Cerebellar pathology and disability worsening in relapsing-remitting multiple sclerosis: A retrospective analysis from the CombiRx trial.

BACKGROUND AND PURPOSE: Cerebellar damage is a valuable predictor of disability, particularly in progressive multiple sclerosis. It is not clear if it could be an equally useful predictor of motor disability worsening in the relapsing-remitting phenotype. AIM: We aimed to determine whether cerebellar damage is an equally useful predictor of motor disability worsening in the relapsing-remitting phenotype. METHODS: Cerebellar lesion loads and volumes were estimated using baseline magnetic resonance imaging from the CombiRx trial (n = 838). The relationship between cerebellar damage and time to disability worsening (confirmed disability progression [CDP], timed 25-foot walk test [T25FWT] score worsening, nine-hole peg test [9HPT] score worsening) was tested in stagewise and stepwise Cox proportional hazards models, accounting for demographics and supratentorial damage. RESULTS: Shorter time to 9HPT score worsening was associated with higher baseline Expanded Disability Status Scale (EDSS) score (hazard ratio [HR] 1.408, p = 0.0042) and higher volume of supratentorial and cerebellar T2 lesions (HR 1.005 p = 0.0196 and HR 2.211, p = 0.0002, respectively). Shorter time to T25FWT score worsening was associated with higher baseline EDSS (HR 1.232, p = 0.0006). Shorter time to CDP was associated with older age (HR 1.026, p = 0.0010), lower baseline EDSS score (HR 0.428, p < 0.0001) and higher volume of supratentorial T2 lesions (HR 1.024, p < 0.0001). CONCLUSION: Among the explored outcomes, single time-point evaluation of cerebellar damage only allows the prediction of manual dexterity worsening. In clinical studies the selection of imaging biomarkers should be informed by the outcome of interest.

Cerebellar damage affects the inference of human motion.

The present study aims at the cerebellum's role in prediction mechanisms triggered by action observation. Five cerebellar patients and six age-paired control subjects were asked to estimate the occluded end point position of the shoulder's trajectories in Sit-to-Stand (STS) or Back-to-Sit (BTS) conditions, following or not biological rules. Contrarily to the control group, the prediction accuracy of the end point position in cerebellar patients did not depend on biological rules. Interestingly, both groups presented similar results when estimating the vanishing position of the target. Taken together, these results suggest that cerebellar damage affectsthe capacity of predicting upcoming actions by observation.

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.

Fetal cerebellar damage in fetuses with severe anemia undergoing intrauterine transfusions.

OBJECTIVE: To evaluate radiologic findings and outcomes of cerebellar injuries in fetuses with severe anemia due to RhD alloimmunization undergoing intrauterine transfusions. METHODS: Imaging of multiplanar neurosonography and magnetic resonance imaging (MRI) were reviewed. Pregnancy outcomes were recorded. RESULTS: Cerebellar injuries were identified after the first intravascular transfusion in four fetuses. Two of these cases were previously reported. The median hemoglobin concentration was 2.1 g/dL. Prenatal neurosonography identified an echogenic collection involving the cerebellum suggestive for hemorrhage in three cases. A progressive hypoplasia of a hemisphere was demonstrated at follow-up examination in one of these cases. Hypoplasia of a cerebellar hemisphere was seen in the fourth fetus. Ultrasound diagnosis was confirmed by prenatal MRI in two cases. In the third case, the postnatal MRI showed as additional finding vermian involvement. One pregnancy was terminated and autopsy confirmed the presence of infratentorial hemorrhage. The remaining infants were delivered alive. At time of writing, a truncal ataxia was diagnosed in the child with vermian hypoplasia, while the other children have met all age-appropriate milestones. CONCLUSIONS: A severe anemia seems to put the fetus at risk of cerebellar damage, despite successful intravascular transfusion.