Cerebellar Dysfunction in Multiple Sclerosis: Considerations for Research and Rehabilitation Therapy.

Introduction. Cerebellar pathology is common among persons with multiple sclerosis (PwMS). The cerebellum is well recognized for its role in motor control and motor learning and cerebellar pathology in multiple sclerosis is associated with enhanced motor impairment and disability progression. The Problem. To mitigate motor disability progression, PwMS are commonly prescribed exercise and task-specific rehabilitation training. Yet, whether cerebellar dysfunction differentially affects rehabilitation outcomes in this population remains unknown. Furthermore, we lack rehabilitation interventions targeting cerebellar dysfunction. The Solution. Here, we summarize the current understanding of the impact of cerebellar dysfunction on motor control, motor training, and rehabilitation in persons with multiple sclerosis. Recommendations. Additionally, we highlight critical knowledge gaps and propose that these guide future research studying cerebellar dysfunction in persons with multiple sclerosis.

Possible affective cognitive cerebellar syndrome in a young patient with COVID-19 CNS vasculopathy and stroke.

Early case series suggest that about one-third of patients with COVID-19 present with neurological manifestations, including cerebrovascular disease, reported in 2%-6% of hospitalised patients. These are generally older patients with severe infection and comorbidities. Here we discuss the case of a previously fit and well 39-year-old man who presented with fever and respiratory symptoms, evolving in pneumonia with hypoxia but only requiring continuous positive airway pressure. After resolution of the respiratory disease, the patient developed focal neurology and was found to have bilateral occipital, thalamic and cerebellar infarcts. A diagnosis of COVID-19 central nervous system vasculopathy was made. He developed a florid neuropsychiatric syndrome, including paranoia, irritability, aggression and disinhibition, requiring treatment with antipsychotics and transfer to neurorehabilitation. Neuropsychometry revealed a wide range of cognitive deficits. The rapid evolution of the illness was matched by fast resolution of the neuropsychiatric picture with mild residual cognitive impairment.

Perioperative Assessment of Cerebellar Masses and the Potential for Cerebellar Cognitive Affective Syndrome.

The cerebellum was long perceived to be a region of limited importance with primary functions in the regulation of motor control. A degree of its functional topography in motor modulation has been traditionally appreciated. However, an evolving body of evidence supports its role in a range of cognitive processes, including executive decision making, language, emotional processing, and working memory. To this end, numerous studies of cerebellar stroke syndromes as well as investigations with functional magnetic resonance imaging and diffusion tensor imaging have given clinicians a better model of the functional topography within the cerebellum and the essential lanes of communication with the cerebrum. With this deeper understanding, neurosurgeons should integrate these domains into the perioperative evaluation and postoperative rehabilitation of patients with cerebellar tumors. This review aims to discuss these understandings and identify valuable tools for implementation into clinical practice.

Investigating the Clinical Significance and Research Discrepancies of Balance Training in Degenerative Cerebellar Disease: A Systematic Review.

OBJECTIVE: The aims of this study were to understand the clinical significance of balance training in degenerative cerebellar disease and to analyze inconsistencies among published data. DESIGN: Five databases were searched from inception to October 8, 2019. Cochrane guidelines informed review methods, and Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were followed. The Australian National Health and Medical Research Council Evidence Hierarchy, PEDro scale, and Joanna Briggs Institute Critical Appraisal Tools were used to evaluate methodological quality. Outcome measures examined included ataxia severity, gait speed, and balance. RESULTS: Fourteen articles were identified that met inclusion criteria. The quality of evidence was moderate to high, with recent articles being of higher quality. Nine of 12 articles showed statistical improvements in ataxia severity (reduction ranging from 1.4 to 2.8 in the Scale for the Assessment and Rating of Ataxia points), three of eight showed statistical improvements in gait speed (average increase of 0.1 m/sec), and six of nine showed improvements in balance measures (average increase of 1.75 in Berg Balance Scale and 1.5 in Dynamic Gait Index). CONCLUSION: Most studies showed statistical and clinically significant ataxia severity improvements in subjects who performed balance training. The amount of balance challenge and frequency of training were important factors in determining the extent of training benefit. Gait speed may also improve if walking exercises are included in the balance training, but more studies need to be conducted. Balance measures statistically improved with training, but these improvements did not meet criteria for clinical significance. TO CLAIM CME CREDITS: Complete the self-assessment activity and evaluation online at http://www.physiatry.org/JournalCME CME OBJECTIVES: Upon completion of this article, the reader should be able to: (1) Describe the cause(s) of discrepancies in the literature regarding the benefits of balance training in degenerative cerebellar disease; (2) Determine if benefits from balance training are clinically meaningful for individuals with cerebellar degeneration; and (3) Understand the best practices gleaned from the current literature regarding balance training for these diseases. LEVEL: Advanced ACCREDITATION: The Association of Academic Physiatrists is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.The Association of Academic Physiatrists designates this Journal-based CME activity for a maximum of 1.0 AMA PRA Category 1 Credit(s)™. Physicians should only claim credit commensurate with the extent of their participation in the activity.

Phase I randomized single-blinded controlled study investigating the potential benefit of aerobic exercise in degenerative cerebellar disease.

OBJECTIVES: To investigate whether people with cerebellar degeneration can perform rigorous aerobic exercise and to assess the clinical impact of training. DESIGN: Randomized single-blinded controlled, feasibility study comparing aerobic training to no training. SETTING: Home intervention, assessments conducted at an academic medical center. SUBJECTS: Twenty individuals with cerebellar degeneration caused by a range of genetic disorders. INTERVENTION: Aerobic training consisted of four weeks of stationary bicycle training, five times per week for 30-minute sessions. Intensity ranged from 65% to 80% of the participant's maximal heart rate determined during cardiopulmonary exercise testing. MAIN MEASURES: Primary outcome measure was change in the Scale for the Assessment and Rating of Ataxia scores. Recruitment rate, adherence, drop-out, and adverse events were also determined. The treatment was considered technically feasible if participants achieved target training frequency, duration, and intensity. RESULTS: The 20 participants mean age was 50 years (standard deviation 15.65 years) and average Scale for the Assessment and Rating of Ataxia score was 9.6 (standard deviation 3.13). Ten participants were randomized to aerobic training and 10 to no training. Seven participants in the aerobic group attained target training duration, frequency, and intensity. There was a mean reduction in ataxia severity of 2.1 points (standard deviation 1.26) with four weeks of aerobic training, whereas ataxia severity increased by 0.3 (standard deviation 0.62) in the control group over the same period. Walking speed, balance measures, and fitness also improved in individuals who performed aerobic exercise. CONCLUSIONS: Rigorous aerobic training is feasible in people with cerebellar degeneration. Improvements in ataxia, balance, and gait are promising.

Virtual Reality Social Prediction Improvement and Rehabilitation Intensive Training (VR-SPIRIT) for paediatric patients with congenital cerebellar diseases: study protocol of a randomised controlled trial.

BACKGROUND: Patients with cerebellar malformations exhibit not only movement problems, but also important deficits in social cognition. Thus, rehabilitation approaches should not only involve the recovery of motor function but also of higher-order abilities such as processing of social stimuli. In keeping with the general role of the cerebellum in anticipating and predicting events, we used a VR-based rehabilitation system to implement a social cognition intensive training specifically tailored to improve predictive abilities in social scenarios (VR-Spirit). METHODS/DESIGN: The study is an interventional randomised controlled trial that aims to recruit 42 children, adolescents and young adults with congenital cerebellar malformations, randomly allocated to the experimental group or the active control group. The experimental group is administered the VR-Spirit, requiring the participants to compete with different avatars in the reaching of recreational equipment and implicitly prompting them to form expectations about their playing preference. The active control group participates in a VR-training with standard games currently adopted for motor rehabilitation. Both trainings are composed by eight 45-min sessions and are administered in the GRAIL VR laboratory (Motekforce Link, Netherlands), an integrated platform that allows patients to move in natural and attractive VR environments. An evaluation session in VR with the same paradigm used in the VR-Spirit but implemented in a different scenario is administered at the beginning (T0) of the two trainings (T1) and at the end (T2). Moreover, a battery of neurocognitive tests spanning different domains is administered to all participants at T0, T2 and in a follow-up session after 2 months from the end of the two trainings (T3). DISCUSSION: This study offers a novel approach for rehabilitation based on specific neural mechanisms of the cerebellum. We aim to investigate the feasibility and efficacy of a new, intensive, social cognition training in a sample of Italian patients aged 7-25 years with congenital cerebellar malformations. We expect that VR-Spirit could enhance social prediction ability and indirectly improve cognitive performance in diverse domains. Moreover, through the comparison with a VR-active control training we aim to verify the specificity of VR-Spirit in improving social perception skills. TRIAL REGISTRATION: ISRCTN, ID: ISRCTN 22332873. Retrospectively registered on 12 March 2018.

Rehabilitation for a complex patient following cerebellar hemorrhage and obstructive hydrocephalus: A case report.

BACKGROUND AND PURPOSE: Inpatient rehabilitation facilities play an integral role in patient progress post-stroke. Cerebellar hemorrhages are an infrequent type of stroke and are therefore less discussed in the literature; however, inpatient rehabilitation continues to be an integral part of patient recovery. The purpose of this case report is to discuss the physical therapy interventions, challenges, and successes for a complex patient with a large cerebellar hemorrhage with obstructive hydrocephalus. Case Description: The patient is a 32-year-old male admitted to an inpatient rehabilitation facility. Prior to admission, the patient spent 1 month at a local hospital following a complicated recovery status post cerebellar hemorrhage with obstructive hydrocephalus. Interventions: The patient participated in at least 3 hours of combined therapy a day, split into 30- and 60-min sessions, and divided between physical, occupational, and speech therapy. Physical therapy interventions focused on various gait and coordination activities. Outcomes: The patient spent 47 days in an inpatient rehabilitation facility and demonstrated improvements in all aspects of the Functional Independence Measure®. The patient improved from an 18 to a 90 on the total FIM® score, allowing the patient to ultimately be discharged home with family able to provide 24/7 supervision.

Rare case of paraneoplastic cerebellar degeneration secondary to high-grade serous carcinoma of tubo-ovarian origin.

This case describes a 69-year-old woman, who presented with rapidly progressive cerebellar symptoms and unintentional weight loss. Full neurological assessment excluded space-occupying lesions, vascular accidents and infection. Surprisingly, a chest, abdomen and pelvis CT showed a left hemipelvis mass, which was subsequently biopsied. A high-grade serous carcinoma of tubo-ovarian origin was found, diagnosing paraneoplastic cerebellar degeneration (PCD) secondary to this. The exact mechanism is not known, but is thought to be immune-mediated. In cases of PCD, after cancer treatment, the neurological disability stabilises to a severe level and will unfortunately be lifelong. Our patient continues to make great progress with intensive rehabilitation for her ongoing balance issues. Early recognition of PCD can lead to a prompt diagnosis of the underlying malignancy and hence subsequent management. This can at least limit the extent of the neurological disability of the disease and increase the survival rate from cancer.

Cerebelitis aguda fulminante, ¿una entidad infradiagnosticada? / Fulminant acute cerebellitis: An under-diagnosed condition?

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The cooking therapy for cognitive rehabilitation of cerebellar damage: A case report and a review of the literature.

BACKGROUND AND IMPORTANCE: The therapeutic approach for cerebellar damages heavily relies on neurorehabilitation since there are no medications that may improve clinical symptoms mainly those related to cognitive dysfunctions. Nevertheless, neurorehabilitation programs tailored to cerebellar damages have never been validated. Here we describe a new rehabilitation approach based on cooking training (CT). The idea that cooking may stimulate cerebellar activity relies on previous evidence demonstrating the beneficial effect on the executive functions as well as in promoting neural plasticity within the cerebellum. Indeed, CT requires motor/mental coordination, thinking flexibly, planning, implementing strategies, shifting and self-monitoring behaviors, all functions drastically affected in cerebellar disorders. CASE DESCRIPTION: A 68-year-old male stroke patient with isolated right cerebellar ischemia in the posterior cerebellum characterized by mild executive dysfunctions. After intensive six weekly two-hour sessions, we found that CT was effective in improving some cognitive abilities in a context of mild motor impairment. In particular, deficits in the execution of the Symbol digit modality test and Wisconsin card-sorting test were recovered. CONCLUSION: The comparison of our data with those reported in previous studies confirmed the Schmahmann's hypothesis on the effectiveness of neurorehabilitation approaches in cerebellar patients acting as external timekeeping of conscious thoughts.

Acute vestibular syndrome in cerebellar stroke : A case report and review of the literature.

The current paper reports on a patient with recurrent rotational vertigo and persistent dizziness and imbalance lasting several weeks, who underwent extensive neuro-otological and radiological examinations. Pathological findings initially included right-sided benign paroxysmal positional vertigo (BPPV), persistent horizontal spontaneous nystagmus (SPN) to the left, and a pathological bedside and video head impulse test (HIT) on the left. The pathological HIT on the left and the SPN to the left indicated a central origin. Therefore, cranial magnetic resonance imaging was performed which revealed a left-sided ischemic stroke in the territory of the medial branch of the posterior inferior cerebellar artery (mPICA).

Cerebellar mutism syndrome: cause and rehabilitation.

PURPOSE OF REVIEW: Mutism of cerebellar origin may occur in the context of various causes but is most frequent in children after resection of a large midline cerebellar tumour. In this review, the endeavour to reach a consensus on name and definition of postoperative mutism of cerebellar origin and associated symptoms is highlighted. In addition, progress in understanding of cause and risk factors for the syndrome is discussed as well as the rehabilitation issues. RECENT FINDINGS: Consensus on the term cerebellar mutism syndrome (CMS) has been reached. The exact pathogenesis of CMS remains unclear. Recently, attention was drawn to the hypothesis that thermal injury might be an important mechanism in the pathogenesis of CMS. Diffusion tensor imaging tractography was found to visualize the damage to relevant pathways that are associated with persistent impairments after recovery of CMS. There is still no established treatment for CMS to date. SUMMARY: By reaching a consensus on terminology and description of CMS, a firm basis has been created for future research. The pathogenesis of CMS seems multifactorial and important risk factors have been found. However, CMS cannot be effectively prevented yet and no established or specific treatment is available, apart from very general rehabilitation and cognitive interventions.

Postoperative Pediatric Cerebellar Mutism After Posterior Fossa Surgery: A Case Report.

Cerebellar mutism syndrome (CMS) is a common complication of posterior fossa surgery that can confound the postanesthetic examination and have long-lasting impacts. There is confusion surrounding its precise description, diagnostic features, and associated morbidity. Here, we discuss the most up-to-date knowledge of CMS drawing from a clinical case in the context of 3 new reports: (1) an international consensus paper presenting a new proposed working definition by the Iceland Delphi Group, (2) a knowledge update by Gadgil et al, (3) and a review of neuroimaging-based data elucidating the etiology of CMS by Patay.

Successful neuropsychological rehabilitation in a patient with Cerebellar Cognitive Affective Syndrome.

The objective of this case study was to describe the neuropsychological rehabilitation of a 16-year-old patient who presented a Cerebellar Cognitive Affective Syndrome (CCAS) following a bilateral cerebellar hemorrhage. The patient presented severe and diffuse cognitive deficits, massive behavioral disorders, and emotion regulation difficulties. The cognitive rehabilitation was performed in the chronic phase (one year after the onset of the hemorrhage) using a transdisciplinary neurobehavioral approach based on the patient's favorite interest (soccer). A significant behavioral and cognitive improvement was observed. The patient became progressively independent in all activities of daily living and was discharged home. The Functional Independence Measure at discharge was 124/126 (vs. 37/126 at entry). The patient was able to complete his schooling despite the mild cognitive and behavioral sequelae. This first description of the use of neurobehavioral therapy in a case of chronic CCAS suggests that (a) major clinical improvement can occur more than one year after the onset of the CCAS, showing the importance of long-term and intensive neurorehabilitation; and (b) when the cerebellum cannot properly play its regulator role in cognition, neuropsychological intervention through a behavioral and cognitive approach can be of great help by acting as an external modulator to help the patient regain control over himself.

Training balance with opto-kinetic stimuli in the home: a randomized controlled feasibility study in people with pure cerebellar disease.

OBJECTIVE: To investigate the feasibility of a randomized controlled trial of a home-based balance intervention for people with cerebellar ataxia. DESIGN: A randomized controlled trial design. SETTING: Intervention and assessment took place in the home environment. PARTICIPANTS: A total of 12 people with spinocerebellar ataxia type 6 were randomized into a therapy or control group. Both groups received identical assessments at baseline, four and eight weeks. INTERVENTIONS: Therapy group participants undertook balance exercises in front of optokinetic stimuli during weeks 4-8, while control group participants received no intervention. MAIN MEASURES: Test-retest reliability was analysed from outcome measures collected twice at baseline and four weeks later. Feasibility issues were evaluated using daily diaries and end trial exit interviews. RESULTS: The home-based training intervention with opto-kinetic stimuli was feasible for people with pure ataxia, with one drop-out. Test-retest reliability is strong (intraclass correlation coefficient >0.7) for selected outcome measures evaluating balance at impairment and activity levels. Some measures reveal trends towards improvement for those in the therapy group. Sample size estimations indicate that Bal-SARA scores could detect a clinically significant change of 0.8 points in this functional balance score if 80 people per group were analysed in future trials. CONCLUSIONS: Home-based targeted training of functional balance for people with pure cerebellar ataxia is feasible and the outcome measures employed are reliable.

Clinical improvements in higher brain function and rapid functional recovery in a case of cerebellar hemorrhage treated by neurocognitive rehabilitation.

Cerebellar stroke rarely causes disorders of higher brain function such as cognitive deficits and emotional dysfunction; hence, the prognosis of these patients is uncertain. We report the case of a 34-year-old patient with cerebellar hemorrhage due to arteriovenous malformation causing higher brain dysfunction who was able to return to full-time employment after neurocognitive rehabilitation. Cerebellar stroke caused by nonatherosclerotic diseases or diaschisis may lead to cognitive deficits but these symptoms can be reversed by appropriate therapy. It is important to assess higher brain function in cerebellar stroke patients and to tailor neurocognitive rehabilitation programs appropriately to promote functional recovery.

Brain changes associated with postural training in patients with cerebellar degeneration: a voxel-based morphometry study.

Recent research indicates that physiotherapy can improve motor performance of patients with cerebellar degeneration. Given the known contributions of the cerebellum to motor learning, it remains unclear whether such observable changes in performance are mediated by the cerebellum or cerebral brain areas involved in motor control and learning. The current study addressed this question by assessing the increase in gray matter volume due to sensorimotor training in cerebellar patients using voxel-based morphometry. Nineteen human subjects with pure cerebellar degeneration and matched healthy controls were trained for 2 weeks on a balance task. Postural and clinical assessments along with structural magnetic resonance imaging were performed pretraining and post-training. The main findings were as follows. First, training enhanced balance performance in cerebellar patients. Second, in contrast to controls patients revealed significantly more post-training gray matter volume in the dorsal premotor cortex. Third, training-related increase in gray matter volume was observed within the cerebellum and was more pronounced in controls than in patients. However, statistically cerebellar changes were at the trend level and thus require additional, independent confirmation. We conclude that sensorimotor training of patients with cerebellar neurodegeneration induces gray matter changes primarily within nonaffected neocortical regions of the cerebellar-cortical loop. Residual function of the cerebellum appears to be exploited suggesting either a recovery from degeneration or intact processes of cerebellar plasticity in the remaining healthy tissue.

Combined statistical analysis method assessing fast versus slow movement training in a patient with cerebellar stroke: a single-case study.

BACKGROUND: Gold standards of data analysis for single-case research do not currently exist. OBJECTIVE: The purpose of this study was to determine whether a combined statistical analysis method is more effective in assessing movement training effects in a patient with cerebellar stroke. DESIGN: A crossover single-case research design was conducted. METHODS: The patient was a 69-year-old man with a chronic cerebellar infarct who received two 5-week phases of finger tracking training at different movement rates. Changes were measured with the Box and Block Test, the Jebsen-Taylor test, the finger extension force test, and the corticospinal excitability test. Both visual analysis and statistical tests (including split-middle line method, t test, confidence interval, and effect size) were used to assess potential intervention effects. RESULTS: The results of the t tests were highly consistent with the confidence interval tests, but less consistent with the split-middle line method. Most results produced medium to large effect sizes. LIMITATIONS: The possibility of an incomplete washout effect was a confounding factor in the current analyses. CONCLUSIONS: The combined statistical analysis method may assist researchers in assessing intervention effects in single-case stroke rehabilitation studies.