Prevalence, recovery and phenotype of dysphagia in patients with ischaemic cerebellar stroke.

BACKGROUND AND PURPOSE: Swallowing is a complex task, moderated by a sophisticated bilateral network including multiple supratentorial regions, the brainstem and the cerebellum. To date, conflicting data exist about whether focal lesions to the cerebellum are associated with dysphagia. Therefore, the aim of the study was to evaluate dysphagia prevalence, recovery and dysphagia pattern in patients with ischaemic cerebellar stroke. METHODS: A retrospective analysis of patients consecutively admitted to an academic stroke centre with ischaemic stroke found only in the cerebellum was performed. The presence of dysphagia was the primary end-point and was assessed by a speech-language pathologist, according to defined criteria. Dysphagia pattern was evaluated by analysing the videos of the flexible endoscopic evaluation of swallowing. Brain imaging was used to identify lesion size and location associated with dysphagia. RESULTS: Between January 2016 and December 2021, 102 patients (35.3% female) with a mean age of 52.8 ± 17.3 years were included. Thirteen (12.7%) patients presented with dysphagia. The most frequently observed flexible endoscopic evaluation of swallowing phenotype was premature spillage (n = 7; 58.3%), whilst significant residues or aspiration did not occur. One patient died (7.7%); the other patients showed improvement of dysphagia and one patient (7.7%) was discharged with dietary restrictions. CONCLUSIONS: Although the involvement of the cerebellum in deglutition has become increasingly evident, isolated lesions to the cerebellum are less likely to cause clinically relevant and persisting dysphagia compared to other brain regions. The observed dysphagia pattern shows a lack of coordination and control, resulting in premature spillage or fragmented bolus transfer in some patients.

Association Between First-time Neurologic Events and Metronidazole Treatment: A Case-time Control Study.

PURPOSE: Metronidazole, a widely used antimicrobial medication, has been linked to neurologic adverse drug reactions. This study investigates the association between metronidazole use and first-time neurologic events. METHODS: We conducted a case-time-control study using data from the Danish National Patient Register and the National Prescription Register in years 2013 to 2021. Patients with a first-time diagnosis of encephalopathy, cerebellar dysfunction, or peripheral neuropathy were included. Conditional logistic regression analyses were performed to estimate the risk of neurologic events associated with metronidazole use. FINDINGS: Out of 476,066 first-time metronidazole prescriptions, the 100-day cumulative incidence of peripheral neuropathy was 0.016%, and 0.002% for cerebellar dysfunction or encephalopathy. In the case-time control study, we identified 17,667 persons with a first-time neurologic event and were included for the analysis. The estimated odds ratio for the combined neurologic events was 0.98 (95% CI, 0.59-1.64, P = 0.95) with no statistically significant association across different subgroups and time windows. IMPLICATIONS: Our findings suggest that metronidazole-induced neurologic events may be rarer than previously described, and we did not find any consistent or statistically significant association between metronidazole exposure. Nonetheless, clinicians should remain vigilant to potential neurologic risks in patients receiving metronidazole, to ensure its safe and effective use.

Incidence and clinical features of acute multiple small cerebellar infarction.

OBJECTIVE: To investigate the clinical and imaging features and to identify possible etiology of acute multiple small cerebellar infarction (MSCI). METHODS: We retrospectively enrolled 220 patients with acute cerebellar infarction, divided them into MSCI and large cerebellar infarction (LCI) groups, according to the quantity and size of lesions confirmed by MRI analysis. Clinical and imaging features were compared between the two groups to explore the possible etiology and pathogenesis. RESULTS: Among 220 patients, 90 patients presented MSCI symptoms. The proportions of extracerebellar lesions (P = 0.001) and bilateral infarction (P = 0.001) in the MSCI group were higher than those in the LCI group. No significant differences were found in terms of age, gender, and common vascular risk factors between the two groups. The proportions of vertigo and headache in the MSCI group were significantly lower than those in the LCI group (P < 0.000 and 0.034, respectively), and limb weakness was significantly higher (P = 0.039) in the MSCI patients. Moreover, the proportions of nystagmus and ataxia in the MSCI group were significantly lower than those in the LCI group (P < 0.043 and 0.003, respectively). The MSCI group had higher proportions of ACA and MCA stenosis, while the proportion of posterior circulation stenosis was similar between the two groups. Infarctions involving the posterior inferior cerebellar (PICA) region and mixed territories were far more frequent than those involving the anterior inferior cerebellar artery (AICA) region and superior cerebellar artery (SCA) territory (P < 0.05). Large-artery atherosclerosis and multiple plus undetermined etiology were the main etiological factors of MSCI. CONCLUSION: In patients with acute cerebellar infarction, 30% of patients presented with MSCI. MSCI and LCI showed similar vascular risk factors and vascular stenosis in the posterior circulation system. Patients with MSCI should pay more attention to evaluating anterior circulation vessels' anatomy. Large-artery atherosclerosis was the main pathogenesis of acute MSCI. Assessment of cerebral vessels might be critically required in patients with MSCI complicated atrial fibrillation.

A single center retrospective study of paraneoplastic neurological syndromes with positive onconeural antibodies.

Paraneoplastic neurological syndromes (PNS) are rare immune-mediated disorders, and the detection of onconeural antibodies is helpful for PNS diagnosis. The aim of this study was to investigate the clinical characteristics of patients with PNS with positive onconeural antibodies in a single center in Hubei, China. We retrospectively analyzed the clinical characteristics of 54 patients with positive onconeural antibodies from January 2016 to September 2020. Among 780 patients with suspected PNS, 54 (6.9%) had positive onconeural antibodies. Of those 54 patients, 28 (51.8%) were diagnosed with definite PNS and 13 (24.1%) with possible PNS. Eighteen (33.3%) patients were confirmed with cancer. Ten PNS syndromes were detected among the 28 patients with definite PNS, and they had either classical (12/28, 42.8%) or non-classical syndromes (17/28, 60.7%). Peripheral neuropathy (9/28, 32.1%), subacute cerebellar degeneration (4/28, 14.3%), and limbic encephalitis (4/28, 14.3%) were the most common PNS syndromes. The anti-CV2/CRMP5-antibody was observed most frequently. Lung cancer was the most common tumor type. For patients with possible PNS, peripheral neuropathy was the most common PNS syndrome, and the anti-Tr-antibody was the most frequent onconeural antibody. Immunotherapy was effective in treating PNS. The anti-CV2/CRMP5-antibody was the most subsequently observed antibody. The manifestations of PNS are diverse and include peripheral neuropathy, subacute cerebellar degeneration, and limbic encephalitis. In patients with PNS, lung cancer was the most common tumor.

Cerebellar Mutism Syndrome: An Overview of the Pathophysiology in Relation to the Cerebrocerebellar Anatomy, Risk Factors, Potential Treatments, and Outcomes.

Cerebellar mutism syndrome (CMS) is one the most disabling postoperative neurological complications after posterior fossa surgery in children. CMS is characterized by a transient mutism with a typical onset demonstrated within 2 days postoperatively accompanied by associated ataxia, hypotonia, and irritability. Several hypotheses for the anatomical basis of pathophysiology and risk factors have been suggested. However, a definitive theory and treatment protocols have not yet been determined. Animal histological and electrophysiological studies and more recent human imaging studies have demonstrated the existence of a compartmentalized representation of cerebellar function, the understanding of which might provide more information on the pathophysiology. Damage to the dentatothalamocortical pathway and cerebrocerebellar diaschisis have been described as the anatomical substrate to the CMS. The risk factors, which include tumor type, brainstem invasion, tumor localization, tumor size, and vermal splitting technique, have not yet been clearly elucidated. The efficacy of potential pharmacological and speech therapies has been studied in small trials. Long-term motor speech deficits and associated cognitive and behavioral disturbances have now been found to be common among CMS survivors, affecting their development and requiring rehabilitation, leading to significant financial effects on the healthcare system and distress to the family. The aim of the present review was to outline the cerebellar anatomy and function and its connections in relationship to the pathophysiology and to refine the risk factors and treatment strategies for CMS.

A 28-Year-Old Man from India with SARS-Cov-2 and Pulmonary Tuberculosis Co-Infection with Central Nervous System Involvement.

BACKGROUND Tuberculosis (TB) is a great mimic of central nervous system (CNS) tumors. This mimicry may pose a challenge, as the management of both diseases is quite different. Furthermore, the temporal association of initiating treatment affects prognosis. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) mainly infects the pulmonary system. However, in a patient with concomitant pulmonary tuberculosis, it can be a diagnostic challenge. CASE REPORT A 28-year-old man of Indian origin presented with headache and vomiting. He had a brain mass on imaging suggestive of a glioma. He also had lung infiltrates and was diagnosed with a co-infection by SARS-CoV-2, by a reverse-transcription polymerase chain reaction (RT-PCR) using the GeneXpert system. The mass was excised and was found to be a tuberculoma, diagnosed by Xpert MTB. He received first-line anti-TB and treatment for COVID-19 pneumonia based on local guidelines. CONCLUSIONS This report highlights that COVID-19 can co-exist with other infectious diseases, such as TB. A high degree of clinical suspicion is required to detect TB with atypical presentation. A co-infection of pulmonary and CNS TB with COVID-19 can present a diagnostic challenge, and appropriate patient management relies on an accurate and rapid diagnosis. Surgery may be necessary if there are compressive signs and symptoms secondary to CNS TB. A diagnosis of COVID-19 should not delay urgent surgeries. Further studies are needed to understand the effects of COVID-19 on the clinical course of TB.

Prospective investigation of cerebellar cognitive affective syndrome in a previously non-demented population of acute cerebellar stroke.

OBJECTIVE: In this prospective study, we aimed to investigate the presence and evolution of cerebellar cognitive affective syndrome in a cohort of isolated cerebellar stroke with no known cognitive or psychiatric impairment. We tried to distinguish the unconfounded effect of cerebellar lesions on neuropsychological processing. METHODS: After a meticulous exclusion procedure based on possible confounders, we recruited 14 patients and 13 age-matched healthy controls to the study, prospectively. All of the patients had a detailed initial neuropsychological assessment at the first week and a follow-up assessment at the 4th month after stroke. RESULTS: The prevalence of cognitive or behavioral-affective abnormalities in our cohort were 86% and 64% respectively. The patients exhibited mild and transient affective-behavioral abnormalities except for depressive symptoms that persisted in the subacute stage. They scored lower in general cognitive performance as revealed by mini mental test (p=0.001). Memory, executive functions, attention and working memory, central processing speed, and linguistic abilities were impaired (p<0.001; p=0.001; p=0.007; p=0.05; p<0.001 respectively). Improvement was evident only in memory domain of the cognitive functions in the subacute stage. Cognitive impairment was more likely with a medial or posterolateral infarct (p=0.014). Behavioral-affective abnormalities were not associated with a specific location in our cohort. Age seemed to negatively correlate with the recovery in general cognitive performance on the follow-up. CONCLUSIONS: These findings show that acute denervation of cerebellocortical projections leads to mild affective-behavioral abnormalities, and full-blown cerebellar cognitive affective syndrome is rare. However, cognition was significantly affected after an acute cerebellar infarct even in a previously healthy, non-demented pure population.

Risk Factors of Cerebellar Microembolic Infarctions After Carotid Artery Stenting.

BACKGROUND: This study analyzes the incidence of microembolic infarctions (MIs) in the cerebellum after carotid artery stenting (CAS) to determine the risk factors. METHODS: From 2012 to 2019, 162 CASs in 155 patients were performed at our hospital. Fifty-seven patients (35.7%) showing new MIs on diffusion-weighted imaging after CAS were enrolled. Patients were assigned to either the cerebellar group (n = 14, 8.8%) if their MIs were in the cerebellum and/or cerebrum or the cerebral group (n = 43, 26.9%) if their MIs were only in the cerebrum. Patient characteristics, anatomic features, and clinical data were retrospectively compared between the 2 groups. RESULTS: Advanced age, right-sided carotid stenosis, severe calcification of aortic arch and brachiocephalic trunk, and vertebral artery narrowing with intraprocedural hemodynamic depression (IHD) significantly increased the development of cerebellar MIs. On multivariate analysis, advanced age, right-sided carotid stenosis, and vertebral artery narrowing with IHD were independent predictors of developing new cerebellar MIs. Cerebellar MIs after CAS were not uncommon. CONCLUSIONS: Catheter maneuvering in the aortic arch or the brachiocephalic trunk could be the main cause of thromboemboli in cerebellar MIs. Careful attention should be paid to catheter maneuvering, especially in older patients with right-sided carotid lesions. In addition, cerebellar hypoperfusion caused by vertebral artery narrowing with IHD might reduce washout of debris, a cause of cerebellar MIs.

Multidisciplinary Treatments of True Posterior Inferior Cerebellar Artery Aneurysms: Single-Center Retrospective Study and Treatment Algorithm.

BACKGROUND: True posterior inferior cerebellar artery (PICA) aneurysms outside the vertebral artery-PICA region are rare, with approximately 30 cases reported in just a few papers; no treatment paradigm has been advocated. The objective of this study was to present detailed clinical features and outcomes for several treatments for true PICA aneurysms and suggest an algorithm for treatment strategies. METHODS: We retrospectively analyzed outcomes of patients treated for PICA aneurysms with microsurgical and endovascular treatments. We also investigated the influence of several factors on the modified Rankin Scale score. RESULTS: Cases with PICA aneurysms (n = 36) outside the vertebral artery-PICA region were identified angiographically. Aneurysm locations included anterior medullary (n = 7), lateral medullary (n = 10), tonsillomedullary (n = 4), telovelotonsillar (n = 12), and cortical (n = 3) segments of the PICA. Aneurysm morphology was as follows: dissecting: 22; fusiform: 6; and saccular: 8. On multivariate analysis, age (P = 0.028) and lack of vermian infarction (P =0.037) were associated with a significantly better prognosis. Prognosis was not significantly different for the 5 aneurysm locations and among the 4 treatment groups: clipping/coiling, trapping/parent artery occlusion, trapping/parent artery occlusion + bypass, and observation including external ventricular drainage. CONCLUSIONS: This study suggests that factors associated with significantly better prognosis include age, clip/coil treatments, and no vermian infarction complication. A treatment algorithm for true PICA aneurysms was supported according to pretreatment H and K grade, PICA segments, aneurysm morphology, and 3 types of ischemia linked to the brainstem, cerebellar hemisphere, or vermis.

The Cerebellar Cognitive Affective/Schmahmann Syndrome: a Task Force Paper.

Sporadically advocated over the last two centuries, a cerebellar role in cognition and affect has been rigorously established in the past few decades. In the clinical domain, such progress is epitomized by the "cerebellar cognitive affective syndrome" ("CCAS") or "Schmahmann syndrome." Introduced in the late 1990s, CCAS reflects a constellation of cerebellar-induced sequelae, comprising deficits in executive function, visuospatial cognition, emotion-affect, and language, over and above speech. The CCAS thus offers excellent grounds to investigate the functional topography of the cerebellum, and, ultimately, illustrate the precise mechanisms by which the cerebellum modulates cognition and affect. The primary objective of this task force paper is thus to stimulate further research in this area. After providing an up-to-date overview of the fundamental findings on cerebellar neurocognition, the paper substantiates the concept of CCAS with recent evidence from different scientific angles, promotes awareness of the CCAS as a clinical entity, and examines our current insight into the therapeutic options available. The paper finally identifies topics of divergence and outstanding questions for further research.

Cerebellar mutism syndrome: current approaches to minimize risk for CMS.

PURPOSE: Cerebellar mutism syndrome (CMS) is a serious source of morbidity following posterior fossa surgery in the pediatric population. However, methods for effectively decreasing its incidence and impact remain unclear. It is our aim to examine the impact of adjusting surgical factors, namely the use of a telovelar approach and avoidance of cavitronic ultrasonic aspirator, on the incidence of CMS in our population as well as outlining potential pre-, intra-, and postoperative factors that may contribute to its development. METHODS: Retrospective review was performed to identify patients undergoing posterior fossa surgery for resection of a medulloblastoma. Demographic, surgical, and postoperative data were collected. These data were analyzed for possible correlations to the risk of developing CMS via univariate analysis. For factors found to be significant, a multivariate analysis was performed to assess their independence. RESULTS: Seven of 65 patients (10.8%) developed CMS postoperatively. Factors found to be significantly associated with a higher risk of CMS were the degree of retraction utilized during the procedure (p = 0.0000) and incision of the vermis (p = 0.0294). Although they did not reach the threshold of statistical significance, tumor vascularity (p = 0.19), adoption of a transvermian approach (p = 0.19), and lack of intraoperative imaging (p = 0.17) exhibited strongly suggestive trends towards a correlation with CMS. DISCUSSION: In an effort to reduce the incidence and severity of CMS in our population, our institution adopted surgical practices that minimize tissue trauma and mitigate postoperative edema. This included the use of a telovelar over a transvermian approach to obviate the need for vermian incision, avoidance of the CUSA, and minimization of heavy retraction during surgery. This was successful in reducing the incidence of CMS from 39% in our medulloblastoma patients to 10.8%. The development of CMS after posterior fossa surgery appears to be a "two-hit" phenomenon requiring a combination of existing predisposition, surgical injury, and postoperative exacerbation. Therefore, it is critical to identify the factors involved at each stage and investigate treatments to target them appropriately.

Cerebellar Syndrome Associated with Thyroid Disorders.

Thyroid disorders, including hypothyroidism, hyperthyroidism and Hashimoto encephalopathy, are considered the most common cause of cerebellar dysfunction due to hormonal imbalance. Typically, cerebellar impairment occurs in the course of hypothyroidism and Hashimoto encephalopathy. Information about demographic, clinical and laboratory features of cerebellar disease associated with thyroid disorders is poor. Our review of the literature (1965 to 2018) identified 28 cases associated with hypothyroidism and 37 cases associated with Hashimoto encephalitis. Both patients with hypothyroidism and Hashimoto encephalopathy presented with signs of ataxia that were similarly distributed in the two groups and were mostly predictive of vermis involvement and frequent impairment of cerebellar hemispheres. Additional neurological signs, like dystonia, psychiatric symptoms, ocular disturbances and myoclonus, could be found in the Hashimoto encephalopathy group alone. When present, atrophy of vermis and often of both cerebellar hemispheres was the main imaging abnormality in both hypothyroidism and Hashimoto encephalopathy. Anti-thyroid antibodies could be detected in three quarters of patients with hypothyroidism and in all patients with Hashimoto encephalopathy. In the patients with hypothyroidism, thyroid replacement therapy yielded complete or partial remission of ataxia. In the Hashimoto encephalopathy group, immunosuppressive treatment provided complete remission of ataxia in about 60% of patients, partial remission in the remaining cases. Owing to the treatable nature of the condition and the high prevalence of thyroid disease among general population, cerebellar syndrome associated with thyroid disorders should be considered an important clinical entity. Information from this review will hopefully stimulate and strengthen awareness of thyroid-associated ataxia among clinicians.

The Cerebellar Cognitive Affective Syndrome-a Meta-analysis.

The cerebellar cognitive affective syndrome (CCAS) was first described by Schmahmann and Sherman in 1998. Despite their clear depiction of the syndrome, it is our experience that the CCAS has not yet found solid ground as a disease entity in routine clinical practice. This made us question the dimension of the CCAS in cerebellar patients. We performed a systematic review of the literature according to the PRISMA guidelines, in order to answer the question whether patients with acquired isolated cerebellar lesions perform significantly worse on neuropsychological testing compared to healthy controls. Studies were selected based on the predefined eligibility criteria and quality assessment. The systematic search resulted in ten studies, mainly observational cohorts consecutively including adult patients with isolated cerebellar lesions. Patients were compared to healthy controls, and neuropsychological investigation was done within one year of diagnosis. Meta-analysis of the twelve tests that were done in two or more studies showed that cerebellar patients perform significantly worse on Phonemic Fluency, Semantic Fluency, Stroop Test (naming, reading and interference), Block Design test and WMS-R visual memory. Cerebellar patients have significant and relevant deficits in the visuospatial, language and executive function domain. This meta-analysis therefore emphasizes the importance of the cerebellar cognitive affective syndrome as described by Schmahmann and Sherman.

Cerebral small vessel disease in patients with spontaneous cerebellar hemorrhage.

BACKGROUND: Spontaneous cerebellar-intracerebral hemorrhage (ICH) can be associated with both cerebral amyloid angiopathy (CAA) and hypertensive small vessel disease (HTN-SVD, i.e. arteriolosclerosis). To better understand the underlying microangiopathy of cerebellar-ICH, we aimed to evaluate the spatial distribution of supratentorial cerebral microbleeds (CMBs) and neuropathologic profiles in these patients. METHODS: We enrolled consecutive cerebellar-ICH patients. Clinical variables and MRI markers specific for CAA and HTN-SVD were assessed. Patients were classified into categories according to the topography (strictly-lobar, strictly-deep, and mixed) of supratentorial CMBs and comparisons were performed. Available neuropathological material was reviewed to evaluate the presence and severity of arteriolosclerosis and CAA. RESULTS: Ninety-eight cerebellar-ICH patients were enrolled. Fifty patients (51%) had at least one supratentorial CMB. Twelve patients (12%) had strictly lobar-CMBs, 12 patients (12%) showed strictly deep-CMBs and mixed-CMBs (lobar and deep CMBs) were present in 26 cerebellar-ICH patients (27%). In multivariable analysis, cerebellar-ICH patients with mixed-CMBs were associated with higher prevalence of hypertension (OR 4.9, 95% confidence interval [CI] 1.2-20, p = 0.017) but with lower prevalence of severe centrum-semiovale enlarged perivascular spaces (OR 0.2, CI 0.05-0.8, p = 0.024) when compared to cerebellar-ICH patients with strictly lobar-CMBs. Vascular risk factors and neuroimaging characteristics were similar between strictly deep-CMBs and mixed-CMBs. Six patients had available neuropathological material for analyses and they all showed some degree of arteriolosclerosis. CONCLUSIONS: Cerebellar-ICH patients frequently show supratentorial CMBs. The mixed-CMBs pattern appears to be the most common. Our radiological and pathological results suggest that the majority of cerebellar-ICH patients harbor HTN-SVD as dominant microangiopathy.

Fourth ventricle index: sonographic marker for severe fetal vermian dysgenesis/agenesis.

OBJECTIVE: Prenatal diagnosis of midbrain-hindbrain (MB-HB) malformations relies primarily on abnormal size and shape of the cerebellum and retrocerebellar space, particularly 'open fourth ventricle' (4V), the most common indicator of MB-HB malformations. The aim of this study was to present the fourth ventricle index (4VI), and to evaluate its role as a marker for severe vermian dysgenesis/agenesis in cases without open 4V. METHODS: This was a prospective cross-sectional study of patients with singleton low-risk pregnancy at 14 + 1 to 36 + 6 gestational weeks presenting between May 2016 and November 2017 for routine ultrasound examination. Axial images of the fetal 4V were obtained and the 4VI was calculated as the ratio between the laterolateral and the anteroposterior diameters. Reference ranges were constructed and retrospectively collected values from 44 fetuses with confirmed anomalies involving severe vermian dysgenesis/agenesis (Joubert syndrome and related disorders, rhombencephalosynapsis, cobblestone malformations and cerebellar hypoplasia) but without open 4V were compared with the normal values. RESULTS: In total, 384 healthy fetuses were enrolled into the study, from which reference ranges were produced, and 44 cases were collected retrospectively. The 4VI in the normal fetuses was always > 1. In affected fetuses, it was always below mean -2 SD and < 1. CONCLUSIONS: The 4VI is a sonographic marker for severe fetal vermian dysgenesis/agenesis in the absence of an open 4V. It may be incorporated easily into the routine brain scan; 4VI < 1 indicates a need for dedicated fetal neuroimaging for diagnosis and prenatal counseling. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Behavioral Deficits at 18-22 Months of Age Are Associated with Early Cerebellar Injury and Cognitive and Language Performance in Children Born Extremely Preterm.

OBJECTIVE: To investigate associations in toddlers born extremely preterm (<28 weeks) between neonatal neuroimaging and 18- to 22-month developmental and behavioral outcomes. STUDY DESIGN: Cohort analysis from the Eunice Kennedy Shriver National Institute of Child Health and Human Development Neonatal Research Network Surfactant Positive Airway Pressure and Pulse Oximetry Trial Neuroimaging and Neurodevelopmental Outcomes Study of infants born extremely preterm. Subjects underwent cranial ultrasonography and near-term magnetic resonance imaging (MRI). At 18-22 months of corrected age, the assessment included the Brief Infant Toddler Social Emotional Assessment (BITSEA) Problem and Competence Scale scores and the Bayley Scales of Infant Development, Third Edition (Bayley-III). The BITSEA Problem Scale assesses dysregulation; the Competence Scale assesses social-emotional competence. We examined associations of Problem and Competence scores and positive screen rates with cranial ultrasonography and near-term MRI. Mean BITSEA and Bayley-III scores were compared using ANOVA and positive screen rates with the χ2 test. We computed correlations between BITSEA and Bayley-III scores. RESULTS: Of the 397 children, positive BITSEA screens were found in 34% for the Problem score and 26% for the Competence score. Presence of lesions on near-term MRI that included cerebellar lesions were significantly associated with lower BITSEA Competence but not with Problem scores; Competence scores were inversely related to the presence/significance of lesions. Positive screens on Competence scores and on both Competence and Problem scores were significantly associated with Bayley-III cognitive and language scores <85 (P < .001). CONCLUSIONS: Social-emotional competence contributes to deficits in cognitive and language development. Presence of injury on near-term MRI that includes cerebellar lesions is associated with later social-emotional competence and may be a useful predictor to guide early assessment and intervention. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00063063 and NCT00233324.

Persistent geotropic positional nystagmus in unilateral cerebellar lesions.

OBJECTIVE: To determine the prevalence of central lesions in persistent geotropic positional nystagmus, and characteristics and anatomical substrates of the nystagmus in cerebellar lesions. METHODS: We prospectively recruited 58 patients with persistent geotropic positional nystagmus at the Dizziness Clinic of Pusan National University Hospital. Seven patients with unilateral cerebellar lesions were subjected to analysis of clinical characteristics, oculographic data, and MRI lesions. For comparison, we studied 37 cases of peripheral persistent geotropic positional nystagmus. RESULTS: The prevalence of central lesions in persistent geotropic positional nystagmus was 12% (7/58). Persistent geotropic positional nystagmus in cerebellar lesions was mostly asymmetrical. Horizontal nystagmus changed in direction during the bow-and-lean test with null positions. All patients showed impaired horizontal smooth pursuit bilaterally, and 3 of them also had positional downbeat nystagmus. The peak intensity and asymmetry of persistent geotropic positional nystagmus did not differ between central and peripheral groups (p > 0.05), while there was a difference in the maxima. Lesion overlays revealed that damage to the cerebellar tonsil was responsible for the generation of persistent geotropic positional nystagmus. CONCLUSION: Although persistent geotropic positional nystagmus in cerebellar lesions shares the characteristics of nystagmus measures with peripheral cases, accompanying central oculomotor signs can aid in differentiation. In tonsillar lesions, compensatory rotational feedback due to erroneous estimation of the direction of gravity may generate constant horizontal geotropic positional nystagmus.

Development of a pre-operative scoring system for predicting risk of post-operative paediatric cerebellar mutism syndrome.

BACKGROUND: Despite previous identification of pre-operative clinical and radiological predictors of post-operative paediatric cerebellar mutism syndrome (CMS), a unifying pre-operative risk stratification model for use during surgical consent is currently lacking. The aim of the project is to develop a simple imaging-based pre-operative risk scoring scheme to stratify patients in terms of post-operative CMS risk. METHODS: Pre-operative radiological features were recorded for a retrospectively assembled cohort of 89 posterior fossa tumour patients from two major UK treatment centers (age 2-23yrs; gender 28 M, 61 F; diagnosis: 38 pilocytic astrocytoma, 32 medulloblastoma, 12 ependymoma, 1 high grade glioma, 1 pilomyxoid astrocytoma, 1 atypical teratoid rhabdoid tumour, 1 hemangioma, 1 neurilemmoma, 2 oligodendroglioma). Twenty-six (29%) developed post-operative CMS. Based upon results from univariate analysis and C4.5 decision tree, stepwise logistic regression was used to develop the optimal model and generate risk scores. RESULTS: Univariate analysis identified five significant risk factors and C4.5 decision tree analysis identified six predictors. Variables included in the final model are MRI primary location, bilateral middle cerebellar peduncle involvement (invasion and/or compression), dentate nucleus invasion and age at imaging >12.4 years. This model has an accuracy of 88.8% (79/89). Using risk score cut-off of 203 and 238, respectively, allowed discrimination into low (38/89, predicted CMS probability <3%), intermediate (17/89, predicted CMS probability 3-52%) and high-risk (34/89, predicted CMS probability ≥52%). CONCLUSIONS: A risk stratification model for post-operative paediatric CMS could flag patients at increased or reduced risk pre-operatively which may influence strategies for surgical treatment of cerebellar tumours. Following future testing and prospective validation, this risk scoring scheme will be proposed for use during the surgical consenting process.

Epidemiology of Cerebellar Diseases and Therapeutic Approaches.

Diseases involving the cerebellum occur relatively commonly in children and adults around the globe. Many factors influence their epidemiology including geography, ethnicity, consanguinity, and the methodology used to ascertain patients. In addition, reliable epidemiological data rely heavily on accurate disease classification. Continuous advances in genetic research and neuroimaging modalities have resulted in improved understanding of cerebellar diseases and have led to several revisions in their classification. Recent global epidemiological studies on ataxia reported an estimated overall prevalence rate of 26/100,000 in children, a prevalence rate of dominant hereditary cerebellar ataxia of 2.7/100,000, and a prevalence rate of recessive hereditary cerebellar ataxia of 3.3/100,000. The management of cerebellar diseases is multidisciplinary and multimodal. General supportive and symptomatic therapies should be initiated. Genetic counseling should be offered, where appropriate. Few drugs, specific motor rehabilitation programs, and noninvasive cerebellar stimulation for the treatment of ataxia have been developed and seem to show early promise, but more studies are needed to replicate and fine-tune their benefits further. Some disease-specific treatments are available. For example, acetazolamide or 4-aminopyridine for patients with episodic ataxia type 2 and vitamin E for patients with ataxia caused by vitamin E deficiency.