Basal Ganglia Research Articles

Physiological effects of dual target DBS in an individual with Parkinson's disease and a sensing-enabled pulse generator

IntroductionDeep brain stimulation (DBS) of the subthalamic nucleus (STN) or globus pallidus (GP) is an established therapy for Parkinson's disease (PD). Novel DBS devices can record local field potential (LFP) physiomarkers from the STN or GP. While beta (13–30 Hz) and gamma (40–90 Hz) STN and GP LFP oscillations correlate with PD motor severity and with therapeutic effects of treatments, STN-GP interactions in electrophysiology in patients with PD are not well characterized. MethodsSimultaneous bilateral STN and GP LFPs were recorded in a patient with PD who received bilateral STN-DBS and GP-DBS. Power spectra in each target and STN-GP coherence were assessed in various ON- and OFF-levodopa and DBS states, both at rest and with voluntary movement. ResultsOFF-levodopa and OFF-DBS, beta peaks were present at bilateral STN and GP, coincident with prominent STN-GP beta coherence. Levodopa and dual-target-DBS (simultaneous STN-DBS and GP-DBS) completely suppressed STN-GP coherence. Finely-tuned gamma (FTG) activity at half the stimulation frequency (62.5 Hz) was seen in the STN during GP-DBS at rest. To assess the effects of movement on FTG activity, we recorded LFPs during instructed movement. We observed FTG activity in bilateral GP and bilateral STN during contralateral body movements while on GP-DBS and ON-levodopa. No FTG was seen with STN-DBS or dual-target-DBS. ConclusionDual-target-DBS and levodopa suppressed STN-GP coherence. FTG throughout the basal ganglia was induced by GP-DBS in the presence of levodopa and movement. This bilateral STN-FTG and GP-FTG corresponded with the least severe bradykinesia state, suggesting a pro-kinetic role for FTG.

The structural-functional-connectivity coupling of the aging brain.

Aging primarily affects memory and executive functions, a relationship that may be underpinned by the fact that almost all adults over 60years old develop small vessel disease (SVD). The fact that a wide range of neuropathologies could only explain up to 43% of the variation in age-related cognitive impairment suggests that other factors, such as cognitive reserve, may play a role in the brain's resilience against aging-related cognitive decline. This study aims to examine the relationship between structural-functional-connectivity coupling (SFC), and aging, cognitive abilities and reserve, and SVD-related neuropathologies using a cohort of n = 176 healthy elders from the Harvard Aging Brain Study. The SFC is a recently proposed biomarker that reflects the extent to which anatomical brain connections can predict coordinated neural activity. After controlling for the effect of age, sex, and years of education, global SFC, as well as the intra-network SFC of the dorsolateral somatomotor and dorsal attention networks, and the inter-network SFC between dorsolateral somatomotor and frontoparietal networks decreased with age. The global SFC decreased with total cognitive score. There were significant interaction effects between years of education versus white matter hyperintensities and between years of education versus cerebral microbleeds on inter-network SFC. Enlarged perivascular space in basal ganglia was associated with higher inter-network SFC. Our results suggest that cognitive ability is associated with brain coupling at the global level and cognitive reserve with brain coupling at the inter-functional-brain-cluster level with interaction effect from white matter hyperintensities and cerebral microbleed in a cohort of healthy elderlies.

Effects of botulinum neurotoxin on regularity of head oscillations in cervical dystonia

Introduction: This study explores the effects of botulinum neurotoxin (BoNT) on the relationship between dystonia and tremor, specifically focusing on cervical dystonia (CD) and its connection to head tremor.Methods: Fourteen CD patients were recruited; eight (57%) with clinically observable head oscillations were included in further analysis. A high-resolution magnetic search coil system precisely measured head movements, addressing two questions: 1) BoNT’s effects on head movement amplitude, frequency, and regularity, and 2) BoNT’s influence on the relationship between head position and head oscillations. For the first question, temporal head position measurements of three patients were analyzed before and after BoNT injection. The second question examined the effects of BoNT injections on the dependence of the oscillations on the position of the head.Results: Three distinct trends were observed: shifts from regular to irregular oscillations, transitions from irregular to regular oscillations, and an absence of change. Poincaré analysis revealed that BoNT induced changes in regularity, aligning oscillations closer to a consistent “set point” of regularity. BoNT injections reduced head oscillation amplitude, particularly in head orientations linked to high-intensity pre-injection oscillations. Oscillation frequency decreased in most cases, and overall variance in the amplitude of head position decreased post-injection.Discussion: These findings illuminate the complexity of CD but also suggest therapeutic potential for BoNT. They show that co-existing mechanisms contribute to regular and irregular head oscillations in CD, which involve proprioception and central structures like the cerebellum and basal ganglia. These insights advocate for personalized treatment to optimize outcomes that is based on individual head oscillation characteristics.

A Case of Posterior Communicating Artery Aneurysm Combined With an Atypical Origin of the Ipsilateral Ophthalmic Artery

Objective: Anatomy of the ophthalmic artery (OA) is of great practical importance in surgery for anterior circulation aneurysms. In most cases, the OA arises from the supraclinoid segment of the internal carotid artery (ICA) and enters the orbit through the optic canal inferolaterally to the optic nerve. The OA arising from the anterior cerebral artery (ACA) and entering the optic canal above the optic nerve is extremely rare.Clinical case: We report a case of a 29-year-old female patient admitted to Scientific Research Institute – Ochapovsky Regional Clinical Hospital No. 1 (Krasnodar, Russian Federation) with a ruptured true aneurysm of the left posterior communicating artery (PCoA) and an abnormal origin of the OA from the ACA. The disease manifested itself as ischemic stroke in the basal ganglia on the left causing diagnostic difficulties during initial hospitalization. Cerebral angiography confirmed a PCoA aneurysm and origin of the OA from the ipsilateral ACA. During surgery the neck of the aneurysm was clipped, and the aneurysm dome was opened to control the extent of clipping and remove thrombotic masses. During their removal, hemorrhage appeared from the posterior pole of the aneurysm. During the exploration it was found that the aneurysm was a dilated PCoA connecting the right ICA with the right posterior cerebral artery (PCA). A thrombosed anterior thalamoperforating artery emerged from the superior surface of the dome, and its junction with the precommunicating segment of the right PCA was detected in the region of the posterior pole of the aneurysm. The second clip was placed at the communication site between the aneurysm and the right PCA. The patient was discharged in satisfactory condition with a stable neurological status.Conclusions: The combination of abnormal anatomy of arteries at the base of the brain and cerebral aneurysms increases the risk of tactical errors and perioperative complications. Each such case requires a careful comparison of the clinical, imaging, and intraoperative findings. In the presented case of the patient with the ruptured rare PCoA aneurysm, the disease manifested atypically due to the partial thrombosis of the aneurysm and hemorrhage into the aneurysmal wall. The imaging findings revealed lacunar stroke in the thalamoperforating artery basin and an ICA aneurysm with no signs of subarachnoid hemorrhage. During the surgery the aneurysm was initially regarded as typical saccular. The opening of the dome and removal of all thrombotic masses made it possible to determine the eccentric fusiform nature of the aneurysm, perform its radical exclusion, and prevent fatal hemorrhage in the postoperative period.

The human subthalamic nucleus transiently inhibits active attentional processes.

The subthalamic nucleus (STN) of the basal ganglia is key to the inhibitory control of movement. Consequently, it is a primary target for the neurosurgical treatment of movement disorders like Parkinson's Disease, where modulating the STN via deep-brain stimulation (DBS) can release excess inhibition of thalamo-cortical motor circuits. However, the STN is also anatomically connected to other thalamo-cortical circuits, including those underlying cognitive processes like attention. Notably, STN-DBS can also affect these processes. This suggests that the STN may also contribute to the inhibition of non-motor activity, and that STN-DBS may cause changes to this inhibition. We here tested this hypothesis in humans. We used a novel, wireless outpatient method to record intracranial local field potentials (LFP) from STN DBS implants during a visual attention task (Experiment 1, N=12). These outpatient measurements allowed the simultaneous recording of high-density EEG, which we used to derive the steady-state visual evoked potential (SSVEP), a well-established neural index of visual attentional engagement. By relating STN activity to this neural marker of attention (instead of overt behavior), we avoided possible confounds resulting from STN's motor role. We aimed to test whether the STN contributes to the momentary inhibition of the SSVEP caused by unexpected, distracting sounds. Furthermore, we causally tested this association in a second experiment, where we modulated STN via DBS across two sessions of the task, spaced at least one week apart (N=21, no sample overlap with Experiment 1). The LFP recordings in Experiment 1 showed that reductions of the SSVEP after distracting sounds were preceded by sound-related γ-frequency (>60Hz) activity in the STN. Trial-to-trial modeling further showed that this STN activity statistically mediated the sounds' suppressive effect on the SSVEP. In Experiment 2, modulating STN activity via DBS significantly reduced these sound-related SSVEP reductions. This provides causal evidence for the role of the STN in the surprise-related inhibition of attention. These findings suggest that the human STN contributes to the inhibition of attention, a non-motor process. This supports a domain-general view of the inhibitory role of the STN. Furthermore, these findings also suggest a potential mechanism underlying some of the known cognitive side-effects of STN-DBS treatment, especially on attentional processes. Finally, our newly-established outpatient LFP recording technique facilitates the testing of the role of subcortical nuclei in complex cognitive tasks, alongside recordings from the rest of the brain, and in much shorter time than perisurgical recordings.

Multiple time delay induced Hopf bifurcation of a cortex - basal ganglia model for Parkinson’s Disease

Multiple time delay induced Hopf bifurcation of a cortex - basal ganglia model for Parkinson’s Disease

MicroRNA-7 attenuates secondary brain injury following experimental intracerebral hemorrhage via inhibition of NLRP3

Background and purposeThe pathophysiological mechanisms underlying brain injury resulting from intracerebral hemorrhage (ICH) remain incompletely elucidated, and efficacious therapeutic interventions to enhance the prognosis of ICH patients are currently lacking. Previous research indicates that MicroRNA-7 (miR-7) can suppress the expression of Nod-like receptor protein 3 (NLRP3), thereby modulating neuroinflammation in Parkinson's disease pathogenesis. However, the potential regulatory effects miR-7 on NLRP3 inflammasome after ICH are yet to be established. This study aims to ascertain whether miR-7 mitigates secondary brain injury following experimental ICH by inhibiting NLRP3 and to investigate the underlying mechanisms. MethodsAn ICH model was established by stereotaxically injecting 100 μL of autologous blood into the right basal ganglia of Sprague-Dawley (SD) rats. Subsequently, these rats were allocated into three groups: sham, ICH + Vehicle, and ICH + miR-7, each comprising 18 animals. Twelve hours post-modeling, rats received intraventricular injections of 10 μL physiological saline, 10 μL phosphate, and 10 μL phosphate-buffered saline solution containing 0.5 nmol of miR-7 mimics, respectively. Neurological function was assessed on day three post-modeling, followed by euthanasia for brain tissue collection. Brain water content was determined using the dry-wet weight method. The expression of inflammatory cytokines in cerebral tissues surrounding the hematoma was analyzed through immunohistochemistry and Western blot assays. These cytokines were re-evaluated using Reverse Transcription-Polymerase Chain Reaction (RT-PCR). Moreover, bioinformatics tools were employed to predict miR-7′s binding to NLRP3. A wild-type luciferase reporter gene vector and a corresponding mutant vector were constructed, followed by transfection of miR-7 mimics into HEK293T cells to assess luciferase activity. ResultsOur study demonstrates that the administration of miR-7 mimics markedly reduced neurological function scores and attenuated brain edema in rats following ICH. A significant upregulation of NLRP3 expression in microglia/macrophage adjacent to the hematoma was observed, substantially reduced after the treatment with miR-7 mimics. Furthermore, this intervention ameliorated neurodegenerative changes and effectively decreased the protein and mRNA levels of pro-inflammatory cytokines, namely TNF-α, IL-1β, IL-6, and Caspase1, in the cerebral tissues proximate to the hematomas. In addition, miR-7 mimics distinctly inhibited the luciferase activity associated with the wild-type reporter gene, an effect not mirrored in its mutant variant. ConclusionsThe miR-7 suppressed NLRP3 expression in microglia/macrophage to reduce the production of inflammatory cytokines, leading to conducting certain neuroprotection post-ICH in rats.

Differential patterns of functional connectivity in tremor dominant Parkinson's disease and essential tremor plus.

Tremor dominant Parkinson's disease (TDPD) and essential tremor plus (ETP) syndrome are commonly encountered tremor dominant neurological disorders. Although the basal ganglia thalamocortical (BGTC) and cerebello thalamocortical (CTC) networks are implicated in tremorogenesis, the extent of functional connectivity alterations across disorders is uncertain. This study aims to evaluate functional connectivity of the BGTC and CTC in TDPD and ETP. Resting state functional MRI was acquired for 25 patients with TDPD, ETP and 22 healthy controls (HC). Following pre-processing and denoising, seed-to-voxel based connectivity was carried out at FDR < 0.05 using ROIs belonging to the BGTC and CTC. Fahn-Tolosa-Marin tremor rating scale (FTMRS) was correlated with the average connectivity values at FDR < 0.05. Compared to HC, TDPD showed decreased connectivity between cerebellum and pre, post central gyrus. While, ETP showed decreased connectivity between pallidum and occipital cortex, precuneus, cuneus compared to HC. In comparison to ETP, TDPD showed increased connectivity between precentral gyrus, pallidum, SNc with the default mode network (DMN), and decreased connectivity between cerebellum with superior, middle frontal gyrus was observed. Tremor severity positively correlated with connectivity between SNc and DMN in TDPD, and negatively correlated with pallidal connectivity in ETP. Pattern of BGTC, CTC involvement is differential i.e., higher connectivity of the BGTC nodes in TDPD, and higher connectivity of cerebellar nodes in ETP. The interesting observation of pallidal involvement in ETP suggests the role of BGTC in the pathogenesis of ETP, and indicated similarities in concepts of tremor genesis in TDPD and ETP.

Degeneration in Nucleus basalis of Meynert signals earliest stage of Alzheimer’s disease progression

Nucleus Basalis of Meynert (NbM), a crucial source of cholinergic projection to the entorhinal cortex (EC) and hippocampus (HC), has shown sensitivity to neurofibrillary degeneration in the early stages of Alzheimer’s Disease. Using deformation-based morphometry (DBM) on up-sampled MRI scans from 1447 Alzheimer’s Disease Neuroimaging Initiative participants, we aimed to quantify NbM degeneration along the disease trajectory. Results from cross-sectional analysis revealed significant differences of NbM volume between cognitively normal and early mild cognitive impairment cohorts, confirming recent studies suggesting that NbM degeneration happens before degeneration in the EC or HC. Longitudinal linear mixed-effect models were then used to compare trajectories of volume change after realigning all participants into a common timeline based on their cognitive decline. Results indicated the earliest deviations in NbM volumes from the cognitively healthy trajectory, challenging the prevailing idea that Alzheimer’s originates in the EC. Converging evidence from cross-sectional and longitudinal models suggest that the NbM may be a focal target of early AD progression, which is often obscured by normal age-related decline.

Angong niuhuang pill improves the neurological function of younger patients with basal ganglia and cerebral lobe intracerebral hemorrhage: A randomized controlled trial

Background &amp; Objective: Spontaneous intracerebral hemorrhage (SICH) is one of the stroke forms with the highest disability and mortality rates and significant differences in prognosis among patients with different locations of SICH. Angong Niuhuang Pill (ANP) 安宫牛黄丸 is one of the most well-known traditional Chinese patent medicines used in the clinical emergency treatment of cardio-cerebrovascular diseases. This prospective study was performed to determine the therapeutic effect of ANP in different locations of SICH. Methods: Patients with basal ganglia(n=81) and cerebral lobe (n=67) hemorrhage were randomly assigned into two groups: the standard medical management (SMM) group and the combined ANP as well as medical management group (ANP group). Fisher exact test and Mann- Whitney U test were used for comparing the differences between two groups. Primary and secondary outcomes were the 6-month modified Rankin Scale (mRS) scores and the 2-week Brunnstrom stage ratings. Results: For patients with basal ganglia hemorrhage and cerebral lobe hemorrhage, there were no significant differences observed between the ANP group and the SMM group in terms of patient age (53.1±5.3 vs 52.7±6.4; 54.7±4.8 vs 52.7±5.9), sex (male:64.3% vs 53.8%; 63.6% vs 58.8%), body mass index (25.1±2.1 vs 24.2±2.6; 25.6±2.0 vs 25.0±2.4), mean hematoma volume (17.1±6.6 vs 18.6±5.7; 18.4±7.4 vs 18.9±6.0), as well as the proportions of hypertension (88.1%vs71.8%; 90.9% vs 76.5%), alcoholism (40.5% vs 51.3%; 54.6% vs 41.2%), smoking (40.5% vs 38.5%; 36.4% vs 38.2%), and diabetes mellitus (21.4% vs 18%; 12.1% vs 17.7%). The proportion of basal ganglia hemorrhage patients with a 6-month mRS score of 0-2 in the ANP group was significantly higher than that in the SMM group (26.2% vs. 2.6%, P=0.004), but it was not significantly improved in patients with cerebral lobe hemorrhage (57.6% vs 38.2%, P=0.145). The 2-week Brunnstrom stage ratings of patients with basal ganglia hemorrhage were significant difference (P=0.048). But for patients with cerebral lobe hemorrhage, there was no significant difference (P=0.164). The incidence of liver dysfunction, renal dysfunction, gastrointestinal dysfunction, hypothermia and allergy in the two groups of patients was not significantly different. Conclusions: ANP has different therapeutic effects on different locations of SICH, and has the most obvious effect on improving the long-term neurological function of patients with basal ganglia hemorrhage.

Deep Learning Accelerated Brain Diffusion-Weighted MRI with Super Resolution Processing

ObjectivesTo investigate the clinical feasibility and image quality of accelerated brain diffusion-weighted imaging (DL-DWI) with deep learning image reconstruction and super resolution. MethodsEighty-five consecutive patients with clinically indicated MRI at a 3T scanner were prospectively included. Conventional diffusion-weighted data (c-DWI) with four averages were obtained. Reconstructions of one and two averages, as well as DL-DWI, were accomplished. Three experienced readers evaluated the acquired data using a 5-point Likert scale regarding overall image quality, overall contrast, diagnostic confidence, occurrence of artefacts and evaluation of the central region, basal ganglia, brainstem, and cerebellum. To assess interrater agreement, Fleiss’ kappa (ϰ) was determined. Signal intensity (SI) levels for basal ganglia and the central region were estimated via automated segmentation, and SI values of detected pathologies were measured. ResultsIntracranial pathologies were identified in thirty-five patients. DL-DWI was significantly superior for all defined parameters, independently from applied averages (p-value <0.001). Optimum image quality was achieved with DL-DWI by utilizing a single average (p-value <0.001), demonstrating very good (80.9%) to excellent image quality (14.5%) in nearly all cases, compared to 12.5% with very good and 0% with excellent image quality for c-MRI (p-value <0.001). Comparable results could be shown for diagnostic confidence. Inter-rater Fleiss' Kappa demonstrated moderate to substantial agreement for virtually all defined parameters, with good accordance, particularly for the assessment of pathologies (p=0.74). Regarding SI values, no significant difference was found. ConclusionUltra-fast diffusion-weighted imaging with super resolution is feasible, resulting in highly accelerated brain imaging while increasing diagnostic image quality.

Moving across disorders: A cross-sectional study of cognition in early onset ataxia and dystonia

BackgroundEarly onset ataxia (EOA) and Early Onset Dystonia (EOD) are movement disorders developing in young people (age <25 per definition). These disorders result from dysfunctional networks involving the cerebellum and basal ganglia. As these structures are also important for cognition, cognitive deficits can be expected in EOA and EOD. EOA and EOD sometimes co-occur, but in those cases the predominant phenotype is determining. A pending question is whether predominantly EOA and EOD have different profiles of cognitive impairment. ObjectivesWe investigated whether cognitive functions were impaired in patients with either predominant EOA or predominant EOD and whether cognitive profiles differed between both patient groups. MethodsThe sample consisted of 26 EOA and 26 EOD patients with varying etiology but similar duration and severity of the disorder. Patient samples were compared to a group of 26 healthy controls, all matched on age and gender. All participants underwent neuropsychological testing for verbal intelligence, memory, working memory, attention/cognitive speed, executive functions, emotion recognition and language. ResultsEOA and EOD patients both performed significantly worse than healthy controls on tests of verbal intelligence, working memory and executive functions. Additionally, attention/cognitive speed and emotion recognition were impaired in the EOA group. Compared to EOD, EOA patients performed worse on attention/cognitive speed and verbal intelligence. ConclusionsOur results show overall similar profiles of cognitive deficits in both patient groups, but deficits were more pronounced in the patients with EOA. This suggests that more severe cognitive impairment is related to more severe cerebellar network dysfunction.

Gamma-delta T-cell lymphoma of the central nervous system: A case report and review of the literature.

Primary T-cell lymphoma (TCL) of the central nervous system (CNS) is a rare and potentially aggressive entity. We describe a case of TCL presenting in the basal ganglia with γδ receptor expression and a remarkably aggressive clinical course. To the best of our knowledge, this is the fifth reported case of γδ TCL presenting in the CNS. We review existing literature, including the previously reported cases of γδ TCL of the CNS. In our case, a 69-year-old male presented with acute onset dysarthria and right-sided weakness, with initial imaging concerning for stroke. Repeat imaging demonstrated a 2.6-cm mass in the left basal ganglia-corona radiata. Pathologic examination of a stereotactic biopsy revealed TCL with γδ receptor phenotype. The patient suffered rapid clinical decline and passed away within 6 weeks of initial diagnosis. This represents an important differential diagnosis and sheds light on the potentially poor prognosis conferred by γδ TCL of the CNS.

The effect of valproate on dystonic movement in a moyamoya patient: A case report

Moyamoya disease (MMD) is a progressive cerebrovascular disorder caused by narrowing of the arteries located at the base of the brain in the basal ganglia. Dystonia is a relatively rare symptom of MMD, and little is known about the treatment of this symptom in MMD patients. We report a case of dystonia in an MMD patient that was alleviated after valproate treatment. A 57-year-old female patient with MMD presented with new-onset dystonia of the left upper extremity and left hemifacial spasm. Although she already suffered from mild cognitive dysfunction, there was no exacerbation when dystonia presented. She was treated with valproate 300 mg b.i.d, and the symptoms were alleviated. We discuss the efficacy of valproate in terms of anatomy and neurotransmitters. In addition, the patient’s symptoms are similar to those associated with LGI-1 encephalitis but are distinguished by the progression of the disease.

Reversible dementia and insomnia in ABGA related encephalitis

BackgroundABGAs are historically associated with Encephalitis Lethargica (EL). Typically ABGAs are also found in children resulting in a variety of neuropsychiatric and extrapyramidal disorders, rare cases are reported in adults with atypical movement disorders. No description of basal ganglia reversible lesions related to ABGAs are reported and these antibodies are not included in the list of autoimmune encephalitis. Methods and resultsA 55 years old female presented sub-acute onset of an anxious-depressive disorder and obsessive-compulsive behavior associated with intractable insomnia affecting sleep onset and sleep maintenance. Brain-MRI showed diffuse hyperintensities on FLAIR sequences in the basal ganglia. A therapy with IV-immunoglobulin was started and the clinical condition improved dramatically and insomnia and psychiatric symptoms resolved completely. ConclusionOur case highlights the importance of making a fast diagnosis. When caught early ABGAs-related encephalitis is susceptible of a good outcome and response to treatment. Reversible insomnia and dementia in our case expand ABGA clinical presentation in adults and favors the hypothesis of an immune pathogenesis for Encephalitis Lethargica, especially in the hyperkinetic form as previously suggested, as in our case.

ERBB1 alleviates secondary brain injury induced by experimental intracerebral hemorrhage in rats by modulating neuronal death via PLC-γ/PKC pathway.

Intracerebral hemorrhage (ICH) is a disease with high rates of disability and mortality. The role of epidermal growth factor receptor 1 (ERBB1) in ICH was elucidated in this study. ICH model was constructed by injecting autologous arterial blood into the right basal ganglia. The protein level of ERBB1 was detected by western blot analysis. To up- and downregulation of ERBB1 in rats, intraventricular injection of a lentivirus overexpression vector of ERBB1 and AG1478 (a specific inhibitor of ERBB1) was used. The cell apoptosis, neuronal loss, and pro-inflammatory cytokines were assessed by TUNEL, Nissl staining, and ELISA. Meanwhile, behavioral cognitive impairment of ICH rats was evaluated after ERBB1-targeted interventions. ERBB1 increased significantly in brain tissue of ICH rats. Overexpression of ERBB1 remarkably reduced cell apoptosis and neuronal loss induced by ICH, as well as pro-inflammatory cytokines and oxidative stress. Meanwhile, the behavioral and cognitive impairment of ICH rats were alleviated after upregulation of ERBB1; however, the secondary brain injury (SBI) was aggravated by AG1478 treatment. Furthermore, the upregulation of PLC-γ and PKC in ICH rats was reversed by AG1478 treatment. ERBB1 can improve SBI and has a neuroprotective effect in experimental ICH rats via PLC-γ/PKC pathway.

Association of abnormal explicit sense of agency with cerebellar impairment in myoclonus-dystonia.

Non-motor aspects in dystonia are now well recognized. The sense of agency, which refers to the experience of controlling one's own actions, has been scarcely studied in dystonia, even though its disturbances can contribute to movement disorders. Among various brain structures, the cerebral cortex, the cerebellum, and the basal ganglia are involved in shaping the sense of agency. In myoclonus dystonia, resulting from a dysfunction of the motor network, an altered sense of agency may contribute to the clinical phenotype of the condition. In this study, we compared the explicit and implicit sense of agency in patients with myoclonus dystonia caused by a pathogenic variant of SGCE (DYT-SGCE) and control participants. We utilized behavioural tasks to assess the sense of agency and performed neuroimaging analyses, including structural, resting-state functional connectivity, and dynamic causal modelling, to explore the relevant brain regions involved in the sense of agency. Additionally, we examined the relationship between behavioural performance, symptom severity, and neuroimaging findings. We compared 19 patients with DYT-SGCE and 24 healthy volunteers. Our findings revealed that patients with myoclonus-dystonia exhibited a specific impairment in explicit sense of agency, particularly when implicit motor learning was involved. However, their implicit sense of agency remained intact. These patients also displayed grey-matter abnormalities in the motor cerebellum, as well as increased functional connectivity between the cerebellum and pre-supplementary motor area. Dynamic causal modelling analysis further identified reduced inhibitory effects of the cerebellum on the pre-supplementary motor area, decreased excitatory effects of the pre-supplementary motor area on the cerebellum, and increased self-inhibition within the pre-supplementary motor area. Importantly, both cerebellar grey-matter alterations and functional connectivity abnormalities between the cerebellum and pre-supplementary motor area were found to correlate with explicit sense of agency impairment. Increased self-inhibition within the pre-supplementary motor area was associated with less severe myoclonus symptoms. These findings highlight the disruption of higher-level cognitive processes in patients with myoclonus-dystonia, further expanding the spectrum of neurological and psychiatric dysfunction already identified in this disorder.

Midline invasion predicts poor prognosis in diffuse hemispheric glioma, H3 G34-mutant: an individual participant data review

IntroductionDiffuse hemispheric glioma, H3 G34-mutant (DHGs), is a newly categorized tumor in pediatric-type diffuse high-grade gliomas, World Health Organization grade 4, with a poor prognosis. Although prognostic factors associated with genetic abnormalities have been reported, few reports have examined the clinical presentation of DHGs, especially from the viewpoint of imaging findings. In this study, we investigated the relationship between clinical factors, including imaging findings, and prognosis in patients with DHGs.MethodsWe searched Medline through the PubMed database using two search terms: “G34” and “glioma”, between 1 April 2012 and 1 July 2023. We retrieved articles that described imaging findings and overall survival (OS), and added one DHG case from our institution. We defined midline invasion (MI) as invasion to the contralateral cerebrum, brainstem, corpus callosum, thalamus, and basal ganglia on magnetic resonance imaging. The primary outcome was 12-month survival, estimated using Kaplan–Meier curves and logistic regression.ResultsA total of 96 patients were included in this study. The median age was 22 years, and the proportion of male patients was 48.4%. Lesions were most frequently located in the frontal lobe (52.6%). MI was positive in 39.6% of all patients. The median OS was 14.4 months. Univariate logistic regression analysis revealed that OS was significantly worse in the MI-positive group compared with the MI-negative group. Multivariate logistic regression analysis revealed that MI was an independent prognostic factor in DHGs.ConclusionsIn this study, MI-positive cases had a worse prognosis compared with MI-negative cases.Previous presentationsNo portion of this study has been presented or published previously.

Neurological and Vascular Behçet's Disease in a Young Male without Classic Triad of Behçet's Disease: A Case Report and Literature Review.

Neuro-Behçet's disease (NBD) is an uncommon presentation in Behçet's disease (BD) with severe course and worse prognosis. Both vascular and NBD presentation without the classical triad of BD in a single patient is rarely reported. Here a 48-year-old male had an extensive aortic aneurysm eroding vertebra for which he was diagnosed as vascular BD. Two years later, he was presented with a severe headache and cerebrovascular accident, his brain imaging showed hyperintensity in the right thalamus, basal ganglia, temporal lobe, and internal capsule, suggesting the 'cascade sign' of NBD. Surprisingly, he never had oral or genital ulcers or skin and eye involvement. He had a good response to infliximab. Clustering of BD phenotype is an emerging area of interest. It is hypothesised that severe phenotype of vascular and parenchymal NBD can happen in the same patient owing to similar underlying pathology. This case is unique due to its severe phenotype with no features of the typical triad of BD.

Study on location of cerebral lesion among patients with hemorrhagic cerebrovascular accident

Background: In hemorrhagic cerebrovascular accident, bleeding occurs directly into the brain parenchyma. Intracerebral hemorrhage usually occurs at certain sites in the brain, i.e., thalamus, putamen, cerebellum, and brain stem. The surrounding area of brain may be damaged by pressure produced by the mass effect of the hematoma. Increase in intracranial pressure occurs. Aims and Objectives: The aim and objective of the study are to compare the localization of cerebral lesions with the frequency of intraventricular hemorrhage and to study the prognosis of hemorrhagic cerebrovascular accident (CVA) as per its anatomical location in computed tomography (CT) scan. Materials and Methods: One-year observational cross-sectional study was conducted in 60 patients with CT scan diagnosed hemorrhagic CVA. Results: Regarding the site of the hemorrhagic CVA, the most commonly affected area was basal ganglia (46.7%) followed by thalamus (18.3%); other areas include pons (8.3%), paraventricular (8.3%), cerebellar (6.7%), lobar (5%), internal capsule (3.3%), and intraventricular (3.3%). Conclusion: This study reflects a spectrum of cerebral location of hemorrhagic stroke which may help in the management of hemorrhagic CVA patients.