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 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 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 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).

scholarly journals Verbal and Nonverbal Memory in Primary Progressive Aphasia: The Three Words-Three Shapes Test

2013 ◽  
Vol 26 (1-2) ◽  
pp. 67-76 ◽  
Author(s):  
Sandra Weintraub ◽  
Emily Rogalski ◽  
Emily Shaw ◽  
Sabrina Sawlani ◽  
Alfred Rademaker ◽  
...  
Keyword(s):  
Left Hemisphere ◽  
Test Performance ◽  
Memory Loss ◽  
Primary Progressive Aphasia ◽  
Signs And Symptoms ◽  
Published Data ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
On Line ◽  
Memory Network

Objectives:To investigate cognitive components and mechanisms of learning and memory in primary progressive aphasia (PPA) using a simple clinical measure, the Three Words Three Shapes Test (3W3S).Background:PPA patients can complain of memory loss and may perform poorly in standard tests of memory. The extent to which these signs and symptoms reflect dysfunction of the left hemisphere language versus limbic memory network remains unknown.Methods:3W3S data from 26 patients with a clinical diagnosis of PPA were compared with previously published data from patients with typical dementia of the Alzheimer type (DAT) and cognitively healthy elders.Results:PPA patients showed two bottlenecks in new learning. First, they were impaired in the effortless (but not effortful) on-line encoding of verbal (but not non-verbal) items. Second, they were impaired in the retrieval (but not retention) of verbal (but not non-verbal) items. In contrast, DAT patients had impairments also in effortful on-line encoding and retention of verbal and nonverbal items.Conclusions:PPA selectively interferes with spontaneous on-line encoding and subsequent retrieval of verbal information. This combination may underlie poor memory test performance and is likely to reflect the dysfunction of the left hemisphere language rather than medial temporal memory network.

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 Cortical cholinergic denervation in primary progressive aphasia with Alzheimer pathology

Neurology ◽  
2019 ◽  
Vol 92 (14) ◽  
pp. e1580-e1588 ◽  
Author(s):  
M.-Marsel Mesulam ◽  
Nava Lalehzari ◽  
Farzan Rahmani ◽  
Daniel Ohm ◽  
Ryan Shahidehpour ◽  
...  
Keyword(s):  
Detailed Analysis ◽  
Left Hemisphere ◽  
Basal Forebrain ◽  
Cholinergic Neurons ◽  
Primary Progressive Aphasia ◽  
Neurotrophin Receptor ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Cortical Areas ◽  
The Status

ObjectiveTo investigate the status of the basal forebrain cholinergic system in primary progressive aphasia (PPA) as justification for cholinergic therapy.MethodsA cohort of 36 brains from PPA participants with the neuropathology of Alzheimer disease (PPA-AD, n = 14) or frontotemporal lobar degeneration (PPA-tau, n = 12; PPA-TDP, n = 10) were used for semiquantitative rating of degeneration and gliosis of basal forebrain cholinergic neurons (BFCN). A subpopulation of 5 PPA-AD and 7 control brains underwent detailed analysis of BFCN pathology and cortical cholinergic axonal loss employing immunohistochemical and histochemical methods and stereologic analysis.ResultsSemiquantitatively, 11 (∼80%) PPA-AD participants were rated as having moderate/severe BFCN loss and gliosis, whereas none of the PPA-tau and only 1 (10%) PPA-TDP participant received such a rating. Detailed analysis in the subpopulation of PPA-AD participants revealed substantial tangle formation, loss of BFCN, and degeneration of cortical cholinergic axons. Compared to controls, loss of p75 low affinity neurotrophin receptor-positive BFCN was detected in the PPA-AD participants (p < 0.01). Acetylcholinesterase-positive cholinergic axons in all cortical areas studied displayed loss in PPA-AD (p < 0.005–0.0001). The loss was more severe in the language-dominant left hemisphere and, within the left hemisphere, in language-affiliated cortical areas.ConclusionsOur results demonstrate prominent depletion of BFCN and cortical cholinergic axons in PPA-AD when compared with normal control or other neuropathologic variants of PPA. The demonstration of cholinergic denervation with an anatomy that fits the clinical picture suggests that cholinergic treatment is justified in patients with PPA who have positive AD biomarkers.

scholarly journals THE CLINICAL CASE OF PRIMARY PROGRESSIVE APHASIA

2019 ◽  
Vol 26 (1) ◽  
pp. 61-67
Author(s):  
Natalya V. Shuleshova ◽  
Aleksey V. Sizov ◽  
Irina V. Kupriianova ◽  
Victoria V. Zvereva
Keyword(s):  
Clinical Case ◽  
Brain Magnetic Resonance Imaging ◽  
Primary Progressive Aphasia ◽  
Clinical Feature ◽  
Resonance Imaging ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Fast Progression ◽  
Jakob Disease ◽  
Sensory Functions

The article described the rare clinical case of the patient with probable transmissible encephalopathy (Creutzfeldt–Jakob disease) in whom rapidly forming primary progressive aphasia was the herald clinical feature, and motor and sensory functions were preserved. Creutzfeldt–Jakob disease was diagnosed, and it was confirmed by clinical picture with fast progression of the disease, together with neurovisualization (brain magnetic resonance imaging, FLAIR and DWI options) and electroencephalography pattern. Other possible causes of primary progressive aphasia were excluded.

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