Nonfluent Aphasia Research Articles

Dysphasia: metastatic prostate cancer to the leptomeninges: a case report

BackgroundLeptomeningeal metastasis occurs in 5% of patients with prostate cancer and indicates a very poor prognosis.Case presentationA 60-year-old Caucasian male patient diagnosed with metastatic castration-resistant prostate cancer with sclerotic bone metastases and soft tissue metastases underwent multiple courses of chemotherapy and hormone therapy. The diagnosis of prostate cancer is based on elevated prostate-specific antigen levels and tissue biopsy. He subsequently presented with expressive aphasia. Nonspecific, diffuse irregular dural/pachymeningeal thickening enhancement was noted on magnetic resonance imaging. Upon evaluation by neurology, electroencephalogram was negative for an epileptiform correlate. The workup included a lumbar puncture to rule out infectious etiology. The patient’s neurological status stabilized, and he was discharged home with a plan for continued therapy with abiraterone and prednisone. Due to advanced malignancy, the patient enrolled in hospice and died 3 weeks after hospital discharge.ConclusionsCentral nervous system metastasis occurs very rarely in prostate cancer. With the increase in life expectancy and advances in oncologic therapy for prostate cancer, physicians should be aware of and consider central nervous system metastasis in men aged 50 years and above.

Prediction of Aphasia Outcome Using Low Frequency Transcranial Magnetic Stimulation in Post-Stroke Non-Fluent Aphasia

Background: Stroke, a global health concern, often leads to language impairment (aphasia). In Egypt, with a high stroke prevalence, aphasia affects a significant number of survivors. Noninvasive techniques like repetitive transcranial magnetic stimulation (rTMS) show promise in rehabilitation. Cultural variations in test results emphasize the need for region-specific considerations in standardized assessments for post-stroke care. Aim of the work: To evaluate the role of low frequency rTMS to speed up the recovery of post-stroke aphasia for a better quality of life. Patients and Methods: This randomized clinical trial investigates the effects of (rTMS) on post-stroke aphasia. Patients with post-stroke non-fluent aphasia were divided into two groups, with one receiving inhibitory rTMS and the other sham sessions. Inclusion criteria involve adults with a single left hemisphere stroke, while exclusion criteria include prior strokes. The study includes comprehensive assessments for aphasia, and depression. The stimulation protocol involves 20 minutes of daily rTMS for ten days, targeting the right inferior frontal gyrus. The study aims to evaluate the rTMS impact on language recovery. Results: The study evaluated low-frequency rTMS for post-stroke aphasia recovery in 30 patients, comparing TMS and sham groups. No significant demographic or cerebrovascular risk factor differences were found. While rTMS group showed improvement in depression, there were no significant differences between TMS and sham groups in aphasia improvement. Conclusion: Low-frequency rTMS demonstrated no significant superiority over sham stimulation in post-stroke aphasia recovery. Both groups exhibited improved depression without notable distinctions.

The clinical and genetic spectrum of paediatric speech and language disorders.

Speech and language disorders are known to have a substantial genetic contribution. Although frequently examined as components of other conditions, research on the genetic basis of linguistic differences as separate phenotypic subgroups has been limited so far. Here, we performed an in-depth characterization of speech and language disorders in 52 143 individuals, reconstructing clinical histories using a large-scale data-mining approach of the electronic medical records from an entire large paediatric healthcare network. The reported frequency of these disorders was the highest between 2 and 5 years old and spanned a spectrum of 26 broad speech and language diagnoses. We used natural language processing to assess the degree to which clinical diagnoses in full-text notes were reflected in ICD-10 diagnosis codes. We found that aphasia and speech apraxia could be retrieved easily through ICD-10 diagnosis codes, whereas stuttering as a speech phenotype was coded in only 12% of individuals through appropriate ICD-10 codes. We found significant comorbidity of speech and language disorders in neurodevelopmental conditions (30.31%) and, to a lesser degree, with epilepsies (6.07%) and movement disorders (2.05%). The most common genetic disorders retrievable in our analysis of electronic medical records were STXBP1 (n = 21), PTEN (n = 20) and CACNA1A (n = 18). When assessing associations of genetic diagnoses with specific linguistic phenotypes, we observed associations of STXBP1 and aphasia (P = 8.57 × 10-7, 95% confidence interval = 18.62-130.39) and MYO7A with speech and language development delay attributable to hearing loss (P = 1.24 × 10-5, 95% confidence interval = 17.46-infinity). Finally, in a sub-cohort of 726 individuals with whole-exome sequencing data, we identified an enrichment of rare variants in neuronal receptor pathways, in addition to associations of UQCRC1 and KIF17 with expressive aphasia, MROH8 and BCHE with poor speech, and USP37, SLC22A9 and UMODL1 with aphasia. In summary, our study outlines the landscape of paediatric speech and language disorders, confirming the phenotypic complexity of linguistic traits and novel genotype-phenotype associations. Subgroups of paediatric speech and language disorders differ significantly with respect to the composition of monogenic aetiologies.

Delayed cyst formation after radiosurgery for arteriovenous malformation: A case report and critical review

Background: Stereotactic radiosurgery (SRS) is a validated treatment option for cerebral arteriovenous malformations (AVMs), even if a greater knowledge of its potential delayed complications is still being acquired. Case Description: A 49-year-old man suffered multiple episodes of cerebral hemorrhage in an approximate 10-year follow-up interval in the context of a left central core AVM with deep venous drainage into the internal cerebral veins (Spetzler Martin Grade 4) despite being treated with gamma knife radiosurgery at two separate timepoints, and with an almost complete obliteration confirmed. Approximately 10 years after the first radiosurgery treatment, he developed severe motor aphasia, Grade 3 right hemiparesis, progressive confusion, and memory deficits. Cerebral imaging revealed cystic degeneration in the AVM’s periphery. Cyst fenestration and cystoperitoneal shunt were attempted. The treatments were temporarily effective, but a progressive cyst enlargement recurred with clinical deterioration. The patient was therefore proposed for surgical mass and cyst excision through an interhemispheric transcallosal approach. The postoperative magnetic resonance imaging showed complete removal of the lesion, and an uneventful post-operative course ensued. At the 6-month follow-up, our patient experienced a noticeable improvement in his speech, power, dexterity and was able to walk autonomously. Conclusion: Cystic degeneration of AVMs is a possible long-term complication after SRS. Long-term follow-up and data on such patients remain crucial, even with evidence of complete nidal obliteration.

MRI-Based Multi-Class Relevance Vector Machine Classification of Neurodegenerative Diseases.

Machine learning algorithms are a promising automated candidate that can help mitigate the growing need for dementia experts. Despite the substantial development in MRI-based machine learning analyses, case misclassification is a universal finding, yet the reasons behind misclassification are poorly understood. We implemented a multi-class classification approach that uses relevance vector machine and logistic classification to classify research participants based on their whole-brain T1-weighted MRI scans. A total of 468 participants from seven diagnostic classes were included: 144 healthy controls, 84 Alzheimer's disease, 108 behavioral variant frontotemporal dementia (bvFTD), 30 semantic variant primary progressive aphasia (svPPA), 30 non-fluent variant primary progressive aphasia (nfvPPA), 30 corticobasal syndrome (CBS), and 42 progressive supranuclear palsy syndrome (PSPS). We compared the algorithm's diagnostic accuracy against the clinical, pathological, genetic, and quantitative imaging data. The exact neurodegenerative syndrome was predicted in 71% of the cases, the neurodegenerative disease spectrum was predicted in 80% of the cases, and the algorithm distinguished controls from any dementia in 85% of the cases. The algorithm showed high performance in diagnosing healthy controls, moderate performance in diagnosing AD, bvFTD, and svPPA, and low performance in diagnosing CBS, nfvPPA, and PSPS. Based on the quantitative imaging data, most of the misclassified neurodegenerative cases had minimal atrophy and brain volumes comparable to healthy controls. In AD, early-onset AD cases with minimal brain atrophy represented most of the misclassified cases. In bvFTD, FTD genetic mutation carriers (predominantly C9orf72 repeat expansion), FTD phenocopy, patients meeting only possible bvFTD criteria represented most misclassified cases. Case misclassification in machine learning studies in neurodegenerative diseases results from neurodegenerative disease heterogeneity and the limitations of structural MRI's ability to capture the whole gamut of biological changes. Larger and more inclusive datasets that are representative of population biologic heterogeneity are needed to train better machine learning techniques, and a margin of error is expected and should be acceptable, like the uncertainty of a clinical diagnosis by a dementia expert.

Efficacy of Bihemispheric tDCS in Rehabilitation of Non-Fluent Aphasia: A Single-subject Pilot Study

Objective: Transcranial direct current stimulation (tDCS) has emerged as a potentially effective complementary tool in rehabilitation of aphasia. However, there is no consensus regarding the optimal tDCS montage to augment language outcomes in aphasia. Against this background, the present study aimed to examine efficacy of tDCS combined with language therapy in aphasia rehabilitation and to compare two different montages. Methods: A right-handed participant suffering from chronic, non-fluent aphasia following stroke affecting the left hemisphere underwent a 5-week procedure involving tDCS coupled with language therapy. The procedure comprised two 5-day treatments of bihemispheric tDCS (over inferior frontal and posterior temporal sites determined using the international 10-20 EEG system). As part of both treatments, the left hemispheric targets were excited through anodal tDCS while simultaneously inhibiting their right-hemispheric homologues through cathodal tDCS. Baseline, post-treatment and follow-up assessments were obtained using a comprehensive language assessment tool. Results: An increase in language outcomes, particularly in repetition, was observed following the treatments. It was also found that therapy gains were more robust following bihemispheric stimulation of the posterior temporal sites compared to the inferior frontal targets. Conclusion: Bihemispheric tDCS coupled with language therapy appears to be effective in remediating language symptoms, particularly in terms of the repetition ability, in aphasia.

Time reference in French-speaking people with fluent and non-fluent aphasia (part II): a cluster analysis

ABSTRACT Background Tense production is often impaired in people with aphasia (PWA). Interestingly, the literature suggests that not all tenses are affected in the same way, although the pattern of dissociation is still debated in the literature. The heterogeneity of tense production deficits in PWA might partly explain this lack of consensus in the literature. While this heterogeneity has been demonstrated by descriptive statistics or double dissociations in several studies, no study has used cluster analyses to highlight the main patterns of tense dissociation. Furthermore, the factors underlying this heterogeneity have been little explored. Aims Our study aimed to use cluster analyses on tense production performance to (1) identify the main patterns of dissociation between tenses in PWA and (2) explore the clinical (aphasia type and severity) and cognitive (executive disorders and temporality) factors that may underlie these patterns. Methods & procedures Twenty-one French-speaking participants with fluent and non-fluent aphasia completed a verb inflection production task, as well as language and cognitive tests. Cluster analyses were performed on the differences in performance between the tenses on the verb inflection production task. Generalized linear mixed models and nonparametric statistics were used to analyze the effect of tense and its interaction with the clusters, and to compare the clusters on the clinical and cognitive variables. Outcomes & results Cluster analyses revealed two main clusters, one with worse performances in the past and future than in the present (PWA_1 – 70% of the PWA), and the other with worse performances in the past and present than in the future (PWA_2 – 30%). The type and severity of aphasia, and cognitive test performance did not differ between the two clusters. On the other hand, the PWA_2 cluster had a longer time post-onset than the PWA_1 cluster. Conclusions Our results confirm the heterogeneity of verb inflection deficits in PWA. They suggest that most PWA would have difficulties with past and future tenses, questioning the presence of discourse linking in these two tenses. However, a minority of PWA, who are clinically and cognitively indistinguishable from the others, do not follow this pattern. Adopting the strategy of producing a morphologically simple tense in French – future – could explain the high performance in the future tense and the errors in substituting other tenses with the future in this subgroup. Finally, the clinical and research implications of this heterogeneity, in relation to therapies and the lack of consensus in the literature, are discussed.

Time reference in French-speaking people with fluent and non-fluent aphasia (part I): tense dissociations, task effects and cognitive predictors

ABSTRACT Background People with fluent and non-fluent aphasia frequently have time reference deficits, which could be more pronounced for the past than for the present and future tenses. However, this performance pattern (past < non-past) is not consensual and might depend on the type of task used. Furthermore, the influence of cognitive processes on time reference deficits remains little explored, as does the origin of these difficulties (e.g. pre-phonological, morpho-phonological). Aims The primary aims of the study were (1) to identify time reference deficits and (2) dissociations between tenses in French speakers with fluent and non-fluent aphasia and (3) to specify the effect of tasks on performance and tense dissociations. Secondly, our study also aimed to determine whether time reference deficits were (4) influenced by cognitive functions and (5) explained by pre-phonological or morpho-phonological deficits. Methods & procedures Twenty-one French-speaking participants with fluent and non-fluent aphasia and 21 matched control subjects performed three tasks assessing time reference (verb inflection production, verb inflection selection, and adverb selection) and five neuropsychological tests (verbal and non-verbal working memory, inhibition, flexibility, and temporality). Quantitative and qualitative analyses were conducted. Outcomes & results Participants with fluent and non-fluent aphasia had worse performances than controls on the three time-reference tasks. The performance of the participants with fluent and non-fluent aphasia was quantitatively similar but qualitatively different on the verb inflection production task (i.e. different error types). A main task effect was also found: the verb inflection production task was the only one to show worse performances in the past than in the present and future. Regarding cognitive predictors, verbal working memory and temporality were linked to the three tasks assessing time reference. In contrast, non-verbal working memory was associated only with the two selection tasks, possibly related to the mental timeline. Finally, task analysis suggested a pre-phonological (encoding of diacritic features deficit) rather than a morpho-phonological origin. Conclusions Our study is the first to replicate time reference deficit in French speakers with aphasia. It suggests that the type of task might influence the performance profiles observed and help to shed light on the origin of the difficulties. Cognitive functions that have rarely been investigated before may also influence performance. These results have important clinical implications by questioning the tasks used to assess time reference. They also underline the importance of identifying the origin of difficulties in proposing targeted treatment.

Sentence comprehension in Malay-speaking adults with aphasia: the role of affix integration

ABSTRACT Background One of the impairments in comprehension mostly reported for aphasia is that in reversible passive sentences, where a systematic asymmetry with reversible active sentences has been attested across languages. In several accounts, this selective impairment has been proposed to be the result of the specific syntax of passive sentences. However, some results particularly from flexible word order languages suggest that a pattern where both active and passive sentences are impaired also exists. Aims In this study, we examined the actives and passives in the comprehension of a flexible word order language, namely standard Malay, in people with aphasia (PWA), aiming to confirm that the predominant pattern in this language is one of generalised impairment across reversible sentences (active and passive), and not an asymmetrical one. The role of fluency in determining the pattern of impairment was also explored. Methods & procedures Fourteen healthy adults and 20 PWA, 14 with fluent aphasia and 6 with non-fluent aphasia, took part in a comprehension study in standard Malay. Standard Malay is a flexible word order language that relies on the parsing of the voice affix on the verb to correctly interpret both active and passive sentences. Participants were tested on a sentence-picture matching task on comprehension of active and passive reversible clauses. Outcomes and results The study revealed that PWA were overall less accurate than healthy speakers in comprehension of reversible active and passive sentences, and non-fluent PWA were less accurate than fluent PWA with no effect of sentence type (active/passive). While not predominant, an asymmetric pattern was present in some participants. We propose that an impairment in thematic role assignment leads to a generalised impairment in comprehension of reversible sentences, while preserved thematic role mapping and impaired syntax lead to an asymmetric impairment. Conclusions The study confirmed that speakers of Malay mostly show a generalised impairment in both active and passive sentences. Given the nature of the language, we propose that this suggests that impairment in Malay is predominantly shown on affix integration, first in mapping of thematic roles, and then in the full syntactic processing.

Expanding the scope: multimodal dimensions in aphasia discourse analysis-preliminary findings.

Aphasia, resulting from acquired brain injury, disrupts language processing and usage, significantly impacting individuals' social communication and life participation. Given the limitations of traditional assessments in capturing the nuanced challenges faced by individuals with aphasia, this study seeks to explore the potential benefits of integrating multimodal communication elements into discourse analysis to better capture narrative proficiency in this population. This study examined how incorporating multimodal communication elements (e.g., physical gestures, writing, drawing) into discourse analysis may affect the narrative outcomes of persons with aphasia compared to those observed using methods that exclude multimodal considerations. Participants included individuals with chronic aphasia and age-and education-matched healthy controls who completed a storytelling task-the Bear and the Fly story. Macrolinguistic scores were obtained using verbal-only and multimodal scoring approaches. Additionally, the frequency and type of multimodal communication use during storytelling were examined in relation to aphasia characteristics. Statistical analyses included both within-group and between-group comparisons as well as correlational analyses. Individuals with aphasia scored significantly higher in terms of their macrolinguistic abilities when multimodal scoring was considered compared to verbal-only scoring. Within the aphasia group, there were prominent differences noted in macrolinguistic scores for both fluent and nonfluent aphasia. Specifically, both groups scored higher on Main Concepts when multimodal scoring was considered, with the nonfluent group demonstrating significantly higher Main Concept and total macrolinguistic rubric scores in multimodal scoring compared to verbal scoring on the storytelling task. Additionally, aphasia severity showed moderate positive correlations with total macrolinguistic scores, indicating that individuals with less severe aphasia tended to produce higher quality narratives. Lastly, although persons with aphasia used different types of nonverbal modalities (i.e., drawing, writing), the use of meaning-laden gestures was most predominant during storytelling, emphasizing the importance of multimodal elements in communication for individuals with aphasia. Our preliminary study findings underscore the importance of considering multimodal communication in assessing discourse performance among individuals with aphasia. Tailoring assessment approaches based on aphasia subtypes can provide valuable insights into linguistic abilities and inform targeted intervention strategies for improving communication outcomes.

Unusual patterns of codeswitching in an unbalanced bilingual person with aphasia: effects of language and executive functions impairment

ABSTRACT Background Bilingual individuals with aphasia may exhibit unique patterns of language impairment and recovery. While code-switching (CS) is a common feature of bilingual speech, pathological CS following brain damage can provide insights into the neural mechanisms controlling bilingual language processing. Aims This study aimed to examine language impairment and recovery patterns in IZ, an unbalanced Hebrew-English bilingual with non-fluent aphasia, focusing on the unusual CS behavior observed during his subacute phase of recovery. Methods and procedures IZ underwent language and narrative assessments in both languages during the subacute and chronic phases of recovery. Ten cognitive tests were administered to assess various domains of cognitive function. Language performance was evaluated through naming and syntactic processing tasks in L1-Hebrew and L2-English. The frequency and type of CS were analyzed using the matrix language frame model. Outcomes and results During the subacute phase, IZ displayed significant language deficits in both L1-Hebrew and L2-English, with more severe impairment in L1-Hebrew naming and L2-English syntactic processing. He exhibited frequent involuntary CS, primarily using L1-Hebrew as the matrix language while embedding L2-English content words. Cognitive testing revealed reduced performance across multiple domains. In the chronic phase, IZ showed parallel improvements in both languages, particularly in L1-Hebrew naming and L2-English syntactic processing, along with a marked reduction in CS frequency. Conclusions IZ’s case demonstrates a double dissociation between lexical and syntactic processing across L1 and L2. The results suggest that matrix language selection in bilinguals may be governed by distinct neural mechanisms for each language, with specific brain regions responsible for activating lexical representations. Damage to these regions may lead to pathological CS, as observed in this case. This study underscores the importance of understanding bilingual language control mechanisms in aphasia rehabilitation.

Self-Improved Language Production in Nonfluent Aphasia Through Automated Recursive Self-Feedback

Self-Improved Language Production in Nonfluent Aphasia Through Automated Recursive Self-Feedback

A systematic review of melodic intonation therapy used by speech therapists on speech recovery for patients with non-fluent aphasia

Background: Many studies have reported positive results regarding the benefits of melodic intonation therapy (MIT) in patients with non-fluent aphasia. Currently, there is no specific inclusion of speech therapists (STs) in MIT research. Investigating effective speech therapy (ST) techniques to address the language functions hindered by non-fluent aphasia could yield evidence for aphasia rehabilitation research. Objective: This systematic review (SR) examines the effectiveness of the traditional MIT protocol used by STs on speech recovery for patients with non-fluent aphasia after stroke. It also discusses other characteristics of the traditional MIT, such as the participants, the MIT protocol applied, the therapy intensity, and the role of STs. Materials and methods: This SR followed the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) 2020 statement. The four computerized databases (PubMed, Embase, ICTRP, and Google Scholar) were searched in February 2024 to review all empirical findings. We also conducted a hand search in relevant journals. The search yielded 538 studies, of which 2 met the criteria and underwent review. The methodological quality of the included studies was evaluated using the Cochrane Collaboration’s tool for assessing the risk of bias. Furthermore, the protocol was registered in PROSPERO under the reference CRD42024508733. Results: This review included 2 randomized controlled trials (RCTs) involving 44 patients. We found evidence that MIT significantly improved speech recovery, precise language repetition, and functional communication in patients with non-fluent aphasia. STs were interventionists in MIT research and used MIT following the American manual, and they had previously received MIT training. Conclusion: Our review provides some evidence of the effectiveness of MIT on speech recovery in patients with non-fluent aphasia after stroke. MIT may be a practical alternative to standard ST. There is some indication that MIT requires music therapy (MT) skills and training; therefore, STs must also have these abilities.

Language and executive functions in patients with transcortical motor aphasia and Broca’s aphasia

ABSTRACT This study investigated language and executive functions (EF) in people with transcortical motor aphasia (TMA) and Broca’s aphasia (BA). Participants included 19 patients with TMA, 19 patients with BA, and 25 healthy controls. Verbal Fluency tests, Stroop tests and Trail-Making tests were administered to all participants, and the Boston Diagnostic Aphasia Examination (BDAE) was administered to participants with aphasia. Results showed that (1) both groups of patients with aphasia had poorer performance on Verbal Fluency tests, Stroop tests and Trail-Making tests than healthy controls; (2) participants with BA had superior performance on Stroop tests and Trail-Making tests, but not on Verbal Fluency tests, than participants with TMA, and (2) the performance on Verbal Fluency tests, Stroop tests and Trail-Making was significantly correlated with the performance on BDAE for participants with TMA, but not for participants with BA. These results suggest that EF deficits are present in both patients with TMA and those with BA. They also show that the relationship between EF deficits and language impairments in people with aphasia might depend on the type of aphasia, aspects of language, and the components of EF measured.

Dynamic degree centrality in stroke-induced Broca's aphasia varies based on first language: A functional MRI study.

This study sought to explore dynamic degree centrality (DC) variability in particular regions of the brain in patients with poststroke Broca aphasia (BA) using a resting-state functional magnetic resonance imaging (rs-fMRI) approach, comparing differences between Uyghur and Chinese BA patients. This study investigated two factors, language and BA status, and divided patients into four groups: Uyghur aphasia patients (UA), Uyghur normal control subjects (UN), Chinese aphasia patients (CA), and Chinese normal subjects (CN) who underwent rs-fMRI analysis. Two-way analysis of variance (ANOVA) was used to calculate the comprehensive differences in dynamic DC among these four groups. Correlations between DC and language behavior were assessed with partial correlation analyses. Two-way ANOVA revealed comparable results for the results of pairwise comparisons of dynamic DC variability among the four groups in the right middle frontal gyrus/orbital part (ORBmid.R), right superior frontal gyrus/dorsolateral, and right precuneus (PCUN.R), with results as follows: UA<UN, CA>CN, UA<CA, and UN>CN (p<.05, with the exception of the p-values for UA and UN in superior frontal gyrus/dorsolateral). In contrast, the opposite results were observed for the right calcarine fissure and surrounding cortex (CAL.R, p<.05). The observed enhancement of dynamic DC variability in ORBmid.R and PCUN.R among Chinese BA patients and in CAL.R in Uyghur BA patients may be attributable to language network restructuring. Overall, these results suggest that BA patients who use different language families may exhibit differences in the network mechanisms that characterize observed impairments of language function.

A case of MERS presenting with acute motor aphasia and tetraparesis and literature review

BackgroundMild encephalopathy wıth reversible splenial lesions (MERS) are a clinical–radiological diagnosis that can be diagnosed with radiological imaging and can be accompanied by impaired consciousness, aphasia, headache, and paralysis. MERS can be divided into two different types based on radiological findings; Involvement of the splenium is seen in type 1, and involvement of the splenium and deep white matter is seen in type 2. MERS Type 2 is very rare in adults. Here, we describe a patient diagnosed with MERS Type 2 and our therapeutic intervention.Case presentationA 22-year-old woman presented with slurred speech, weakness in the extremities and headache that started 14 h ago. The patient had sore throat and fever of 37.6 °C for 2 days, and medical history or family history were unremarkable. Neurological examination revealed that she was conscious, cooperative, with no signs of meningeal irritation. Speech comprehension, naming, and repetition were preserved, motor examination revealed 5/5 left upper extremity strength, 4/5 right upper extremity strength, and 2/5 bilateral lower extremity strength. Diffusion-weighted magnetic resonance imaging (MRI-DWI) revealed bilateral deep white matter and splenium symmetrical diffusion restriction. Blood tests showed high C-reactive protein (CRP) and no leukocytosis. No cells were detected in the cerebrospinal fluid (CSF), protein was 151 mg/dl, glucose was within normal limits. The patient, who developed quadriparesis during follow-up, was started on pulse dose steroids. Neurological examination improved to patient’s baseline at the 12th hour of treatment. In the 72nd hour control MRI-DWI, it was seen that the lesions had regressed. Culture/Polymerase chain reaction (PCR) for bacterial/viral infection agents came back negative. After steroids were given for 5 days, patient was discharged at her baseline.ConclusionsMERS is a rare diagnosis in adults and can be radiologically confused with acute toxic leukoencephalopathy. It can be distinguished clinically by the absence of prior infection, fever, and toxic agent exposure. Although there is no consensus in the literature regarding the treatment, the clinical picture improved rapidly after steroid in our case. Despite being rare, it should be considered in the differential diagnosis of patients with deep white matter lesions in adults.

Association of cortical morphology, white matter hyperintensity, and glymphatic function in frontotemporal dementia variants.

Frontotemporal dementia (FTD) can be phenotypically divided into behavioral variant FTD (bvFTD), nonfluent variant primary progressive aphasia (nfvPPA), and semantic variant PPA (svPPA). However, the neural underpinnings of this phenotypic heterogeneity remain elusive. Cortical morphology, white matter hyperintensities (WMH), diffusion tensor image analysis along the perivascular space (DTI-ALPS), and their interrelationships were assessed in subtypes of FTD. Neuroimaging-transcriptional analyses on the regional cortical morphological deviances among subtypes were also performed. Changes in cortical thickness, surface area, gyrification, WMH, and DTI-ALPS were subtype-specific in FTD. The three morphologic indices are related to whole-brain WMH volume and cognitive performance, while cortical thickness is related to DTI-ALPS. Neuroimaging-transcriptional analyses identified key biological pathways linked to the formation and/or spread of TDP-43/tau pathologies. We found subtype-specific changes in cortical morphology, WMH, and glymphatic function in FTD. Our findings have the potential to contribute to the development of personalized predictions and treatment strategies for this disorder. Cortical morphologic changes, white matter hyperintensities (WMH), and glymphatic dysfunction are subtype-specific. Cortical morphologic changes, WMH, and glymphatic dysfunction are inter-correlated. Cortical morphologic changes and WMH burden contribute to cognitive impairments.

Reversible cerebral vasoconstriction syndrome post-cardiac transplantation: a therapeutic dilemma: case report

BackgroundReversible cerebral vasoconstriction syndrome (RCVS) is characterized by diffuse, multifocal segmental narrowing of cerebral arteries and can result in ischaemic stroke. Causal factors, identified in 60% of cases, include immunosuppressant pharmacotherapy. The few reports following heart transplantation are almost all in Asian recipients. We report on a Caucasian Australian patient with immunotherapy induced RCVS post heart transplantation to highlight the state of knowledge of the condition and the treatment dilemma it poses.Case presentationA 51-year-old female underwent orthotopic heart transplantation at our institution. Induction immunotherapy comprised basiliximab, mycophenolate mofetil and methylprednisolone. On day 6 post-transplantation the patient was transitioned to oral prednisolone and tacrolimus. On day 7 the patient began to experience bilateral, severe, transient occipital and temporal headaches. On day 9 tacrolimus dose was up-titrated. A non-contrast computed tomography brain (CTB) was normal. Endomyocardial biopsy on day 12 demonstrated moderate Acute Cellular Rejection (ACR), which was treated with intravenous methylprednisolone. That evening the patient experienced a 15-minute episode of expressive dysphasia. The following morning she became confused, aphasic, and demonstrated right sided neglect and right hemianopia. A CT cerebral perfusion scan demonstrated hypoperfusion in the left middle cerebral artery (MCA) territory and cerebral angiography revealed widespread, focal multi-segmental narrowing of the anterior and posterior circulations. A diagnosis of RCVS was made, and nimodipine was commenced. As both steroids and tacrolimus are potential triggers of RCVS, cyclosporin replaced tacrolimus and methylprednisolone dose was reduced. A further CTB demonstrated a large left MCA territory infarct with left M2 MCA occlusion. The patient made steady neurological improvement. She was discharged 34 days post-transplantation with mild residual right lower limb weakness and persistent visual field defect on verapamil, cyclosporine, everolimus, mycophenolate mofetil and prednisolone.ConclusionReversible cerebral vasoconstriction syndrome is rare after orthotopic heart transplantation. Until now, RCVS has been almost exclusively described in Asian recipients, and is typically caused by immunotherapy. The condition may lead to permanent neurological deficits, and in the absence of definitive treatments, early recognition and imaging based diagnosis is essential to provide the opportunity to remove the causal agent(s). Co-existent ACR, can pose unique treatment difficulties.

Topic maintenance in non-fluent aphasia conversation

Background: Studies on communication partner training programs for persons with aphasia reveal improvements in the conversation partners’ communication skills. This article explores the strategies for topic maintenance developed by the conversational partners of three people with non-fluent aphasia before and after an intervention on conversational strategies. Method: We used conversation analysis to examine video-recorded samples of everyday conversations (pre- and post-treatment and follow-up) between three couples. The assessment for topic management was performed with Conversation Analysis Profile for People with Aphasia (CAPPA). The intervention was conducted over a period of five weeks. The partners were trained to use strategies for better topic maintenance (multiple-choice, multimodality, and message verification). At six weeks post-therapy, a third recording was made. Results: After the intervention, changes in topic maintenance were observed. The use of strategies is related to the pre-existing context and the presence of visual support. Discussion/Conclusion: Individuals with non-fluent aphasia and their conversation partners engage in multimodal conversations and benefit from the use of strategies to overcome barriers to understanding, leading them to maintain a conversational topic.

Cerebrospinal Fluid Total and Phosphorylated Tau Protein in Behavioral Variant Frontotemporal Dementia, Progressive Supranuclear Palsy, Corticobasal Syndrome and Non-Fluent Agrammatic Primary Progressive Aphasia: A Systematic Review and Meta-Analysis.

(1) Background: Frontotemporal lobar degeneration (FTLD) is a generic term which refers to multiple pathologies, including FTLD-tau. The most common FTLD-tau diseases are Pick's disease (PiD), progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). These diseases share four major syndromes: behavioral variant frontotemporal dementia (bvFD), Richardson syndrome (RS), corticobasal syndrome (CBS) and non-fluent agrammatic primary progressive aphasia (nfa-PPA). The primary aim of this meta-analysis was to examine the diagnostic performance of CSF total (t-tau) and phosphorylated (p-tau) protein in bvFTD, RS, CBS, nfa-PPA and pathologically or genetically defined tauopathy. (2) Methods: A systematic review and meta-analysis was performed on all studies with >10 subjects in a bvFTD/RS/CBS/nfa-PPA group and control group and available data on CSF t-tau or p-tau (mean, SD). Cohen's d was used to quantify the effect size of each study (3) Results: The PSP/tauopathy patients exhibited decreased levels of CSF p-tau compared to the control subjects. The CBS/bvFTD/nfa-PPA cohorts exhibited an increase in t-tau compared to the control groups. (4) Conclusions: Tauopathies may exhibit an inherent decrease in CSF p-tau. The admixture of AD patients in FTD cohorts and high heterogeneity among studies on rare diseases are significant confounding factors in FTLD studies.