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 ◽  
...  

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).

Neurology ◽  
2018 ◽  
Vol 90 (5) ◽  
pp. e396-e403 ◽  
Author(s):  
Garam Kim ◽  
Shahrooz Vahedi ◽  
Tamar Gefen ◽  
Sandra Weintraub ◽  
Eileen H. Bigio ◽  
...  

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.


2021 ◽  
Author(s):  
Felix Mueller-Sarnowski ◽  
Nico Sollmann ◽  
Axel Schröder ◽  
Leen Houri ◽  
Sebastian Ille ◽  
...  

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.


2019 ◽  
Vol 12 (5) ◽  
pp. e228938 ◽  
Author(s):  
Alexandra Clemans Apple ◽  
Qinwen Mao ◽  
Eileen Bigio ◽  
Borna Bonakdarpour

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.


Author(s):  
Felix Mueller-Sarnowski ◽  
Nico Sollmann ◽  
Axel Schröder ◽  
Leen Houri ◽  
Sebastian Ille ◽  
...  

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.


2016 ◽  
Vol 8 (1) ◽  
pp. 78-86 ◽  
Author(s):  
Young Kyoung Jang ◽  
Seongbeom Park ◽  
Hee Jin Kim ◽  
Hanna Cho ◽  
Chul Hyoung Lyoo ◽  
...  

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.


2011 ◽  
Vol 69 (3) ◽  
pp. 446-451 ◽  
Author(s):  
Fábio Pascotto de Oliveira ◽  
Jaderson Costa da Costa ◽  
Sabine Possa Marroni ◽  
Ana Maria Marques da Silva ◽  
Sthefanie Hansen Barreiro ◽  
...  

There are individuals who have a progressive language deficit without presenting cognitive deficits in other areas. One of the diseases related to this presentation is primary progressive aphasia (PPA). OBJECTIVE: Identify by means of diffusion tensor imaging (DTI) and measurements of cortical volume, brain areas that lead to dysphasia when presenting signs of impaired connectivity or reduced volume. METHOD: Four patients with PPA were evaluated using DTI, and measurements of cortical volumes in temporal areas. These patients were compared with two normal volunteers. RESULTS: There is a trend to a difference in the number and volume of related fibers between control group and patients with PPA. Comparing cortical volumes in temporal areas between groups yielded a trend to a smaller volume in PPA patients. CONCLUSION: Patients with PPA have a trend to impairment in cortical and subcortical levels regarding relevant areas.


2006 ◽  
Vol 17 (2) ◽  
pp. 77-87 ◽  
Author(s):  
Serena Amici ◽  
Maria Luisa Gorno-Tempini ◽  
Jennifer M. Ogar ◽  
Nina F. Dronkers ◽  
Bruce L. Miller

We present a review of the literature on Primary Progressive Aphasia (PPA) together with the analysis of neuropschychological and neuroradiologic profiles of 42 PPA patients. Mesulam originally defined PPA as a progressive degenerative disorder characterized by isolated language impairment for at least two years. The most common variants of PPA are: (1) Progressive nonfluent aphasia (PNFA), (2) semantic dementia (SD), (3) logopenic progressive aphasia (LPA). PNFA is characterized by labored speech, agrammatism in production, and/or comprehension. In some cases the syndrome begins with isolated deficits in speech. SD patients typically present with loss of word and object meaning and surface dyslexia. LPA patients have word-finding difficulties, syntactically simple but accurate language output and impaired sentence comprehension. The neuropsychological data demonstrated that SD patients show the most characteristic pattern of impairment, while PNFA and LPA overlap within many cognitive domains. The neuroimaging analysis showed left perisylvian region involvement. A comprehensive cognitive, neuroimaging and pathological approach is necessary to identify the clinical and pathogenetic features of different PPA variants.


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

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