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Neurology. Genetics Apr 2024Pathogenic variants in are associated with a spectrum of epilepsy-aphasia syndromes (EASs). Seizures as well as speech and language disorders occur frequently but vary...
BACKGROUND AND OBJECTIVES
Pathogenic variants in are associated with a spectrum of epilepsy-aphasia syndromes (EASs). Seizures as well as speech and language disorders occur frequently but vary widely in severity, both between individuals and across the life span. The link between this phenotypic spectrum and brain characteristics is unknown. Specifically, altered brain networks at the root of speech and language deficits remain to be identified. Patients with pathogenic variants in offer an opportunity to interrogate the impact of glutamate receptor dysfunction on brain development.
METHODS
We characterized brain anomalies in individuals with pathogenic variants and EASs, hypothesizing alterations in perisylvian speech-language regions and the striatum. We compared structural MRI data from 10 individuals (3 children and 7 adults, 3 female) with pathogenic variants with data from age-matched controls (N = 51 and N = 203 in a secondary analysis). We examined cortical thickness and volume in 4 a priori hypothesized speech and language regions (inferior frontal, precentral, supramarginal, and superior temporal) and across the whole brain. Subcortical structures (hippocampus, basal ganglia, thalamus) and the corpus callosum were also compared.
RESULTS
Individuals with pathogenic variants showed increased thickness and volume in the posterior part of Broca's area (inferior frontal gyrus, pars opercularis). For thickness, the effects were bilateral but more pronounced in the left (large effect size, η = 0.37) than the right (η = 0.12) hemisphere. Both volume and thickness were also higher in the bilateral superior temporal region while the supramarginal region showed increased thickness only. Whole-brain analyses confirmed left-sided thickness increases in Broca's area, with additional increases in the occipital and superior frontal cortices bilaterally. Hippocampal volume was reduced in the left hemisphere. There were no age-dependent effects or corpus callosum group differences.
DISCUSSION
Anomalies in perisylvian regions, with largest differences in Broca's area, suggest an altered development of classical speech-language networks in -related EAS. Left hippocampal reduction suggests a role for this structure in early speech and language development and is consistent with gene expression in that region. Overall, elucidating the neural correlates of EAS provides insights into the impact of dysfunction, opening avenues for targeted intervention in developmental syndromes with compromised speech-language development.
PubMed: 38715655
DOI: 10.1212/NXG.0000000000200129 -
Frontiers in Neurology 2023Loss of time is a major obstacle to efficient stroke treatment. Our telestroke path intends to optimize prehospital triage using a video link connecting ambulance...
Real-time video analysis allows the identification of large vessel occlusion in patients with suspected stroke: feasibility trial of a "telestroke" pathway in Northwestern Switzerland.
BACKGROUND AND AIM
Loss of time is a major obstacle to efficient stroke treatment. Our telestroke path intends to optimize prehospital triage using a video link connecting ambulance personnel and a stroke physician. The objectives were as follows: (1) To identify patients suffering a stroke and (2) in particular large vessel occlusion (LVO) strokes as candidates for endovascular treatment. We have chosen the Rapid Arterial Occlusion Evaluation (RACE) scale for this purpose.
METHODS
This analysis aimed to verify the feasibility of prehospital stroke identification by video assessment. In this prospective telestroke cohort study, we included 97 subjects, in which the RACE score (items: facial palsy, arm and leg motor function, head and gaze deviation, and aphasia or agnosia) was applied, and the assessment videotaped by a trained member of the Emergency Medical Services (EMS) in the field using a mobile device. Each recorded patient video was independently assessed by three experienced stroke physicians from a certified stroke center and compared to the neuroimaging gold standard. Within this feasibility study, the stroke code was not altered by the outcome of the RACE assessment, and all patients underwent the standard procedures within the emergency unit.
RESULTS
We analyzed 97 patients (median age 78 years, 53% women), of whom 51 (52.6%) suffered an acute stroke, 12 (23.5%) of which were due to an LVO and 46 patients had symptoms mimicking a stroke. The sensitivity of stroke identification was 77.8%, and specificity was 53.6%. In regard to the identification of an LVO, sensitivity was 69.4% and specificity was 84.3%. The inter-rater agreement in the RACE-score assessment was ICC = 0.82 (intraclass-correlation coefficient).
CONCLUSION
These results confirm our hypothesis that the local telestroke concept is feasible. It allows correct (i) stroke and (ii) LVO identification in the majority of the cases and thus has the potential to assist in efficient prehospital triage.
PubMed: 37941577
DOI: 10.3389/fneur.2023.1232401 -
Alternative Therapies in Health and... Sep 2023It is estimated that 25% of the patients in Pakistan experience stroke resulting in problems with language. Among many of the conditions, problem with verbal expressive... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
It is estimated that 25% of the patients in Pakistan experience stroke resulting in problems with language. Among many of the conditions, problem with verbal expressive production (Broca's Aphasia) is one of the main problem faced by people having stoke. Many traditional therapies are incorporated to treat symptoms of Aphasia including fluent and non- fluent Aphasia.
OBJECTIVES
The primary objective of the current study was to determine the effectiveness of Verbal Expressive Skill Management Program in Urdu (VESMP-U) with convention speech therapy, Melodic Intonation therapy (MIT) in enhancing the verbal expressive skills in patients with severe Broca's Aphasia. Another objective of this study was to compare the efficacy of Verbal Expressive Skill Management Program in Urdu (VESMP-U) with traditional therapy, as well as the quality of life of patients with severe Broca's Aphasia.
METHODS
A randomized control trial (NCT03699605, clinicaltrials.gov) was conducted from November 2018 - June 2019 in Pakistan railway Hospital (PRH). Patients having a three-month history of severe Broca's Aphasia, aged between 40-60 years, bilingual (Urdu and English language) and having the ability to use a smart phone were included in the study. Patients with cognitive impairments were excluded. Total of 77 patients were evaluated for eligibility criteria according to the G Power software for sample size. Out of 77, 54 individuals fulfilled the inclusion criteria. The participants were divided into 2 groups (27 each) through sealed envelope method. Patients of both groups were assessed pre and post intervention using the Boston Diagnostic Aphasia Examination (BADE) battery (Primary outcome measure). Experimental group n = 25 received VESMP-U therapy and control group n = 25 (2 drop out in each group) received MIT for 16 weeks i.e. 4 days per week having 64 sessions altogether. Each intervention session lasted up to 30-45 minutes for both groups.
RESULTS
Within and between group analysis after intervention showed that the VESMP-U group had significantly improved BDAE scores (P = .001; 95% CI) than the MIT group for all variables (articulatory intelligibility, phrase length, grammatical form, prosody/intonation, spontaneous speech, word finding, repetition, and auditory comprehension). The BDAE scores of participants in experimental group having VESMP-U therapy pre- and post-intervention were statistically significant (P = .001; 95% CI), which indicates that participant's communication skills were enhanced by use of VESMP-U.
CONCLUSION
Android based application VESMP-U has been found to be effective in improving expression and quality of life of patients with severe Broca's aphasia.
Topics: Adult; Humans; Middle Aged; Aphasia, Broca; Asian People; Pakistan; Quality of Life; Stroke
PubMed: 37295011
DOI: No ID Found -
Neurology. Genetics Apr 2024To introduce the first case in which primary progressive apraxia of speech (PPAOS) is associated with TAR DNA-binding protein 43 (TDP-43) instead of 4-repeat tau.
OBJECTIVES
To introduce the first case in which primary progressive apraxia of speech (PPAOS) is associated with TAR DNA-binding protein 43 (TDP-43) instead of 4-repeat tau.
METHODS
This patient was identified through a postmortem autopsy. Following an initial diagnostic evaluation, he participated in 3 annual research visits during which speech, language, cognitive, and neurologic assessments were administered. Neuroimaging was also acquired.
RESULTS
Apraxia of speech was diagnosed at his initial visit with a comprehensive neurologic examination further revealing subtle motor findings in the right hand. At subsequent visits, agrammatic aphasia and motor symptoms consistent with corticobasal syndrome were evident. Cognition and behavior remained relatively intact until advanced stages. FDG-PET revealed hypometabolism in the right temporoparietal cortex and left premotor and motor cortices. There was also low-level signal in the right temporoparietal cortex on tau-PET. A sequence variation in the progranulin gene was identified (GRN c.1A>C, p.Met1). Pathologic diagnosis was TDP-43 Type A with an atypical distribution of inclusions in premotor and motor cortices.
DISCUSSION
This case report demonstrates that TDP-43 Type A inclusions in an atypical distribution can present clinically as PPAOS. The sequence variation in the progranulin gene and asymmetric temporoparietal cortex involvement were the strongest indications of the unusual neuropathophysiology prior to autopsy.
PubMed: 38515991
DOI: 10.1212/NXG.0000000000200134 -
Biomedicines Jun 2024The cerebellum is emerging as a promising target for noninvasive brain stimulation (NIBS). A systematic review was conducted to evaluate the effects of cerebellar NIBS... (Review)
Review
The cerebellum is emerging as a promising target for noninvasive brain stimulation (NIBS). A systematic review was conducted to evaluate the effects of cerebellar NIBS on both motor and other symptoms in stroke rehabilitation, its impact on functional ability, and potential side effects (PROSPERO number: CRD42022365697). A systematic electronic database search was performed by using PubMed Central (PMC), EMBASE, and Web of Science, with a cutoff date of November 2023. Data extracted included study details, NIBS methodology, outcome measures, and results. The risk of bias in eligible studies was also assessed. Twenty-two clinical studies involving 1016 participants were finally included, with a focus on outcomes related to post-stroke motor recovery (gait and balance, muscle spasticity, and upper limb dexterity) and other functions (dysphagia and aphasia). Positive effects were observed, especially on motor functions like gait and balance. Some efficiency was also observed in dysphagia rehabilitation. However, findings on language recovery were preliminary and inconsistent. A slight improvement in functional ability was noted, with no serious adverse effects reported. Further studies are needed to explore the effects of cerebellar NIBS on post-stroke non-motor deficits and to understand how cerebellar engagement can facilitate more precise treatment strategies for stroke rehabilitation.
PubMed: 38927555
DOI: 10.3390/biomedicines12061348 -
BMC Geriatrics Dec 2023Corticobasal syndrome (CBS) is a neurodegenerative disease diagnosed based on clinical manifestations such as asymmetrical parkinsonism, limb apraxia, and speech and...
BACKGROUND
Corticobasal syndrome (CBS) is a neurodegenerative disease diagnosed based on clinical manifestations such as asymmetrical parkinsonism, limb apraxia, and speech and language impairment. The background pathology of CBS is commonly a variety of proteinopathies, but association with cerebrovascular disease has also been reported. Foix-Chavany-Marie syndrome (FCMS) is a rare neurological disorder characterized by facio-pharyngo-glossal diplegia with automatic-voluntary movement dissociation presenting with bilateral paresis of the facial, lingual, pharyngeal and masticatory muscles. FCMS is commonly attributable to stroke. Transactive response DNA binding protein of 43 kD (TDP-43) proteinopathy is also known as the pathological background of FCMS, while the pathological background of the majority of CBS cases consists of diverse tauopathies instead of TDP-43 proteinopathy. In this report, we describe a case mimicking FCMS that was finally diagnosed as CBS with suggested 4-repeat tauopathy.
CASE PRESENTATION
A 68-year-old female started experiencing difficulty speaking followed by difficulty writing, and especially texting, several years before her visit. Her impairment had been gradually worsening, and she came to our hospital. On neurological examination, she demonstrated the facial apraxia, frontal lobe dysfunction, and upper motor neuron signs. She presented some characteristics suggestive of FCMS. Her symptoms exhibited rapid progression and myoclonus, parkinsonism, and left-side dominant cortical sensory deficit occurred, resulting in the fulfillment of diagnostic criteria for CBS after 9 months. Tau PET imaging displayed notable ligand uptake in the brainstem, subthalamic nuclei, basal ganglia, and bilateral subcortical frontal lobe, suggesting that her pathological background was 4-repeat tauopathy. As a result of her progressive dysphagia, she became unable to eat and passed away after 12 months.
CONCLUSION
We hereby present an atypical case of CBS showing clinical features mimicking FCMS at first presentation. TDP-43 proteinopathy was suspected based on the clinical symptoms in the early stages of the disease; however, the clinical course and imaging findings including tau PET suggested that her pathological background was 4-repeat tauopathy.
Topics: Female; Humans; Aged; Deglutition Disorders; Corticobasal Degeneration; Neurodegenerative Diseases; Syndrome; Apraxias; Parkinsonian Disorders; TDP-43 Proteinopathies
PubMed: 38087192
DOI: 10.1186/s12877-023-04564-z -
Communications Biology Jun 2024Thalamic aphasia results from focal thalamic lesions that cause dysfunction of remote but functionally connected cortical areas due to language network perturbation....
Thalamic aphasia results from focal thalamic lesions that cause dysfunction of remote but functionally connected cortical areas due to language network perturbation. However, specific local and network-level neural substrates of thalamic aphasia remain incompletely understood. Using lesion symptom mapping, we demonstrate that lesions in the left ventrolateral and ventral anterior thalamic nucleus are most strongly associated with aphasia in general and with impaired semantic and phonemic fluency and complex comprehension in particular. Lesion network mapping (using a normative connectome based on fMRI data from 1000 healthy individuals) reveals a Thalamic aphasia network encompassing widespread left-hemispheric cerebral connections, with Broca's area showing the strongest associations, followed by the superior and middle frontal gyri, precentral and paracingulate gyri, and globus pallidus. Our results imply the critical involvement of the left ventrolateral and left ventral anterior thalamic nuclei in engaging left frontal cortical areas, especially Broca's area, during language processing.
Topics: Humans; Male; Middle Aged; Female; Ventral Thalamic Nuclei; Aphasia; Magnetic Resonance Imaging; Stroke; Thalamus; Aged; Adult; Connectome; Frontal Lobe; Nerve Net; Neural Pathways
PubMed: 38849518
DOI: 10.1038/s42003-024-06399-9 -
Frontiers in Neurology 2024Post-stroke aphasia (PSA) is one of the most devastating symptoms after stroke, yet limited treatment options are available. Prolonged intermittent theta burst...
BACKGROUND
Post-stroke aphasia (PSA) is one of the most devastating symptoms after stroke, yet limited treatment options are available. Prolonged intermittent theta burst stimulation (piTBS) is a promising therapy for PSA. However, its efficacy remains unclear. Therefore, we aim to investigate the efficacy of piTBS over the left supplementary motor area (SMA) in improving language function for PSA patients and further explore the mechanism of language recovery.
METHODS
This is a randomized, double-blinded, sham-controlled trial. A total of 30 PSA patients will be randomly allocated to receive either piTBS stimulation or sham stimulation for 15 sessions over a period of 3 weeks. The primary outcome is the Western Aphasia Battery Revised (WAB-R) changes after treatment. The secondary outcomes include The Stroke and Aphasia Quality of Life Scale (SAQOL-39 g), resting-state electroencephalogram (resting-state EEG), Event-related potentials (ERP), brain derived neurotrophic factor (BDNF). These outcome measures are assessed before treatment, after treatment, and at 4-weeks follow up. This study was registered in Chinese Clinical Trial Registry (No. ChiCTR23000203238).
DISCUSSION
This study protocol is promising for improving language in PSA patients. Resting-state EEG, ERP, and blood examination can be used to explore the neural mechanisms of PSA treatment with piTBS.
CLINICAL TRIAL REGISTRATION
https://www.chictr.org.cn/index.html, ChiCTR2300074533.
PubMed: 38725649
DOI: 10.3389/fneur.2024.1348862 -
Journal of Neuroengineering and... Jan 2024Analysis of tongue movement would benefit from a reference showcasing healthy tongue capability. We aimed to develop a reference of tongue capability and evaluated the...
BACKGROUND
Analysis of tongue movement would benefit from a reference showcasing healthy tongue capability. We aimed to develop a reference of tongue capability and evaluated the role of visual feedback on the expression of movement.
METHODS
Using a wireless tracking intraoral wearable device, we composed probability distributions of the tongue tip as subjects were asked to explore the entire sensing surface area. Half of the 32 subjects received live visual feedback of the location of the center of the tongue tip contact.
RESULTS
We observed that the visual feedback group was 51.0% more consistent with each other in the position domain, explored 21.5% more sensing surface area, and was 50.7% more uniformly distributed. We found less consistent results when we evaluated velocity and acceleration.
CONCLUSION
Visual feedback best established a healthy capability reference which can be used for designing new interfaces, quantifying tongue ability, developing new diagnostic and rehabilitation techniques, and studying underlying mechanisms of tongue motor control.
Topics: Humans; Feedback, Sensory; Tongue; Movement; Feedback
PubMed: 38166962
DOI: 10.1186/s12984-023-01293-7 -
Medicine May 2024Although several rehabilitation interventions are effective in post-stroke aphasia (PSA), the efficacy of different rehabilitation interventions compared to each other... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Although several rehabilitation interventions are effective in post-stroke aphasia (PSA), the efficacy of different rehabilitation interventions compared to each other remains controversial. Here, we aimed to compare the effectiveness of varying rehabilitation interventions in PSA.
METHODS
Randomized controlled trials on 8 kinds of rehabilitation interventions to improve speech function in patients with PSA were searched by computer from 10 databases, including PubMed, Web of Science, Cochrane, OVID, CINAHL, Embase, CNKI, WanFang, CBM, and VIP. The search scope was from the establishment of the database to August 2023. The literature screening, extraction of basic information, and quality assessment of the literature were conducted independently by 2 researchers. Network meta-analysis (NMA) was performed using Stata 17.0 software.
RESULTS
Fifty-four studies involving 2688 patients with PSA were included. The results of NMA showed that: ① in terms of improving the severity of aphasia, the therapeutic effects of repetitive transcranial magnetic stimulation were the most significant; ② motor imagery therapy was the most effective in improving spontaneous speech, repetition, and naming ability; ③ in terms of improving listening comprehension ability, the therapeutic effects of mirror neuron therapy was the most significant.
CONCLUSION
The 8 rehabilitation interventions have different focuses in improving the speech function of PSA patients, and the clinical therapists can select the optimal rehabilitation interventions in a targeted manner according to the results of this NMA and the patients' conditions and other relevant factors.
Topics: Humans; Aphasia; Stroke Rehabilitation; Network Meta-Analysis; Randomized Controlled Trials as Topic; Stroke; Treatment Outcome; Transcranial Magnetic Stimulation
PubMed: 38787993
DOI: 10.1097/MD.0000000000038255