  
Primary Progressive Aphasia ◽  
Clinical Syndrome ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Memory Clinics ◽  
The Right

Background: Right temporal variant frontotemporal dementia (rtvFTD) has been generally considered as a right sided variant of semantic variant primary progressive aphasia (svPPA), which is a genetically sporadic disorder. Recently, we have shown that rtvFTD has a unique clinical syndrome compared to svPPA and behavioral variant frontotemporal dementia. Objective: We challenge the assumption that rtvFTD is a sporadic, non-familial variant of FTD by identifying potential autosomal dominant inheritance and related genes in rtvFTD. Methods: We collected all subjects with a diagnosis of FTD or primary progressive aphasia who had undergone genetic screening (n = 284) and subsequently who had a genetic variant (n = 48) with a diagnosis of rtvFTD (n = 6) in 2 specialized memory clinics. Results: Genetic variants in FTD related genes were found in 33% of genetically screened rtvFTD cases; including MAPT (n = 4), GRN (n = 1), and TARDBP (n = 1) genes, whereas only one svPPA case had a genetic variant in our combined cohorts. Additionally, 4 out of 6 rtvFTD subjects had a strong family history for dementia. Conclusion: Our results demonstrate that rtvFTD, unlike svPPA, is not a pure sporadic, but a heterogeneous potential genetic variant of FTD, and screening for genetic causes for FTD should be performed in patients with rtvFTD.

scholarly journals Sleep talking and primary progressive aphasia: case study and autopsy findings in a patient with logopenic primary progressive aphasia and dementia with Lewy bodies

2019 ◽  
Vol 12 (5) ◽  
pp. e228938 ◽  
Author(s):  
Alexandra Clemans Apple ◽  
Qinwen Mao ◽  
Eileen Bigio ◽  
Borna Bonakdarpour
Keyword(s):  
Right Hemisphere ◽  
Lewy Bodies ◽  
Primary Progressive Aphasia ◽  
Language Abilities ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Speech Output ◽  
Sleep Talking ◽  
The Right

This case study highlights the parasomnia behaviours of an individual with primary progressive aphasia, a type of dementia known for decline in language abilities. Despite a paucity of speech during the day, this individual had concurrent sleep talking at night; a combination which, to our knowledge, has never been reported before. Post-mortem pathology confirmed clinical suspicion of both Alzheimer and Lewy body diseases, both asymmetric to the left side. Given this rare left-sided asymmetrical pathology, we hypothesise that the relatively preserved right hemisphere may have allowed for access to intact overlearned phrases which usually originate from the right hemisphere to appear while asleep. A second hypothesis is also presented which postulates that bottom-up processing may have overridden top-down apathy during sleep and allowed for speech output in this case.

scholarly journals Neuronavigated repetitive transcranial magnetic stimulation as novel mapping technique provides insights into language function in primary progressive aphasia

2021 ◽  
Author(s):  
Felix Mueller-Sarnowski ◽  
Nico Sollmann ◽  
Axel Schröder ◽  
Leen Houri ◽  
Sebastian Ille ◽  
...  
Keyword(s):  
Transcranial Magnetic Stimulation ◽  
Magnetic Stimulation ◽  
Right Hemisphere ◽  
Repetitive Transcranial Magnetic Stimulation ◽  
Primary Progressive Aphasia ◽  
Language Function ◽  
Language Mapping ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
The Right

AbstractNavigated repetitive transcranial magnetic stimulation (nrTMS) is an innovative technique that provides insight into language function with high accuracy in time and space. So far, nrTMS has mainly been applied in presurgical language mapping of patients with intracranial neoplasms. For the present study, nrTMS was used for language mapping in primary progressive aphasia (PPA). Seven patients (median age: 70 years, 4 males) with the non-fluent variant of PPA (nfvPPA) were included in this pilot study. Trains of nrTMS (5 Hz, 100% resting motor threshold) caused virtual lesions at 46 standardized cortical stimulation targets per hemisphere. Patients’ errors in a naming task during stimulation were counted. The majority of errors induced occurred during frontal lobe stimulation (34.3%). Timing errors and non-responses were most frequent. More errors were induced in the right hemisphere (58%) than in the left hemisphere (42%). Mapping was tolerated by all patients, however, discomfort or pain was reported for stimulation of frontal areas. The elevated right-hemispheric error rate in our study could be due to a partial shift of language function to the right hemisphere in neurodegenerative aphasia during the course of disease and therefore points to the existence of neuronal plasticity in nfvPPA. While this is an interesting finding for neurodegenerative disorders per se, its promotion might also harbor future therapeutic targets.

scholarly journals A Dextral Primary Progressive Aphasia Patient with Right Dominant Hypometabolism and Tau Accumulation and Left Dominant Amyloid Accumulation

2016 ◽  
Vol 8 (1) ◽  
pp. 78-86 ◽  
Author(s):  
Young Kyoung Jang ◽  
Seongbeom Park ◽  
Hee Jin Kim ◽  
Hanna Cho ◽  
Chul Hyoung Lyoo ◽  
...  
Keyword(s):  
Left Hemisphere ◽  
Right Hemisphere ◽  
Brain Magnetic Resonance Imaging ◽  
Primary Progressive Aphasia ◽  
Clinical Findings ◽  
Severe Form ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Amyloid Accumulation ◽  
The Right

Background: Primary progressive aphasia (PPA) is a degenerative disease that presents as progressive decline of language ability with preservation of other cognitive functions in the early stages. Three subtypes of PPA are known: progressive nonfluent aphasia, semantic dementia, and logopenic aphasia (LPA). Patients and Methods: We report the case of a 77-year-old patient with PPA whose clinical findings did not correspond to the three subtypes but mainly fit LPA. Unlike other LPA patients, however, this patient showed a right hemisphere predominant glucose hypometabolism and tau accumulation and a left hemisphere predominant amyloid deposition. The right-handed patient presented with comprehension difficulty followed by problems naming familiar objects. This isolated language problem had deteriorated rapidly for 2 years, followed by memory difficulties and impairment of daily activities. Using a Korean version of the Western Aphasia Battery, aphasia was consistent with a severe form of Wernicke's aphasia. According to the brain magnetic resonance imaging and 18F-fludeoxyglucose positron emission tomography results, right hemisphere atrophy and hypometabolism, more predominant on the right hemisphere than the left, were apparent despite the fact that Edinburgh Handedness Questionnaire scores indicated strong right-handedness. On Pittsburgh compound B-PET, amyloid accumulation was asymmetrical with the left hemisphere being more predominant than the right, whereas 18F-T807-PET showed a right dominant tau accumulation. Conclusions: This is the first report of atypical PPA, in which the patient exhibited crossed aphasia and asymmetrical amyloid accumulation.

scholarly journals Right Hemisphere Involvement in Non-Fluent Primary Progressive Aphasia

2007 ◽  
Vol 18 (4) ◽  
pp. 239-243 ◽  
Author(s):  
Claudia Repetto ◽  
Rosa Manenti ◽  
Maria Cotelli ◽  
Marco Calabria ◽  
Orazio Zanetti ◽  
...  
Keyword(s):  
Right Hemisphere ◽  
Primary Progressive Aphasia ◽  
Point Of View ◽  
Unexpected Finding ◽  
Cognitive Domains ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Deficit ◽  
Crossed Aphasia ◽  
The Right

We described a 56-years-old man with a diagnosis of “non-fluent primary progressive aphasia” (NfPPA). An accurate neuropsychological, neurological and neuroimaging evaluation was performed in order to assess clinical and behavioural features of the patient.From a neuropsychological point of view, the patient showed a typical cognitive profile of subjects affected by NfPPA: a prominent language deficit, associated with impairments in several cognitive domains after three years from the onset of the symptomatology.The most intriguing feature is that SPECT revealed hypoperfusion in the right frontal cortex, albeit the patient is right-handed.This unexpected finding shows that NfPPA may arise not only from cortical abnormalities in the language-dominant left hemisphere, but also from right hemisphere involvement in a right hander (crossed aphasia).

Prominent hypometabolism of the right temporoparietal and frontal cortex in two left-handed patients with primary progressive aphasia

2002 ◽  
Vol 249 (9) ◽  
pp. 1263-1267 ◽  
Author(s):  
Alexander Drzezga ◽  
Timo Grimmer ◽  
Hartwig Siebner ◽  
Satoshi Minoshima ◽  
Markus Schwaiger ◽  
...  
Keyword(s):  
Frontal Cortex ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Left Handed ◽  
The Right

scholarly journals A clinical-radiological framework of the right temporal variant of frontotemporal dementia

Brain ◽  
2020 ◽  
Vol 143 (9) ◽  
pp. 2831-2843 ◽  
Author(s):  
Hulya Ulugut Erkoyun ◽  
Colin Groot ◽  
Ronja Heilbron ◽  
Anne Nelissen ◽  
Jonathan van Rossum ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Diagnostic Criteria ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Behavioural Changes ◽  
The Right ◽  
Mental Slowness

Abstract The concept of the right temporal variant of frontotemporal dementia (rtvFTD) is still equivocal. The syndrome accompanying predominant right anterior temporal atrophy has previously been described as memory loss, prosopagnosia, getting lost and behavioural changes. Accurate detection is challenging, as the clinical syndrome might be confused with either behavioural variant FTD (bvFTD) or Alzheimer’s disease. Furthermore, based on neuroimaging features, the syndrome has been considered a right-sided variant of semantic variant primary progressive aphasia (svPPA). Therefore, we aimed to demarcate the clinical and neuropsychological characteristics of rtvFTD versus svPPA, bvFTD and Alzheimer’s disease. Moreover, we aimed to compare its neuroimaging profile against svPPA, which is associated with predominant left anterior temporal atrophy. Of 619 subjects with a clinical diagnosis of frontotemporal dementia or primary progressive aphasia, we included 70 subjects with a negative amyloid status in whom predominant right temporal lobar atrophy was identified based on blinded visual assessment of their initial brain MRI scans. Clinical symptoms were assessed retrospectively and compared with age- and sex-matched patients with svPPA (n = 70), bvFTD (n = 70) and Alzheimer’s disease (n = 70). Prosopagnosia, episodic memory impairment and behavioural changes such as disinhibition, apathy, compulsiveness and loss of empathy were the most common initial symptoms, whereas during the disease course, patients developed language problems such as word-finding difficulties and anomia. Distinctive symptoms of rtvFTD compared to the other groups included depression, somatic complaints, and motor/mental slowness. Aside from right temporal atrophy, the imaging pattern showed volume loss of the right ventral frontal area and the left temporal lobe, which represented a close mirror image of svPPA. Atrophy of the bilateral temporal poles and the fusiform gyrus were associated with prosopagnosia in rtvFTD. Our results highlight that rtvFTD has a unique clinical presentation. Since current diagnostic criteria do not cover specific symptoms of the rtvFTD, we propose a diagnostic tree to be used to define diagnostic criteria and call for an international validation.

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