Search

Background: Deep brain stimulation (DBS) targeting the subthalamic nucleus (STN) has emerged as a potent treatment for alleviating motor symptoms in Parkinson's disease (PD). Despite its effectiveness, the impact of high frequency STN-DBS on cerebellar oscillations remains unclear, posing an intriguing challenge for neural modulation. Given the direct and indirect connections between the STN and cerebellum, we investigated whether STN-DBS affects cerebellar oscillations. Objective: To observe the effects of STN-DBS on cerebellar oscillations in patients with PD. Methods: We recruited 15 PD patients receiving STN-DBS. Electroencephalographic (EEG) signals were recorded from cerebellar regions during resting-state conditions in both the OFF-DBS and STN-DBS conditions. Our analyses centered on spectral features, particularly theta and beta oscillations, guided by prior research and correlation tests to investigate the relationship between oscillatory changes and motor symptom severity. Results: In the mid-cerebellar (Cbz) region, we observed a significant increase in the relative power in all frequency bands, including theta and beta oscillations during STN-DBS, showing the global effect of DBS. Importantly, the correlation results indicated significant associations between mid-cerebellar (Cbz) beta power during the OFF condition and motor severity, which were not evident during STN-DBS. Interestingly, correlations between beta power and motor severity were not observed at the mid-occipital (Oz) and mid-frontal (Cz) regions. Notably, signal similarity analyses demonstrated no evidence of volume conduction effects between the mid-cerebellar (Cbz) and nearby mid-occipital (Oz) regions. Conclusions: While these findings provide valuable insights into the complex interplay between STN-DBS and neural oscillations, further research is essential to decipher their precise functional significance and clinical implications. Understanding these intricacies may contribute to the optimization of DBS therapies for PD. Plain Language Summary: Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is an effective treatment for reducing motor symptoms in Parkinson's disease (PD). However, its effects on brain activity, specifically in the cerebellum, are not well understood. This study aimed to investigate how STN-DBS affects cerebellar brain waves in PD patients. We recruited 15 PD patients undergoing STN-DBS and recorded their brain activity including cerebellar region using EEG. We compared the brain wave patterns during periods when the DBS was turned OFF and when it was turned ON, focusing on specific brain wave frequencies (theta and beta). The results showed a significant increase in brain wave power across all frequencies in the mid-cerebellar region during STN-DBS. Additionally, there was a strong link between beta power in the cerebellum and motor symptom severity when DBS was OFF, which was not present when DBS was ON. This relationship was specific to the cerebellum and not found in other brain regions. The findings suggest that STN-DBS significantly alters cerebellar brain activity and that these changes are related to improvements in motor symptoms. However, more research is needed to fully understand the functional significance and potential clinical applications of these findings for optimizing DBS treatment in PD patients. [ABSTRACT FROM AUTHOR]

Anti-Purkinje cell cytoplasmic antibody type 2 (PCA-2) is associated with various neurological conditions in adults. However, related studies have not been conducted in children. The present study aimed to characterize the clinical features and outcomes of PCA-2-related autoimmune cerebellar degeneration in pediatric patients. A total of 357 pediatric patients with acute or subacute cerebellar ataxia were recruited for the study from June 2015 to September 2022. Of these, PCA-2 was identified in four patients. Information on the clinical manifestations, patient response to treatment, and outcomes was collected and analyzed. The patient cohort in the present study included two boys and two girls, with the age of onset from six to 12 years. Axial ataxia was the most remarkable symptom observed in the entire patient cohort (four of four), followed by dysmetria in 75% (three of four), dysarthria in 50% (two of four), and nystagmus in 25% (one of four) of patients. Cognitive impairment was present in one patient. Peripheral neuropathy, which is an extracerebellar symptom, was found in two patients. One patient was diagnosed with a pelvic neuroblastoma before the onset of ataxia. The presence of oligoclonal bands was confirmed in the cerebrospinal fluid, and cerebellar atrophy was observed. Immunotherapy, including glucocorticoids and/or intravenous immunoglobulin, was administered to all four patients immediately following diagnosis, and mycophenolate mofetil was administered to three patients. Three patients responded to immunotherapy. In children, PCA2-associated autoimmune cerebellar degeneration is rare, and they show comparatively fewer symptoms than adults. Timely and appropriate immunotherapy is beneficial. [ABSTRACT FROM AUTHOR]

Background: Tremor is the most common movement disorder, with significant functional and psychosocial consequences. Oral medications have been disappointing or limited by side effects. Surgical techniques are effective but associated with risks and adverse events. Botulinum toxin (BT) represents a promising avenue but there is still no double-blind evidence of efficacy on upper limb function. A systematic review on the effects of BT in upper-limb tremor was conducted. Methods: A systematic search of the literature was conducted up to July 2023, including the keywords "botulinum toxin" and "tremor". All randomized controlled trials (RCTs) and open-label studies were analyzed. Independent reviewers assessed their methodological quality. Results: There were only eight published RCTs and seven published open-label studies, with relatively small sample sizes. This review suggests that BT is more effective when injections are patient-tailored, with analyses based on clinical judgement or kinematics. Subjective and objective measures frequently improve but transient weakness may occur after injections, especially if wrist or fingers extensors are targeted. A number of studies had methodological limitations. Conclusions: The authors discuss how to optimize tremor assessments and effects of BT injection. Controlled evidence is still lacking but it is suggested that distal "asymmetric" BT injections (targeting flexors/pronators while sparing extensors/supinators) and proximal injections, involving shoulder rotators when indicated, may avoid excessive weakness while optimizing functional benefit. [ABSTRACT FROM AUTHOR]

We present a case of a 70-year-old male who presented with left-sided weakness and dysarthria. Cranial imaging was suggestive of a cerebellar infarct and the patient was treated with aspirin and clopidogrel. Two months later a fall prompted further cranial imaging, which was concerning for an intracranial mass with vasogenic edema. Computed tomography angiogram (CTA) was negative for vascular lesion. Ultimately, a DSA revealed a Borden III dAVF between the right occipital artery and the posterior cerebellar vein that was treated with endovascular embolization.

Abstract Background The clinical features of cerebellar high-grade gliomas (cHGGs) in adults have not been thoroughly explored. This large-scale, population-based study aimed to comprehensively outline these traits and construct a predictive model. Methods Patient records diagnosed with gliomas were collected from various cohorts and analyzed to compare the features of cHGGs and supratentorial HGGs (sHGGs). Cox regression analyses were employed to identify prognostic factors for overall survival and to develop a nomogram for predicting survival probabilities in patients with cHGGs. Multiple machine learning methods were applied to evaluate the efficacy of the predictive model. Results There were significant differences in prognosis, with SEER-cHGGs showing a median survival of 7.5 months and sHGGs 14.9 months (p

Abstract Background Over the last decade, there has been an emerging trend of recreational misuse of several drugs and inhaled solvent including ethyl chloride. This case report follows CARE guidelines and highlights, with supporting video, the neurological features of ethyl chloride intoxication. Case presentation A 48-year-old man was seen for the sudden occurrence of an unsteady gait with dizziness. His only medical history was a chronic and treated HIV infection without any complications. Clinical examination revealed a cerebellar syndrome associated with impairment of short-term memory. Biological and radiological workups were normal. After several days, the patient recalled ethyl chloride inhalation. He fully recovered after being discharged from hospital. Conclusion Clinicians should recognise the clinical features and neurological manifestations of ethyl chloride intoxication due to the potential fatal cardiovascular complications of this intoxication.

Background Objective Methods Results Conclusions There are more than 30 immunoglobulin G (IgG) autoantibody biomarkers of autoimmune cerebellar ataxia reported.To describe a novel IgG biomarker targeting neurobeachin, a scaffolding protein integral to synapse formation and function.Clinical and laboratory‐based assessment was used for a novel diagnostic biomarker of autoimmune ataxia.A 46‐year‐old woman had ataxia onset followed by bilateral knee pain, attributable to rheumatoid arthritis. She was negative for standard neural antibodies. Her ataxia stabilized and tremor initially improved after corticosteroid treatment, but she progressed to wheelchair‐dependence (on methotrexate and then rituximab) over 4 years, in keeping with the trajectory encountered by many with autoimmune ataxia. In contrast, her inflammatory arthritis remitted with immunotherapy. Neurobeachin‐IgG was unmasked in serum and cerebrospinal fluid using protein arrays and confirmed using neurobeachin‐specific confocal immunofluorescence and Western blot.Neurobeachin‐IgG is a new neural antibody biomarker of cerebellar ataxia among a growing antibody repertoire, collectively common but often individually rare. © 2024 International Parkinson and Movement Disorder Society. [ABSTRACT FROM AUTHOR]

The cerebellum is important for motor adaptation. Lesions to the vestibulo-cerebellum selectively cause gait ataxia. Here we investigate how such damage affects locomotor adaptation when performing the 'broken escalator' paradigm. Following an auditory cue, participants were required to step from the fixed surface onto a moving platform (akin to an airport travellator). The experiment included three conditions: 10 stationary (BEFORE), 15 moving (MOVING) and 10 stationary (AFTER) trials. We assessed both behavioural (gait approach velocity and trunk sway after stepping onto the moving platform) and neuromuscular outcomes (lower leg muscle activity, EMG). Unlike controls, cerebellar patients showed reduced after-effects (AFTER trials) with respect to gait approach velocity and leg EMG activity. However, patients with cerebellar damage maintain the ability to learn the trunk movement required to maximise stability after stepping onto the moving platform (i.e., reactive postural behaviours). Importantly, our findings reveal that these patients could even initiate these behaviours in a feedforward manner, leading to an after-effect. These findings reveal that the cerebellum is crucial for feedforward locomotor control, but that adaptive locomotor behaviours learned via feedback (i.e., reactive) mechanisms may be preserved following cerebellum damage. [ABSTRACT FROM AUTHOR]

Purpose: The Posterior Fossa Society, an international multidisciplinary group, hosted its first global meeting designed to share the current state of the evidence across the multidisciplinary elements of pediatric post-operative cerebellar mutism syndrome (pCMS). The agenda included keynote talks from world-leading speakers, compelling abstract presentations and engaging discussions led by members of the PFS special interest groups. Methods: This paper is a synopsis of the first global meeting, a 3-day program held in Liverpool, England, UK, in September 2022. Results: Topics included nosology, patient and family experience, cerebellar modulation of cognition, and cerebellar cognitive affective syndrome. In addition, updates from large-scale studies were shared as well as abstracts across neuroradiology, neurosurgery, diagnosis/scoring, ataxia, and rehabilitation. Conclusions: Based on data-driven evidence and discussions, each special interest group created research priorities to target before the second global meeting, in the spring of 2024. [ABSTRACT FROM AUTHOR]

Background: Over the last decade, there has been an emerging trend of recreational misuse of several drugs and inhaled solvent including ethyl chloride. This case report follows CARE guidelines and highlights, with supporting video, the neurological features of ethyl chloride intoxication. Case presentation: A 48-year-old man was seen for the sudden occurrence of an unsteady gait with dizziness. His only medical history was a chronic and treated HIV infection without any complications. Clinical examination revealed a cerebellar syndrome associated with impairment of short-term memory. Biological and radiological workups were normal. After several days, the patient recalled ethyl chloride inhalation. He fully recovered after being discharged from hospital. Conclusion: Clinicians should recognise the clinical features and neurological manifestations of ethyl chloride intoxication due to the potential fatal cardiovascular complications of this intoxication. [ABSTRACT FROM AUTHOR]

Abstract Objective To detect demographic, clinical, and radiological predictors of patient outcomes and prognosis in spontaneous cerebellar hematoma patients that may guide accurate decision making. Methods This retrospective study was conducted on 45 patients with spontaneous cerebellar hematoma who underwent surgical treatment. The patient data including demographics (age, gender), preoperative consciousness at the time of admission, medical comorbidities (HTN, DM), radiological findings (location, volume, and diameter of the cerebellar hematoma, hydrocephalus, compression of the fourth ventricle, effacement of the quadrigeminal cistern, intraventricular hematoma, tight posterior fossa, and brain stem compression), and treatment methods were collected and correlated with prognosis. Outcome assessment was done using the Glasgow Outcome Scale (GOS), classified into good outcome (favorable result; GOS > 4) and poor outcome (unfavorable result; GOS

Objective: To detect demographic, clinical, and radiological predictors of patient outcomes and prognosis in spontaneous cerebellar hematoma patients that may guide accurate decision making. Methods: This retrospective study was conducted on 45 patients with spontaneous cerebellar hematoma who underwent surgical treatment. The patient data including demographics (age, gender), preoperative consciousness at the time of admission, medical comorbidities (HTN, DM), radiological findings (location, volume, and diameter of the cerebellar hematoma, hydrocephalus, compression of the fourth ventricle, effacement of the quadrigeminal cistern, intraventricular hematoma, tight posterior fossa, and brain stem compression), and treatment methods were collected and correlated with prognosis. Outcome assessment was done using the Glasgow Outcome Scale (GOS), classified into good outcome (favorable result; GOS > 4) and poor outcome (unfavorable result; GOS < 4). Results: There was a statistically significant correlation (p = 0.030) between the time to surgery and GOS, with more favorable outcomes in patients who underwent early surgery compared to late surgery. Unfavorable outcomes were detected in patients with higher Taneda grade and Kirollos grade, and both were statistically significant (p = 0.001). The presence of hydrocephalus and/or brainstem compression had a statistically significant (p < 0.001) negative impact on outcomes. Patients with extensive IVH had an unfavorable outcome compared to other patients (p = 0.016). The mortality rate in our study was 28.9%. Conclusions: Surgical management of spontaneous cerebellar hematoma is challenging. Many factors influence the appropriate decision making and surgical outcome. Predictors of an unfavorable outcome include the level of consciousness at admission, delayed time to surgery, a higher grade of 4th ventricular effacement (Kirollos) and quadrigeminal cistern effacement (Taneda), extensive IVH, hydrocephalus, and brainstem compression (statistically significant). There was no statistical significance regarding gender, age, hematoma volume, or hematoma diameter in relation to an unfavorable outcome. [ABSTRACT FROM AUTHOR]

Introduction: Electroconvulsive therapy (ECT) is widely used for treatment‐resistant depression. However, it is unclear whether/how ECT can be targeted to affect brain regions and circuits in the brain to dynamically regulate mood and cognition. Methods: This study used brain entropy (BEN) to measure the irregular levels of brain systems in 46 major depressive disorder (MDD) patients before and after ECT treatment. Functional connectivity (FC) was further adopted to reveal changes of functional couplings. Moreover, transcriptomic and neurotransmitter receptor data were used to reveal genetic and molecular basis of the changes of BEN and functional connectivities. Results: Compared to pretreatment, the BEN in the posterior cerebellar lobe (PCL) significantly decreased and FC between the PCL and the right temporal pole (TP) significantly increased in MDD patients after treatment. Moreover, we found that these changes of BEN and FC were closely associated with genes' expression profiles involved in MAPK signaling pathway, GABAergic synapse, and dopaminergic synapse and were significantly correlated with the receptor/transporter density of 5‐HT, norepinephrine, glutamate, etc. Conclusion: These findings suggest that loops in the cerebellum and TP are crucial for ECT regulation of mood and cognition, which provides new evidence for the antidepressant effects of ECT and the potential molecular mechanism leading to cognitive impairment. [ABSTRACT FROM AUTHOR]

Background: Tremor is the most common movement disorder, with significant functional and psychosocial consequences. Oral medications have been disappointing or limited by side effects. Surgical techniques are effective but associated with risks and adverse events. Botulinum toxin (BT) represents a promising avenue but there is still no double-blind evidence of efficacy on upper limb function. A systematic review on the effects of BT in upper-limb tremor was conducted. Methods: A systematic search of the literature was conducted up to July 2023, including the keywords “botulinum toxin” and “tremor”. All randomized controlled trials (RCTs) and open-label studies were analyzed. Independent reviewers assessed their methodological quality. Results: There were only eight published RCTs and seven published open-label studies, with relatively small sample sizes. This review suggests that BT is more effective when injections are patient-tailored, with analyses based on clinical judgement or kinematics. Subjective and objective measures frequently improve but transient weakness may occur after injections, especially if wrist or fingers extensors are targeted. A number of studies had methodological limitations. Conclusions: The authors discuss how to optimize tremor assessments and effects of BT injection. Controlled evidence is still lacking but it is suggested that distal “asymmetric” BT injections (targeting flexors/pronators while sparing extensors/supinators) and proximal injections, involving shoulder rotators when indicated, may avoid excessive weakness while optimizing functional benefit.

Objective: To investigate the topical diagnosis, possible etiology and mechanism of spontaneous downbeat nystagmus (sDBN) patients with dizziness/vertigo. Methods: The clinical features of dizziness/vertigo patients accompanied with DBN were retrospectively reviewed in the Vertigo Center of our hospital from January 2018 to March 2021. The clinical features of dizziness/vertigo patients accompanied with DBN were reviewed. Comprehensive VNG, bithermal caloric testing, video-head-impulse test (vHIT), vestibular-evoked myogenic potentials (VEMP), head magnetic resonance imaging (MRI), three-dimensional fluidattenuated incersion recovery magnetic resonance imaging (3D-FLAIR MRI) in the inner ear, serum immunology and other examinations were to determine the lesion site, and analyze its possible etiology and mechanism. Results: A total of 54 patients were included. Among them, 70.4% (n = 38) of DBN patients were diagnosed with episodic vestibular syndrome (EVS), 22.2% (n = 12) with chronic vestibular syndrome (CVS), and 7.4% (n = 4) with acute vestibular syndrome (AVS). Among all the patients, 51.9% of DBN patients had clear etiology, with central lesions of 29.6% and peripheral diseases of 22.2%. The most common diseases in DBN patients were cerebellar lesions (13.0%, n = 7) and vestibular migraine (13.0%, n = 7), followed by benign positional paroxysmal vertigo (7.4%, n = 4) and drug-related dizziness/vertigo (5.6%, n = 3). The other 48.1% of the patients had unknown etiology. 53.8% (14/26) of patients with idiopathic DBN had decreased semicircular canal function, with 42.9% (6/14) decreased posterior semicircular canal function. The posterior semicircular canal gain in DBN patients decreased compared to the anterior semicircular canal in the same conjugate plane. Patients with peripheral DBN were more prone to horizontal/torsional nystagmus during positional testing. Conclusion: In our study, DBN patients have a relative decrease in posterior semicircular canal gain, which is possibly a particular result found in a subset of downbeat nystagmus patients. The changes in nystagmus during positional testing may be helpful in distinguishing between peripheral and central causes. [ABSTRACT FROM AUTHOR]

AbstractBackgroundMethodsResultsConclusionsTranscranial direct current stimulation (tDCS) is widely used in motor recovery. Nevertheless, whether tDCS improves motor learning in healthy older adults is still controversial. This review aims to investigate the effectiveness of tDCS on motor learning in healthy elderly individuals.The PubMed, Cochrane Library, Web of Science and Embase databases were initially searched from inception to December 5, 2022. The standard mean difference (SMD) with the corresponding 95% confidence intervals (CIs) were analysed via random-effect models.Compared with the sham group, no significant effects were found regarding improvement in motor learning based on the speed or accuracy of the task and reaction time for the tDCS intervention group. After subgroup analysis, a significant effect was found for improved motor learning based on reaction time in the primary motor cortex (M1)-cerebellar group.This review revealed that tDCS had no significant effect on improving the speed or accuracy of motor learning in healthy elderly adults. However, it has a significant effect on improving the motor learning ability based on the reaction time of the task (mainly referring to the tDCS stimulation position of M1 and cerebellar), although the results have obvious heterogeneity and uncertainty. [ABSTRACT FROM AUTHOR]

Internal models are essential for the production of accurate movements. The accuracy of saccadic eye movements is thought to be mediated by an internal model of oculomotor mechanics encoded in the cerebellum. The cerebellum may also be part of a feedback loop that predicts the displacement of the eyes and compares it to the desired displacement in real time to ensure that saccades land on target. To investigate the role of the cerebellum in these two aspects of saccade production, we delivered saccade-triggered light pulses to channelrhodopsin-2-expressing Purkinje cells in the oculomotor vermis (OMV) of two male macaque monkeys. Light pulses delivered during the acceleration phase of ipsiversive saccades slowed the deceleration phase. The long latency of these effects and their scaling with light pulse duration are consistent with an integration of neural signals at or downstreamof the stimulation site. In contrast, light pulses delivered during contraversive saccades reduced saccade velocity at short latency and were followed by a compensatory reacceleration which caused gaze to land on or near the target. We conclude that the contribution of the OMV to saccade production depends on saccade direction; the ipsilateral OMV is part of a forward model that predicts eye displacement, whereas the contralateral OMV is part of an inverse model that creates the force required to move the eyes with optimal peak velocity for the intended displacement. [ABSTRACT FROM AUTHOR]

Background: Cerebellar superficial siderosis (SS) has been recently reported to be present in about 10% of both hereditary and sporadic cerebral amyloid angiopathy (CAA) on 3T MRI using primarily susceptibility-weighted imaging. Objectives: Our aim was to assess cerebellar SS in sporadic CAA patients using 1.5T T2*-weighted MRI and to evaluate possible underlying mechanisms. Method: We retrospectively screened MRI scans of sporadic probable CAA patients initially presenting with intracerebral hemorrhage-, acute subarachnoid hemorrhage- or cortical SS-related symptoms between September 2009 and January 2022 registered in our stroke database. Patients with familial CAA were excluded. On 1.5T T2*-weighted MRI, cerebellar SS (including kappa statistics for interobserver agreement) was assessed together with typical CAA hemorrhagic features and with the presence of supratentorial macrobleed and cortical SS adjacent to the tentorium cerebelli (TC) and TC hemosiderosis. Results: We screened 151 patients and finally included 111 CAA patients (median age 77) with cerebellar SS observed in 6 (5%) patients. Cerebellar SS presence was associated with a higher number of supratentorial macrobleeds (median n = 3 vs. n = 1, p = 0.0012), presence of supratentorial macrobleed adjacent to the TC (p = 0.002), and TC hemosiderosis (p = 0.005). Conclusions: Cerebellar SS in CAA patients can be identified on 1.5T T2*-weighted imaging. Associated MRI characteristics suggest contamination from supratentorial macrobleeds. [ABSTRACT FROM AUTHOR]

Background Hemangioblastomas pose an inherent surgical risk due to the potential for high intraoperative blood loss, especially in larger tumors. One approach to minimize this risk is to use preoperative embolization. Herein, we present our institutional experience treating large and giant cerebellar hemangioblastomas. Methods We performed a retrospective chart review of 19 patients with cerebellar hemangioblastomas that had a maximal diameter of >3 cm. We performed a literature review and included individual patient-level data that met our >3 cm diameter cerebellar hemangioblastoma inclusion criteria. Results Our cohort consisted of 19 patients that received a total of 20 resections for their cerebellar hemangioblastomas. Preoperative embolization was utilized in eight cases (38.1%). One patient experienced transient neurological complications after embolization (12.5%). Tumors of patients in the embolization group had larger median total, solid, and cystic volumes and were more likely to involve the cerebellopontine angle than those in the non-embolized group. Compared with non-embolized patients, embolized patients had less decrease in their hemoglobin, lower volumes of estimated blood loss, reduced rates of postoperative complications and permanent deficits, and greater instances of neurological improvement. The larger cohort (obtained from the combining our cohort with patients identified during a literature review) consisted of 99 patients with 39 receiving preoperative embolization. Conclusion It is important to examine individual patient characteristics when determining eligibility for preoperative embolization. However, improvements in endovascular techniques have made preoperative embolization a safe and effective procedure with minimal risks that can be performed in many patients. [ABSTRACT FROM AUTHOR]

Cerebellar ischemic stroke (CIS) is a morbid neurological event, with potentially fatal consequences. There is currently no objective standard of care regarding when surgical procedures are required for this entity. We retrospectively reviewed 763 patients with CIS, 247 patients of which had a stroke larger than 1 cm in greatest dimension on cranial imaging. In this subgroup, 11% of patients received ventriculostomy, 12% suboccipital craniectomy, and 9% mechanical endovascular thrombectomy. Various clinical and radiographic variables were examined for relationship to surgical procedures, 30-day mortality rate, and modified Rankin scores. The smallest volume of stroke requiring a surgical procedure was 15.5 mL3 (BrainLab Software). Patients receiving surgical procedures had a higher incidence of multi-territory infarctions, hydrocephalus, cistern compression, 4th ventricular compression, as well as younger age, lower admission GCS, higher admission NIHSS, and higher 30-day mortality/disability. Patients deemed to require surgical procedures for CIS have a higher expected morbidity and mortality than those not requiring surgery. Various clinical and radiographic variables, including stroke volume, can be used to guide selection of patients requiring surgery. [ABSTRACT FROM AUTHOR]

Copyright of Clinical Neuroscience / Ideggyógyászati Szemle is the property of LifeTime Media Kft. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: We aimed to relate clinical measures of disability in chronic cerebellar degeneration to structural whole-brain changes using voxel-based and surface-based morphometry (vbm and sbm). We were particularly interested in remote effects of cerebellar degeneration in the cerebral cortex. Methods: We recruited 30 patients with cerebellar degeneration of different aetiologies (downbeat nystagmus syndrome, DBN n = 14, spinocerebellar ataxia, SCA n = 9, sporadic adult late-onset ataxia, SAOA n = 7). All patients were thoroughly characterised in the motor, cognitive, vestibular and ocular–motor domains. Vbm and sbm were used to evaluate structural differences between cerebellar degeneration patients and a group of healthy age- and gender-matched volunteers. Linear regression models were used to correlate functional measures of disease progression and postural stability with whole brain volumetry. Results: Patients with SCA and SAOA showed widespread volume loss in the cerebellar hemispheres and less prominently in the vermis. Patients with DBN showed a distinct pattern of grey matter volume (GMV) loss that was restricted to the vestibular and ocular–motor representations in lobules IX, X and V–VII. Falls were associated with brainstem white matter volume. VBM and SBM linear regression models revealed associations between severity of ataxic symptoms, cognitive performance and preferred gait velocity. This included extra-cerebellar (sub-)cortical hubs of the motor and locomotion network (putamen, caudate, thalamus, primary motor cortex, prefrontal cortex) and multisensory areas involved in spatial navigation and cognition. Conclusion: Functional disability in multiple domains was associated with structural changes in the cerebral cortex. [ABSTRACT FROM AUTHOR]

Rosette-forming glioneuronal tumors (RGNT) are extremely rare mostly benign tumors of the central nervous system, which are often studied for its histological aspects despite relatively small numbers of clinical especially radiological knowledge. Despite the increasing number of publications on different localizations and treatment protocols, the morphologic and temporal development process of this rare tumor entity is not clear. We were able to coincidentally observe the entire course of the tumor growth of a RGNT on subsequent MRI examinations in a typical case with mild clinical symptoms and no other neurological illnesses, thus possible clinical complications were prevented. [ABSTRACT FROM AUTHOR]

Oligodendroglioma is a rare brain tumor. Although it commonly originates in the cerebral hemisphere in adults, in the pediatric population, the location of oligodendroglioma varies and includes the cerebellum, midbrain, and spinal cord. The MRI characteristic of oligodendroglioma is also different between adults and pediatrics. Oligodendroglioma of >3 cm in pediatrics is associated with a poorer prognosis. Surgery and radiotherapy are the modality of choice for such patients. In this case, we present a 12-year-old girl with huge oligodendroglioma (WHO grade II). MRI showed an isointense-inhomogeneous signal on T1W1 and isointense with some region of hyperintense inhomogeneous on T2W1. After a 26-times-radiotherapy regimen, the patient was followed up for MRI evaluation and which revealed a marked reduction of tumor volume. The patient also reported no symptoms and overall clinical improvement.

This article discusses a case study of a 30-year-old man who experienced tinnitus and reduced word recognition after undergoing embolization for a cerebellar arteriovenous malformation (CAVM). The patient had a bilateral inferior colliculus infarction, which is a rare complication of the procedure. The study highlights the auditory effects of lesions in the inferior colliculus and suggests that the reflux of embolization material may contribute to these complications. The authors recommend caution and precise catheter placement to minimize the risk of such complications. [Extracted from the article]

Background: Friedreich Ataxia is the most common recessive ataxia with only one therapeutic drug approved solely in the United States. Objective: The aim of this work was to investigate whether anodal cerebellar transcranial direct current stimulation (ctDCS) reduces ataxic and cognitive symptoms in individuals with Friedreich's ataxia (FRDA) and to assess the effects of ctDCS on the activity of the secondary somatosensory (SII) cortex. Methods: We performed a single‐blind, randomized, sham‐controlled, crossover trial with anodal ctDCS (5 days/week for 1 week, 20 min/day, density current: 0.057 mA/cm2) in 24 patients with FRDA. Each patient underwent a clinical evaluation (Scale for the Assessment and Rating of Ataxia, composite cerebellar functional severity score, cerebellar cognitive affective syndrome scale) before and after anodal and sham ctDCS. Activity of the SII cortex contralateral to a tactile oddball stimulation of the right index finger was evaluated with brain functional magnetic resonance imaging at baseline and after anodal/sham ctDCS. Results: Anodal ctDCS led to a significant improvement in the Scale for the Assessment and Rating of Ataxia (−6.5%) and in the cerebellar cognitive affective syndrome scale (+11%) compared with sham ctDCS. It also led to a significant reduction in functional magnetic resonance imaging signal at the SII cortex contralateral to tactile stimulation (−26%) compared with sham ctDCS. Conclusions: One week of treatment with anodal ctDCS reduces motor and cognitive symptoms in individuals with FRDA, likely by restoring the neocortical inhibition normally exerted by cerebellar structures. This study provides class I evidence that ctDCS stimulation is effective and safe in FRDA. © 2023 International Parkinson and Movement Disorder Society. [ABSTRACT FROM AUTHOR]

Background and purpose: Differentiating between peripheral and central aetiologies can be challenging in patients with acute vertigo, given substantial symptom overlap. A detailed clinical history and focused physical eye movement examination such as the HINTS eye examination appear to be the most reliable approach to identify acute cerebellar/brainstem stroke, outperforming even acute brain imaging. We have observed, however, that isolated vertigo of central cause may be accompanied by acute truncal ataxia, in the absence of nystagmus. Methods: We explored the frequency of ataxia without concurrent nystagmus in a cross section of patients with acute vertigo who presented to the emergency department at two centres in Argentina (Group A) and the UK (Group B). Patients underwent detailed clinical neuro‐otological assessments (Groups A and B), which included instrumented head impulse testing and oculography (Group B). Results: A total of 71 patients in Group A and 24 patients in Group B were included in this study. We found acute truncal ataxia—without nystagmus—in 15% (n = 14) of our overall cohort. Lesions involved stroke syndromes affecting the posterior inferior cerebellar artery, anterior inferior cerebellar artery, and superior cerebellar artery, thalamic stroke, cerebral hemisphere stroke, multiple sclerosis, and a cerebellar tumour. Additional oculomotor deficits did not reliably identify a central cause in these individuals, even with oculography. Conclusions: We have identified a significant subpopulation of patients with acute vertigo in whom the current standard approaches such as the HINTS examination that focus on oculomotor assessment may not be applicable, highlighting the need for a formal assessment of gait in this setting. [ABSTRACT FROM AUTHOR]

The childhood brain tumour medulloblastoma is typically classified into multiple discrete molecular subgroups with characteristic DNA methylation and expression patterns. Several of these subgroups are used as, or proposed to be, an effective basis for treatment stratification. Here, we highlight the close connection between the findings described in a recent series of studies which, together, strongly imply a continuous association between survival outcome, the transcriptional profile of a Group3/Group4 (i.e. non‐WNT/non‐SHH) medulloblastoma and the specific point during early foetal cerebellar development at which initial pathogenic disruption took place. This has important implications for future efforts to model the disease by incorporating driving molecular features into their specific developmental context. This further suggests that instead of relying upon discrete DNA methylation subgroups, using expression biomarkers as the basis of a continuous risk predictor may produce a more effective risk stratification of patients with Group3/Group4 medulloblastoma. [ABSTRACT FROM AUTHOR]

ObjectiveThe objective of this study was to evaluate the existing Spetzler-Martin (SM), Spetzler-Ponce (SP), and Lawton-Young (LY) grading systems for cerebellar arteriovenous malformations (AVMs) and to propose a new grading system to estimate the risks associated with these lesions.MethodsData for patients with cerebellar AVMs treated microsurgically in two tertiary medical centers were retrospectively reviewed. Data from patients at institution 1 were collected from September 1999 to February 2013, and at institution 2 from October 2008 to October 2015. Patient outcomes were classified as favorable (modified Rankin Scale [mRS] score 0-2) or poor (mRS score 3-6) at the time of discharge. Using chi-square and logistic regression analysis, variables associated with poor outcomes were assigned risk points to design the proposed grading system. The proposed system included neurological status prior to treatment (poor, +2 points), emergency surgery (+1 point), age > 60 years (+1 point), and deep venous drainage (deep, +1 point). Risk point totals of 0-1 comprised grade 1, 2-3 grade 2, and 4-5 grade 3.ResultsA total of 125 cerebellar AVMs of 1328 brain AVMs were reviewed in 125 patients, 120 of which were treated microsurgically and included in the study. With our proposed grading system, we found poor outcomes differed significantly between each grade (p < 0.001), while with the SM, SP, and LY grading systems they did not (p = 0.22, p = 0.25, and p = 1, respectively). Logistic regression revealed grade 2 had 3.3 times the risk of experiencing a poor outcome (p = 0.008), while grade 3 had 9.9 times the risk (p < 0.001). The proposed grading system demonstrated a superior level of predictive accuracy (area under the receiver operating characteristic curve [AUROC] of 0.72) compared with the SM, SP, and LY grading systems (AUROC of 0.61, 0.57, and 0.51, respectively).ConclusionsThe authors propose a novel grading system for cerebellar AVMs based on emergency surgery, venous drainage, preoperative neurological status, and age that provides a superior prognostication power than the formerly proposed SM, SP, and LY grading systems. This grading system is clinically predictive of patient outcomes and can be used to better guide vascular neurosurgeons in clinical decision-making.

Central vertigo is a result of vestibular structure dysfunction in the central nervous system. Currently, misdiagnoses between peripheral and central lesions are frequent, and diagnostic testing costs are high. Identifying the characteristics of these 2 conditions is challenging. We can provide better treatment if we can establish a diagnosis earlier. Cerebral cavernous malformation (CCM) at the cerebellum is a cerebellar lesion that causes symptoms of central vertigo. We report a patient, 20th years old, female, with vertigo for 1 month before being admitted. Vertigo was getting worse, and when the patient arrived at our hospital, vertigo was accompanied by headache, right and left abducens nerve palsy, horizontal nystagmus bidirectional, vertical nystagmus, and weakness on the right side of the body. A brain magnetic resonance imaging (MRI) was performed before surgery and shows a lesion suggestive of CCM at the cerebellum with a hemorrhagic component inside and non-communicating hydrocephalus. There is no vascular malformation based on digital subtraction angiography result. MRI is the most sensitive and specific modality for detecting CCM, whereas cerebral angiography rarely detects this malformation. The patient got surgical treatment, with suboccipital decompression procedures and CCM excision. The histopathological results after surgical treatment revealed a cerebral cavernous malformation. Vertigo, headache, double vision, and weakness on the right side of the body were resolved after surgery.

Introduction: Despite current clinical guidelines recommending suboccipital decompressive craniectomy (SDC) in cerebellar infarction when patients present with neurological deterioration, the precise definition of neurological deterioration remains unclear and accurate timing of SDC can be challenging. The current study aimed at characterizing whether clinical outcomes can be predicted by the GCS score immediately prior to SDC and whether higher GCS scores are associated with better clinical outcomes. Methods: In a single-center, retrospective analysis of 51 patients treated with SDC for space-occupying cerebellar infarction, clinical and imaging data were evaluated at the time points of symptom onset, hospital admission, and preoperatively. Clinical outcomes were measured by the mRS. Preoperative GCS scores were stratified into three groups (GCS, 3-8, 9-11, and 12-15). Univariate and multivariate Cox regression analyses were performed using clinical and radiological parameters as predictors of clinical outcomes. Results: In cox regression analysis GCS scores of 12-15 at surgery were significant predictors of positive clinical outcomes (mRS, 1-2). For GCS scores of 3-8 and 9-11, no significant increase in proportional hazard ratios was observed. Negative clinical outcomes (mRS, 3-6) were associated with infarct volume above 6.0 cm³, tonsillar herniation, brainstem compression, and a preoperative GCS score of 3-8 [HR, 2.386 (CI, 1.160-4.906); p = 0.018]. Conclusion: Our preliminary findings suggest that SDC should be considered in patients with infarct volumes above 6.0 cm³ and with GCS between 12 and 15, as these patients may show better long-term outcomes than those in whom surgery is delayed until a GCS score below 11. [ABSTRACT FROM AUTHOR]

Background: Efavirenz (EFV) is associated with neuropsychiatric symptoms. Severe neurotoxicity has been reported but the clinical phenotype and risk factors are poorly defined. Objectives: To characterise clinical presentations, risk factors and outcomes to help clinicians recognise severe neurotoxicity earlier. Method: The authors retrospectively identified adults with supratherapeutic EFV concentrations ( 4 mg/L) obtained during routine clinical care in Cape Town, South Africa. Clinical and laboratory data at the time of EFV quantification were extracted from medical records. Logistic regression was performed to identify associations with neuropsychiatric symptoms, and with severe neurotoxicity. Results: Eighty one patients were included; 62 with neuropsychiatric manifestations (most frequently ataxia [n = 20] and psychomotor slowing [n = 24]); and 19 with hepatotoxicity. Overall, 28 (34.6%) were male, 49 (60.5%) had concomitant isoniazid exposure, and median EFV concentration was 12.1 mg/L (interquartile range [IQR]: 6.6–20.0). Neuropsychiatric symptoms were associated with longer duration of EFV therapy, adjusted odds ratio (aOR) 1.3/180-day increment (95% confidence interval [CI]: 1.0–1.7); higher EFV concentrations, aOR 1.2/1 mg/L increase (95% CI: 1.0–1.4) and isoniazid exposure, aOR 8.2 (95% CI: 2.5–26.7). Severe neuropsychiatric symptoms occurred in 47 (75%) patients at a median of 5.9 months (IQR: 2.1–40.8) after EFV initiation. Severe symptoms odds were 1.2-fold higher (95% CI: 1.1–1.4) per 1 mg/L increase in EFV concentration. Symptoms resolved completely within 1 month in 25 (76%) patients with severe neurotoxicity who discontinued EFV. Conclusion: A concentration–effect relationship for severe neurotoxicity exists, which occurred late and resolved in most patients after EFV discontinuation. Contribution: The authors highlighted clinical heterogeneity and morbidity of EFV-associated neurotoxicity.

This article discusses a rare genetic prion disease called Gerstmann‐Sträussler‐Scheinker disease (GSS) and its phenotypic variability. The authors describe three cases of early-onset GSS in patients aged 15, 17, and 18, which mimicked an anticipation phenomenon. The study conducted whole exome sequencing and identified a missense variant in the PRNP gene. The article highlights the challenges in predicting the age of onset for genetic prion diseases and the need for further research to understand the underlying mechanisms. [Extracted from the article]

IntroductionDespite current clinical guidelines recommending suboccipital decompressive craniectomy (SDC) in cerebellar infarction when patients present with neurological deterioration, the precise definition of neurological deterioration remains unclear and accurate timing of SDC can be challenging. The current study aimed at characterizing whether clinical outcomes can be predicted by the GCS score immediately prior to SDC and whether higher GCS scores are associated with better clinical outcomes.MethodsIn a single-center, retrospective analysis of 51 patients treated with SDC for space-occupying cerebellar infarction, clinical and imaging data were evaluated at the time points of symptom onset, hospital admission, and preoperatively. Clinical outcomes were measured by the mRS. Preoperative GCS scores were stratified into three groups (GCS, 3–8, 9–11, and 12–15). Univariate and multivariate Cox regression analyses were performed using clinical and radiological parameters as predictors of clinical outcomes.ResultsIn cox regression analysis GCS scores of 12–15 at surgery were significant predictors of positive clinical outcomes (mRS, 1–2). For GCS scores of 3–8 and 9–11, no significant increase in proportional hazard ratios was observed. Negative clinical outcomes (mRS, 3–6) were associated with infarct volume above 6.0 cm3, tonsillar herniation, brainstem compression, and a preoperative GCS score of 3–8 [HR, 2.386 (CI, 1.160–4.906); p = 0.018].ConclusionOur preliminary findings suggest that SDC should be considered in patients with infarct volumes above 6.0 cm3 and with GCS between 12 and 15, as these patients may show better long-term outcomes than those in whom surgery is delayed until a GCS score below 11.

Primary large B-cell lymphoma of immune-privileged sites (IP-LBCL) is a rare malignant hematological neoplasm. Involvement of the cerebellum is even rarer and its diagnosis is often difficult to make due to its non-specific clinical and radiological presentation. Methods: We reported 3 cases of cerebellar IP-LBCL followed at our hospital and reviewed the medical literature to unravel the peculiarities of this poorly studied entity. Outcomes: Analyzing our cases and reviewing the literature, we could collect and study 26 cases of cerebellar IP-LBCL. To the best of our knowledge, this is the largest cohort of such patients currently published. Conclusion: Cerebellar IP-LBCL presents more often in adult females with cerebellum-related focal neurological signs such as ataxia, headache, and nausea. Histological confirmation is mandatory for a correct diagnosis and treatment and all cases feature diffuse large B-cell lymphoma histopathology. Compared to other encephalic IP-LBCL, cerebellar cases seem to include a higher number of cases with germinal center B-cell phenotype and better survival. These differences may be related to a different immune microenvironment and especially immunoregulation that distinguishes the cerebellum from other areas of the CNS. [ABSTRACT FROM AUTHOR]

Background: To report three cases of autoimmune cerebellar ataxia related to anti-delta/notch-like epidermal growth factor-related receptor (Tr/DNER) antibodies. Case presentation: Patients with unknown cerebellar ataxia were screened with autoimmune cerebellar ataxia (ACA)-related antibody panel. The anti-Tr antibody was positive in three female patients in whom the onset ages were 43 years, 35 years and 43 years old. The antibody titres of serum and cerebrospinal fluid were all 1:32. Cerebral ataxia was the most prominent presentation. Mild cerebellar atrophy was found in one of the patients. Immunotherapy was effective in all three patients. Conclusion: The Tr antibody is associated with autoimmune ataxia, and it has been suggested that the anti-Tr antibody should be tested in patients with cerebellar ataxia who are negative for routine ACA antibodies. Early immunotherapy may improve patient prognoses. [ABSTRACT FROM AUTHOR]

Background: Efavirenz (EFV) is associated with neuropsychiatric symptoms. Severe neurotoxicity has been reported but the clinical phenotype and risk factors are poorly defined. Objectives: To characterise clinical presentations, risk factors and outcomes to help clinicians recognise severe neurotoxicity earlier. Method: The authors retrospectively identified adults with supratherapeutic EFV concentrations (> 4 mg/L) obtained during routine clinical care in Cape Town, South Africa. Clinical and laboratory data at the time of EFV quantification were extracted from medical records. Logistic regression was performed to identify associations with neuropsychiatric symptoms, and with severe neurotoxicity. Results: Eighty one patients were included; 62 with neuropsychiatric manifestations (most frequently ataxia [ n = 20] and psychomotor slowing [ n = 24]); and 19 with hepatotoxicity. Overall, 28 (34.6%) were male, 49 (60.5%) had concomitant isoniazid exposure, and median EFV concentration was 12.1 mg/L (interquartile range [IQR]: 6.6–20.0). Neuropsychiatric symptoms were associated with longer duration of EFV therapy, adjusted odds ratio (aOR) 1.3/180-day increment (95% confidence interval [CI]: 1.0–1.7); higher EFV concentrations, aOR 1.2/1 mg/L increase (95% CI: 1.0–1.4) and isoniazid exposure, aOR 8.2 (95% CI: 2.5–26.7). Severe neuropsychiatric symptoms occurred in 47 (75%) patients at a median of 5.9 months (IQR: 2.1–40.8) after EFV initiation. Severe symptoms odds were 1.2-fold higher (95% CI: 1.1–1.4) per 1 mg/L increase in EFV concentration. Symptoms resolved completely within 1 month in 25 (76%) patients with severe neurotoxicity who discontinued EFV. Conclusion: A concentration–effect relationship for severe neurotoxicity exists, which occurred late and resolved in most patients after EFV discontinuation. Contribution: The authors highlighted clinical heterogeneity and morbidity of EFV-associated neurotoxicity. [ABSTRACT FROM AUTHOR]

(1) Objective: Resting-state fMRI studies have indicated that juvenile myoclonic epilepsy (JME) could cause widespread functional connectivity disruptions between the cerebrum and cerebellum. However, the directed influences or effective connectivities (ECs) between these brain regions are poorly understood. In the current study, we aimed to evaluate the ECs between the cerebrum and cerebellum in patients with new-onset JME. (2) Methods: Thirty-four new-onset JME patients and thirty-four age-, sex-, and education-matched healthy controls (HCs) were included in this study. We compared the degree centrality (DC) between the two groups to identify intergroup differences in whole-brain functional connectivity. Then, we used a Granger causality analysis (GCA) to explore JME-caused changes in EC between cerebrum regions and cerebellum regions. Furthermore, we applied a correlation analysis to identify associations between aberrant EC and disease severity in patients with JME. (3) Results: Compared to HCs, patients with JME showed significantly increased DC in the left cerebellum posterior lobe (CePL.L), the right inferior temporal gyrus (ITG.R) and the right superior frontal gyrus (SFG.R), and decreased DC in the left inferior frontal gyrus (IFG.L) and the left superior temporal gyrus (STG.L). The patients also showed unidirectionally increased ECs from cerebellum regions to the cerebrum regions, including from the CePL.L to the right precuneus (PreCU.R), from the left cerebellum anterior lobe (CeAL.L) to the ITG.R, from the right cerebellum posterior lobe (CePL.R) to the IFG.L, and from the left inferior semi-lunar lobule of the cerebellum (CeISL.L) to the SFG.R. Additionally, the EC from the CeISL.L to the SFG.R was negatively correlated with the disease severity. (4) Conclusions: JME patients showed unidirectional EC disruptions from the cerebellum to the cerebrum, and the negative correlation between EC and disease severity provides a new perspective for understanding the cerebro-cerebellar neural circuit mechanisms in JME. [ABSTRACT FROM AUTHOR]

Human cerebellum plays a vital role in motor coordination, regulation of muscle tone and maintaining the equilibrium of the body. It seldom shows anatomical/morphological variations. Herein, we report the presence of a small additional lobe projecting out on the superior surface of the right cerebellar hemisphere in the para-vermal area in an adult male cadaver. There was a notch on the tentorial surface of the occipital lobe of the right cerebral hemisphere, corresponding to the additional lobe of cerebellum. The additional lobe was histologically normal, with no evidence of any tumour cells. Knowledge of this variation is of importance to radiologists, neuroanatomists and neurosurgeons. [ABSTRACT FROM AUTHOR]

We present a case of a 70-year-old male who presented with left-sided weakness and dysarthria. Cranial imaging was suggestive of a cerebellar infarct and the patient was treated with aspirin and clopidogrel. Two months later a fall prompted further cranial imaging, which was concerning for an intracranial mass with vasogenic edema. Computed tomography angiogram (CTA) was negative for vascular lesion. Ultimately, a DSA revealed a Borden III dAVF between the right occipital artery and the posterior cerebellar vein that was treated with endovascular embolization., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Introduction: Biallelic intronic AAGGG repeat expansion in the replication factor C subunit 1 (RFC1) gene was recently identified in two/third of patients with cerebellar ataxia, sensory neuropathy, and bilateral vestibular areflexia syndrome (CANVAS). The phenotypic spectrum has expanded since (i.e., parkinsonism, motor neuron involvement, cognitive decline); no behavioral symptoms have been reported yet. Case report: We report an Italian family that met the diagnostic criteria for CANVAS, and RFC1-expansion was detected in five of seven. All the affected members presented behavioral-psychiatric symptoms (anxiety, panic attacks, alcohol abuse) before the multisystemic RFC1-expansion manifestation. The disease course was progressive, with ataxia and behavioral-cognitive aspects as the most disabling symptoms. Conclusion: These behavioral-cognitive observations may broaden the RFC1-expansion phenotypic spectrum and highlight the importance of investigating the whole non-motor symptoms in ataxic patients. [ABSTRACT FROM AUTHOR]

Myxopapillary ependymomas are a variant of ependymoma located almost exclusively in the conus medullaris-cauda equina-filum terminale region of the spinal cord. Intracranial myxopapillary ependymomas are rare, occurring either through cerebrospinal fluid seeding from the spinal cord or as primary intracranial myxopapillary ependymoma. We present a case of a large myxopapillary ependymoma in the cerebellum of a 33-year-old male, in whom the tumor was found incidentally after presentation following trauma; however, he did endorse a history of migraines and nausea/vomiting. The tumor was stereotactically resected with gross total resection. This may represent possibly the largest intracranial primary myxopapillary ependymoma. Of note, the World Health Organization 2021 classification re-classified myxopapillary ependymoma grading from grade 1 in 2016 classification to grade 2 in 2021 classification, in light of frequent recurrences, and follow-up with surveillance is recommended.