Search

The purpose of this investigation was to explore the feasibility of fMRI in the study of developmental stuttering. Speech contrasts (loud versus silent reading) and language contrasts (reading of semantically meaningful text versus nonsense words) of six developmental stutterers and six nonstutterers were compared using a commercial 1 Tesla MR-Scanner (Siemens Expert). Results indicate that mapping cortical function in persons who stutter is indeed feasible, even with a 1TMR-system. Compared to normals the stutterers seemed to employ different and particularly less differentiated auditory and motor feedback strategies in speech. They apparently rely on auditory processing and on cerebellar contribution as much during silent reading as during reading aloud. Moreover, they showed a greater involvement of the right hemisphere in language processing, activating not only the typical language areas on the left but also and with equal magnitude the right side homologues of these areas. In spite of the promising results, at present several practical problems such as possible movement artifacts and possible masking through scanner noise still hamper a more straightforward use of fMRI in the study of developmental stuttering. [ABSTRACT FROM AUTHOR]

A 38-year-old right-handed male with no history of speech or language problems presented with neurogenic stuttering following an ischaemic lesion of the left thalamus. He stuttered severely in propositional speech (conversation, monologue, confrontation naming, and word retrieval) but only slightly in non-propositional speech (automatic speech, sound, word and sentence repetition, and reading aloud). It is suggested that thalamic stuttering may constitute a distinct clinical entity. [ABSTRACT FROM AUTHOR]

Background: Dysregulation of the gut microbiome has been implicated in Parkinson's disease (PD). This study aimed to evaluate the clinical effects and safety of a single faecal microbiota transplantation (FMT) in patients with early-stage PD., Methods: The GUT-PARFECT trial, a single-centre randomised, double-blind, placebo-controlled trial was conducted at Ghent University Hospital between December 01, 2020 and December 12, 2022. Participants (aged 50-65 years, Hoehn and Yahr stage 2) were randomly assigned to receive nasojejunal FMT with either healthy donor stool or their own stool. Computer-generated randomisation was done in a 1:1 ratio through permutated-block scheduling. Treatment allocation was concealed for participants and investigators. The primary outcome measure at 12 months was the change in the Movement Disorders Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS) motor score obtained during off-medication evaluations. Intention-to-treat analysis was performed using a mixed model for repeated measures analysis. This completed trial is registered on ClinicalTrials.gov (NCT03808389)., Findings: Between December 2020 and December 2021, FMT procedures were conducted on 46 patients with PD: 22 in the healthy donor group and 24 in the placebo group. Clinical evaluations were performed at baseline, 3, 6, and 12 months post-FMT. Full data analysis was possible for 21 participants in the healthy donor group and 22 in the placebo group. After 12 months, the MDS-UPDRS motor score significantly improved by a mean of 5.8 points (95% CI -11.4 to -0.2) in the healthy donor group and by 2.7 points (-8.3 to 2.9) in the placebo group (p = 0.0235). Adverse events were limited to temporary abdominal discomfort., Interpretation: Our findings suggested a single FMT induced mild, but long-lasting beneficial effects on motor symptoms in patients with early-stage PD. These findings highlight the potential of modulating the gut microbiome as a therapeutic approach and warrant a further exploration of FMT in larger cohorts of patients with PD in various disease stages., Funding: Flemish PD patient organizations (VPL and Parkili), Research Foundation Flanders (FWO), Biocodex Microbiota Foundation., Competing Interests: JR has received grants from Beneo, Cargill, Colruyt group, Danone, DSM, J&J, MRM/Prodigest, Nestle, Pfizer, and Takeda; and has received consulting and/or speaking fees from Aphea, Biofortis, DSM, Ferring, GSK, Janssen Pharmaceuticals, Metagenics, MSD, MRM/Prodigest, Nutricia, Sanofi, Takeda, Tsumura. RV has received grants from MRM Health, Prodigest, CellCarta, Evox Therapeutics and Sanofi. All other authors declare no competing interests., (© 2024 The Author(s).)

Background & Aims: The mismatch negativity (MMN) and P300 event-related potentials (ERPs) have been studied in relation to phoneme discrimination and categorization, respectively. Although the effects of aging and sex on pure-tone perception have been widely investigated using these ERPs, evidence relating to phoneme perception is scarce. The current study aimed to provide insight into the effects of aging and sex on phoneme discrimination and categorization, as measured through the MMN and P300., Method: An inattentive and attentive oddball paradigm containing a phonemic articulation place contrast were administered during EEG registration in sixty healthy individuals (thirty males and females), of which an equal number of young (20-39 years), middle-aged (40-59 years) and elderly (60+ years) subjects were included. The amplitude, onset latency and topographical distribution of the MMN and P300 effect, as well as the amplitude of the P1-N1-P2 complex, were analyzed for age group and sex differences., Results: With respect to aging, elderly subjects demonstrated a reduced MMN and P300 amplitude compared to the young group, whereas the scalp distribution of both components was unaffected. No aging effects on the P1-N1-P2 complex were found. In elderly individuals, the P300 was found to be delayed compared to the young group, while no such effect on MMN latency could be observed. No differences in MMN and P300 measures could be identified between males and females., Conclusion: Differential effects of aging were found on the MMN and P300, specifically in terms of latency, in relation to phoneme perception. In contrast, sex was found to scarcely affect both processes., Competing Interests: Declaration of competing interest None of the authors reported a conflict of interest., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Prior work on patients with Parkinson's disease (PD) has shown that the administration of dopaminergic medication in the early to intermediate stages of PD benefits (motor) functions associated with the dopamine-depleted dorsal striatal circuitry but may 'overdose' and interfere with (cognitive) functions associated with the relatively intact ventral striatal circuitry. The present study aimed to elucidate this so-called dopamine overdose hypothesis for the action control domain. Using a within-subject design in a sample of 13 people with PD, we evaluated the effect of dopaminergic medication on two cognitive processes underlying goal-directed behaviour, namely action selection and initiation through event binding and conflict adaptation. We also investigated whether individual differences in the magnitude of medication effects were associated across these processes. Results showed no indications that dopaminergic medication affects action selection and initiation or conflict adaptation in PD patients. Additionally, we observed no correlations between both cognitive processes nor between individual differences in medication effects. Our findings do not support the notion that dopaminergic medication modulates action control processes, suggesting that the dopamine overdose hypothesis may only apply to a specific set of cognitive processes and should potentially be refined., (© 2022 The Authors. Journal of Neuropsychology published by John Wiley & Sons Ltd on behalf of The British Psychological Society.)

Purpose: The clinical use of event-related potentials in patients with language disorders is increasingly acknowledged. For this purpose, normative data should be available. Within this context, healthy aging and gender effects on the electrophysiological correlates of semantic sentence comprehension were investigated., Method: One hundred and ten healthy subjects (55 men and 55 women), divided among three age groups (young, middle aged, and elderly), performed a semantic sentence congruity task in the visual modality during electroencephalographic recording., Results: The early visual complex was affected by increasing age as shown by smaller P2 amplitudes in the elderly compared to the young. Moreover, the N400 effect in the elderly was smaller than in the young and was delayed compared to latency measures in both middle-aged and young subjects. The topography of age-related amplitude changes of the N400 effect appeared to be gender specific. The late positive complex effect was increased at frontal electrode sites from middle age on, but this was not statistically significant. No gender effects were detected regarding the early P1, N1, and P2, or the late positive complex effect., Conclusion: Especially aging effects were found during semantic sentence comprehension, and this from the level of perceptual processing on. Normative data are now available for clinical use.

Background and Objectives: Owing to their extensive clinical and molecular heterogeneity, hereditary neurologic diseases in adults are difficult to diagnose. The current knowledge about the diagnostic yield and clinical utility of exome sequencing (ES) for neurologic diseases in adults is limited. This observational study assesses the diagnostic value of ES and multigene panel analysis in adult-onset neurologic disorders., Methods: From January 2019 through April 2022, ES-based multigene panel testing was conducted in 1,411 patients with molecularly unexplained neurologic phenotypes at the Ghent University Hospital. Gene panels were developed for ataxia and spasticity, leukoencephalopathy, movement disorders, paroxysmal episodic disorders, neurodegeneration with brain iron accumulation, progressive myoclonic epilepsy, and amyotrophic lateral sclerosis. Single nucleotide variants, small indels, and copy number variants were analyzed. Across all panels, our analysis covered a total of 725 genes associated with Mendelian inheritance., Results: A molecular diagnosis was established in 10% of the cases (144 of 1,411) representing 71 different monogenic disorders. The diagnostic yield depended significantly on the presenting phenotype with the highest yield seen in patients with ataxia or spastic paraparesis (19%). Most of the established diagnoses comprised disorders with an autosomal dominant inheritance (62%), and the most frequently mutated genes were NOTCH3 (13 patients), SPG7 (11 patients), and RFC1 (8 patients). 34% of the disease-causing variants were novel, including a unique likely pathogenic variant in APP (Ghent mutation, p.[Asn698Asp]) in a family presenting with stroke and severe cerebral white matter disease. 7% of the pathogenic variants comprised copy number variants detected in the ES data and confirmed by an independent technique., Discussion: ES and multigene panel testing is a powerful and efficient tool to diagnose patients with unexplained, adult-onset neurologic disorders., Competing Interests: The authors report no relevant disclosures. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/NG., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

Parkinson's disease (PD) has increasingly been associated with auditory dysfunction, including alterations regarding the control of auditory information processing. Although these alterations may interfere with the processing of speech in degraded listening conditions, behavioural studies have generally found preserved speech-in-noise recognition in PD. However, behavioural speech audiometry does not capture the neurophysiological mechanisms supporting speech-in-noise processing. Therefore, the aim of this study was to investigate the neural oscillatory mechanisms associated with speech-in-noise processing in PD. Twelve persons with PD and 12 age- and gender-matched healthy controls (HCs) were included in this study. Persons with PD were studied in the medication off condition. All subjects underwent an audiometric screening and performed a sentence-in-noise recognition task under simultaneous electroencephalography (EEG) recording. Behavioural speech recognition scores and self-reported ratings of effort, performance, and motivation were collected. Time-frequency analysis of EEG data revealed no significant difference between persons with PD and HCs regarding delta-theta (2-8 Hz) inter-trial phase coherence to noise and sentence onset. In contrast, significantly increased alpha (8-12 Hz) power was found in persons with PD compared with HCs during the sentence-in-noise recognition task. Behaviourally, persons with PD demonstrated significantly decreased speech recognition scores, whereas no significant differences were found regarding effort, performance, and motivation ratings. These results suggest that persons with PD allocate more cognitive resources to support speech-in-noise processing. The interpretation of this finding is discussed in the context of a top-down mediated compensation mechanism for inefficient filtering and degradation of auditory input in PD., (© 2021 Federation of European Neuroscience Societies and John Wiley & Sons Ltd.)

The exact etiology of Parkinson's disease (PD) remains largely unknown, but more and more research suggests the involvement of the gut microbiota. Interestingly, idiopathic PD patients were shown to have at least a 10 times higher prevalence of Helicobacter suis ( H. suis ) DNA in gastric biopsies compared to control patients. H. suis is a zoonotic Helicobacter species that naturally colonizes the stomach of pigs and non-human primates but can be transmitted to humans. Here, we investigated the influence of a gastric H. suis infection on PD disease progression through a 6-hydroxydopamine (6-OHDA) mouse model. Therefore, mice with either a short- or long-term H. suis infection were stereotactically injected with 6-OHDA in the left striatum and sampled one week later. Remarkably, a reduced loss of dopaminergic neurons was seen in the H. suis/ 6-OHDA groups compared to the control/6-OHDA groups. Correspondingly, motor function of the H. suis -infected 6-OHDA mice was superior to that in the non-infected 6-OHDA mice. Interestingly, we also observed higher expression levels of antioxidant genes in brain tissue from H. suis -infected 6-OHDA mice, as a potential explanation for the reduced 6-OHDA-induced cell loss. Our data support an unexpected neuroprotective effect of gastric H. suis on PD pathology, mediated through changes in oxidative stress.

Competing Interests: No specific funding was received for this work. The authors declare that there are no conflicts of interest relevant to this work.

Behavioral studies on auditory deviance detection in patients with Parkinson's disease (PD) have reported contradictory results. The primary aim of this study was to investigate auditory deviance detection of multiple auditory features in patients with PD by means of objective and reliable electroencephalographic (EEG) measurements. Twelve patients with early-stage PD and twelve age- and gender-matched healthy controls (HCs) were included in this study. Patients with PD participated without their regular dopaminergic medication. All subjects underwent an audiometric screening and performed a passive multi-feature mismatch negativity (MMN) paradigm. Repeated-measures analysis of variance (ANOVA) demonstrated no significant differences between patients with PD and HCs regarding MMN mean amplitude and latency for frequency, duration and gap deviants. Nevertheless, a trend towards increased MMN mean amplitude and latency was found in response to intensity deviants in patients with PD compared to HCs. Increased intensity MMN amplitude may indicate that more neural resources are allocated to the processing of intensity deviances in patients with PD compared to HCs. The interpretation of this intensity-specific MMN alteration is further discussed in the context of a compensatory mechanism for auditory intensity processing and involuntary attention switching in PD.

Purpose Several studies have demonstrated increased auditory thresholds in patients with Parkinson's disease (PD) based on subjective tonal audiometry. However, the pathophysiological mechanisms underlying auditory dysfunction in PD remain elusive. The primary aim of this study was to investigate cochlear and olivocochlear function in PD using objective measurements and to assess the effect of dopaminergic medication on auditory function. Method Eighteen patients with PD and 18 gender- and age-matched healthy controls (HCs) were included. Patients with PD participated in medication on and off conditions. Linear mixed models were used to determine the effect of PD on tonal audiometry, transient evoked and distortion product otoacoustic emissions (OAEs), and efferent suppression (ES). Results Tonal audiometry revealed normal auditory thresholds in patients with PD for their age across all frequencies. OAE signal amplitudes demonstrated a significant interaction effect between group (PD vs. HC) and frequency, indicating decreased OAEs at low frequencies and increased OAEs at high frequencies in patients with PD. No significant differences were found between patients with PD and HCs regarding ES. In addition, no significant effect of medication status was found on auditory measurements in patients with PD. Conclusions Altered OAEs support the hypothesis of cochlear alterations in PD. No evidence was found for the involvement of the medial olivocochlear system. Altogether, OAEs may provide an objective early indicator of auditory alterations in PD and should complement subjective tonal audiometry when assessing and monitoring auditory function in PD.

Hippocampal sclerosis (HS) is a common neuropathological finding and has been associated with advanced age, TDP-43 proteinopathy, and cerebrovascular pathology. We analyzed neuropathological data of an autopsy cohort of early-onset frontotemporal dementia patients. The study aimed to determine whether in this cohort HS was related to TDP-43 proteinopathy and whether additional factors could be identified. We examined the relationship between HS, proteinopathies in frontotemporal cortices and hippocampus, Alzheimer disease, cerebrovascular changes, and age. We confirmed a strong association between HS and hippocampal TDP-43, whereas there was a weaker association between HS and frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP). Nearly all of the FTLD-TDP cases had TDP-43 pathology in the hippocampus. HS was present in all FTLD-TDP type D cases, in 50% of the FTLD-TDP A cohort and in 6% of the FTLD-TDP B cohort. Our data also showed a significant association between HS and vascular changes. We reviewed the literature on HS and discuss possible pathophysiological mechanisms between TDP-43 pathology, cerebrovascular disease, and HS. Additionally, we introduced a quantitative neuronal cell count in CA1 to objectify the semiquantitative visual appreciation of HS., (© 2021 American Association of Neuropathologists, Inc. All rights reserved.)

There is accumulating evidence for auditory dysfunctions in patients with Parkinson's disease (PD). Moreover, a possible relationship has been suggested between altered auditory intensity processing and the hypophonic speech characteristics in PD. Nonetheless, further insight into the neurophysiological correlates of auditory intensity processing in patients with PD is needed primarily. In the present study, high-density EEG recordings were used to investigate intensity dependence of auditory evoked potentials (IDAEPs) in 14 patients with PD and 14 age- and gender-matched healthy control participants (HCs). Patients with PD were evaluated in both the on- and off-medication states. HCs were also evaluated twice. Significantly increased IDAEP of the N1/P2 was demonstrated in patients with PD evaluated in the on-medication state compared to HCs. Distinctive results were found for the N1 and P2 component. Regarding the N1 component, no differences in latency or amplitude were shown between patients with PD and HCs regardless of the medication state. In contrast, increased P2 amplitude was demonstrated in patients with PD evaluated in the on-medication state compared to the off-medication state and HCs. In addition to a dopaminergic deficiency, deficits in serotonergic neurotransmission in PD were shown based on increased IDAEP. Due to specific alterations of the N1-P2 complex, the current results suggest deficiencies in early-attentive inhibitory processing of auditory input in PD. This interpretation is consistent with the involvement of the basal ganglia and the role of dopaminergic and serotonergic neurotransmission in auditory gating.

Introduction: Task-specific dystonias are primary focal dystonias characterized by excessive muscle contractions producing abnormal postures during selective motor activities that often involve highly skilled, repetitive movements. Based on the idea of excessive motor excitability and aberrant sensorimotor integration in the pathophysiology of task-specific dystonia, sensorimotor retraining may hold promise. The purpose of this systematic review was to investigate the available evidence about the role of rehabilitation therapy as a treatment for task-specific dystonia., Evidence Acquisition: A systematic review was performed of studies identified through Pubmed and Embase in a structured search strategy by independent author screening. The JBI (Joanna Briggs Institute) Critical Appraisal Checklist and RoB 2 were used to evaluate their methodological quality., Evidence Synthesis: Twenty-one studies were included for qualitative synthesis. Most of the reports are small single group pre-/post-test study designs with a variability in the type of task-specific dystonia and the type of evaluated outcome measures. Rehabilitation interventions were grouped into six categories based upon the underlying theoretical basis of different approaches: 1) movement practice; 2) training with constraint; 3) sensory reorganization; 4) biofeedback training; 5) neuromodulation with training; and 6) compensatory strategies., Conclusions: Although it appears that a number of task-specific dystonia patients may improve with rehabilitation therapy, no definitive conclusions can be drawn. More research in this field is needed, using standardized approaches and clearly defined outcome measures in larger cohorts of task-specific dystonia patients that are clinically and diagnostically well characterized.

Magnesium isotopic analysis of cerebrospinal fluid (CSF) is a potentially interesting approach for studies on neurodegeneration. However, this type of analysis is challenging because of the invasiveness of the sampling and small sample volume. In this work, a novel analytical method was developed for ultrasensitive Mg isotopic analysis of CSF microsamples via multicollector inductively coupled plasma-mass spectrometry (MC-ICP-MS) using high-gain 10 13 Ω Faraday cup amplifiers. The intermediate and internal errors on the δ 26 Mg value were improved up to fourfold using 10 13 Ω resistors for the monitoring of both the 24 Mg and 26 Mg isotopes and up to twofold using a 10 11 Ω resistor for the most abundant 24 Mg isotope and a 10 13 Ω resistor for the 26 Mg isotope. Magnesium isotope ratios measured at a concentration level of 7-10 μg L -1 were in good agreement with those obtained using the conventional method at a concentration level of 150 μg L -1 . The expanded uncertainty for the quality control CSF material obtained at the ultratrace level was ±0.16‰. Ultrasensitive Mg isotopic analysis was carried out for CSF from hydrocephalus patients using only 5 μL of sample. δMg values thus obtained were not significantly different from those obtained using the conventional method using a sample volume of 400 μL instead ( p ≤ 0.05). The Mg isotopic composition of the CSF from hydrocephalus patients ranged between -0.65 and 0.30‰, with a mean δ 26 Mg value of -0.14 ± 0.27‰.

Background: Previous studies on the effect of healthy aging on Theory of Mind (ToM) have produced mixed results. A possible explanation may be that different ToM components and types of inference have not systematically been considered. This study examined the effect of aging on ToM by assessing both first and second order cognitive and affective components within a single task., Methods: We compared performance of young ( M = 18.3y) and older adults ( M = 61.0y) on the Yoni task. This task allows for a within-subject assessment of both first and second order cognitive and affective ToM., Results: We observed that older adults had longer reaction times than young adults across cognitive and affective first order items. For second order items, this age difference was larger for affective than cognitive items. Results showed no indications that these findings could be explained by age differences in speed/accuracy trade-offs., Conclusion: Our findings suggest that decision processes underlying ToM are slower in older adults on both first and second order inferences, but that age differences in these processes between cognitive and affective ToM are selective to second order inferences. We propose that the observed age differences may be associated with cortical and mental changes that occur with aging.

Recovery of stroke-related aphasia can be affected by language therapy in the early and chronic stage. Objectively monitoring therapy-induced neuroplasticity is possible by several measurement techniques including electro- and magneto-encephalography. The obtained event-related potentials (ERPs) and fields (ERFs) provide insights into the neural basis of intact or deficient language processing with milliseconds precision. In this literature review, we highlight the sensitivity of ERPs and ERFs to logopedic interventions by providing an overview of therapy-induced changes in the amplitude, latency and topography of early and mid-to-late components.

Oromandibular dystonia (OMD) is a clinically and etiologically heterogeneous form of focal dystonia with variable social and functional implications. The results of pharmacological treatment and botulinum toxin infiltrations are often unsatisfactory. We performed a systematic review on the effects of oral and dental appliances in patients with OMD. Most of the reports in the literature are single subject descriptions or small case series with a considerable variability in the type of dystonia, the type of evaluated appliances and in the outcome measures. Only one report included a large group of unselected patients that were evaluated with a mixture of outcome measures. Although it appears that a number of OMD patients, especially those who benefit from sensory tricks, may sustain some improvement with the use of oral appliances, no definitive conclusions can be drawn about the type of patients that may benefit, nor about the preferred type or mode of appliance. More research in this field is needed, using standardized approaches and clearly defined outcome measures in larger cohorts of OMD patients that are clinically and diagnostically well characterized.

We present two cases with postural axial tremor predominantly involving the head, trunk, and shoulders. In the first patient, the postural tremor occurred in multiple attacks a day lasting approximately 10 min. The second patient developed a progressive tremor of his head and arms, worsened during sitting and standing. Electrophysiological supported the postural axial tremor in both patients with a varying 3-10 Hz tremor frequency between different muscles and within the same muscles at different times. Postural axial tremor is a rare and complex movement disorder. The majority of cases are caused by acquired cerebellar pathology. However, isolated cases with underlying genetic disorders are described in literature. Here, we illustrate how to differentiate paroxysmal axial tremor from other axial hyperkinetic movement disorders and extend the genetic heterogeneity of this intriguing movement disorder phenotype., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Motor speech requires numerous neural computations including feedforward and feedback control mechanisms. A reduction of auditory or somatosensory feedback may be implicated in disorders of speech, as predicted by various models of speech control. In this paper the effects of reduced somatosensory feedback on articulation and intelligibility of individual phonemes was evaluated by using topical anesthesia of orobuccal structures in 24 healthy subjects. The evaluation was done using a combination of perceptual intelligibility estimation of consonants and vowels and acoustic analysis of motor speech. A significantly reduced intelligibility was found, with a major impact on consonant formation. Acoustic analysis demonstrated disturbed diadochokinesis. These results underscore the clinical importance of somatosensory feedback in speech control. The interpretation of these findings in the context of speech control models, neuro-anatomy and clinical neurology may have implications for subtyping of dysarthria., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Research on auditory processing in Parkinson's disease (PD) has recently made substantial progress. At present, evidence has been found for altered auditory processing in the clinical stage of PD. The auditory alterations in PD have been demonstrated with low-cost and non-invasive assessments that are already used in routine clinical practice. Since auditory alterations have been reported early in disease progression, it would be highly relevant to investigate whether auditory markers could be provided in the prodromal stage of PD. In addition, auditory alterations in early stage PD might be modulated by dopaminergic medication. Therefore, the aim of this review is (1) to summarize the literature on auditory processing in PD with a specific focus on the early disease stages, (2) to give future perspectives on which audiological and electrophysiological measurements could be useful in the prodromal stage of PD and (3) to assess the effect of dopaminergic medication on potential auditory markers in the prodromal stage of PD., (Copyright © 2020 De Groote, De Keyser, Santens, Talsma, Bockstael, Botteldooren and De Letter.)

Background: Paroxysmal dyskinesias (PDs) are a group of central nervous system diseases characterized by episodes of abnormal involuntary hyperkinetic movement without altered consciousness that increasingly have been recognized in dogs., Objectives: To present the phenotypical characterization, treatment, and outcome of a PD observed in Maltese dogs., Animals: Client-owned Maltese dogs (n = 19) with presumed diagnosis of PD., Methods: Data were collected retrospectively from medical records (2014-2019), and supporting information was added prospectively by using a questionnaire directed to the owners of the affected dogs., Results: The episodes were characterized mainly by sudden dystonia of ≥1 limbs and generalized body tremors with preserved consciousness. The mean age of clinical onset was 5.4 years. Episode frequency varied widely both among and within individuals. Median episode duration was 4.5 minutes. Most episodes were stress- or exercise-induced. Acetazolamide was administered to 6 dogs, and 4 dogs experienced a decrease in episode frequency. In 7 dogs that received a gluten-free diet, 6 dogs became episode-free. In 4 dogs, the episodes stopped spontaneously and in 2 dogs no medication or specific diet was given and the episodes continued at the same frequency., Conclusions and Clinical Importance: Given the breed predisposition and regional distribution of the disease, additional research should focus on elucidating the underlying genetic cause doing so might advance both our understanding of the pathophysiology and treatment of this disease, not only in dogs, but also in humans. Regardless of the treatment protocol selected, prognosis appears fair to good., (© 2020 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.)

Altered auditory processing has been increasingly recognized as a non-motor feature in parkinsonian disorders. This systematic review provides an overview of behavioral and electrophysiological literature on central auditory processing in patients with Parkinson's disease (PD), multiple system atrophy (MSA) and progressive supranuclear palsy (PSP). A systematic database search was conducted and yielded 88 studies that met the intelligibility criteria. The collected data revealed distinct impairments in a range of central auditory processes in PD, including altered deviance detection of basic auditory features, auditory brainstem processing, auditory gating and selective auditory attention. In contrast to PD, literature on central auditory processing in atypical parkinsonian disorders was relatively scarce, but provided some evidence for impaired central auditory processing in MSA and PSP. The interpretation of these findings is discussed and suggestions for further research are offered., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Local field potentials evoked by body action and mental action verbs were recorded in the subthalamic nucleus (STN) of 18 patients with Parkinson's disease through the electrodes implanted for deep brain stimulation. Compared with the medication on-condition, the medication off-condition showed a difference in activity in the early time segments, mainly in the right STN, with larger amplitudes for body action verbs. In the on-condition a similar pattern was detected in the left STN. These patterns of early differences in activity evoked by different types of verbs might indicate the potential of the STN to rapidly detect relevant behavioural clues in verbal content and to integrate these in subsequent cortico-subcortical interactions. In addition, these lateralizations allow speculations about shifts in processing activity correlating with dopaminergic denervation. Whether this detection relies on phonological, semantic or grammatical clues remains an open question., (Copyright © 2019 Elsevier Inc. All rights reserved.)

From a holistic point of view, semantic processes are subserved by large-scale subcortico-cortical networks. The dynamic routing of information between grey matter structures depends on the integrity of subcortical white matter pathways. Nonetheless, controversy remains on which of these pathways support semantic processing. Therefore, a systematic review of the literature was performed with a focus on anatomo-functional correlations obtained from direct electrostimulation during awake tumor surgery, and conducted between diffusion tensor imaging metrics and behavioral semantic performance in healthy and aphasic individuals. The 43 included studies suggest that the left inferior fronto-occipital fasciculus contributes to the essential connectivity that allows semantic processing. However, it remains uncertain whether its contributive role is limited to the organization of semantic knowledge or extends to the level of semantic control. Moreover, the functionality of the left uncinate fasciculus, inferior longitudinal fasciculus and the posterior segment of the indirect arcuate fasciculus in semantic processing has to be confirmed by future research., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Background: Myoclonus-dystonia (M-D) due to a pathogenic variant of SGCE is an autosomal dominant inherited movement disorder. Apart from motor symptoms, psychiatric disorders are highly prevalent in patients with M-D. Previous studies suggest, but never tested directly, that the type of psychiatric disorder differs between dystonia syndromes, probably related to disease specific pathology. Little is known about other non-motor symptoms (NMS) in M-D. Here, we systematically study NMS in M-D in direct comparison to other types of dystonia and healthy controls., Methods: Standardized questionnaires were used to assess type and severity of psychiatric co-morbidity, sleep problems, fatigue and quality of life. Results of M-D patients with a pathogenic variant of SGCE were compared to results of idiopathic cervical dystonia (CD) patients, dopa-responsive dystonia (DRD) patients with a pathogenic variant of GCH1 and controls., Results: We included 164 participants: 41 M-D, 51 CD, 19 DRD patients, 53 controls. Dystonia patients (M-D, CD and DRD) had an increased prevalence of psychiatric disorders compared to controls (56-74% vs. 29%). In M-D we found a significantly increased prevalence of obsessive-compulsive disorder (OCD) and psychosis compared to CD and DRD. All dystonia patients had more sleep problems (49-68% vs. 36%) and fatigue (42-73% vs. 15%) than controls. Compared to other dystonia subtypes, M-D patients reported less excessive daytime sleepiness and fatigue., Conclusion: Psychiatric comorbidity is frequent in all dystonia types, but OCD and psychosis are more common in M-D patients. Further research is necessary to elucidate underlying pathways., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Category selective recall in spontaneous speech after stroke has been reported only rarely. We recently described three cases demonstrating transient number speech in the acute stage of left hemispheric stroke and hypothesized a link with multilingualism and mathematical proficiency. In this report, we describe a similar case with a transient episode of utterances of randomly selected letters. Like in the three previous cases, this episode was preceded by a brief stage of mutism and ultimately evolved to Wernicke's aphasia over a period of days. This phenomenon is reviewed with reference to linguistic models and neuroanatomic and neurophysiological correlates.