Cerebellar Scholars’ Challenging Time in COVID-19 Pandemia

Novel coronavirus, SARS-CoV2, has caused pandemic of highly contagious disease called coronavirus disease 2019 (COVID-19), with epicenters in China, Italy,     

Cerebellum: from Development to Disease—the 8th International Symposium of the Society for Research on the Cerebellum and Ataxias

In recent years, there has been tremendous growth in research on cerebellar motor and non-motor functions. Cerebellum is particularly involved in the spectrum of neurodevelopmental diseases. The 8th International Symposium of the Society for Research on the Cerebellum and Ataxia (SRCA) was held in Winnipeg, Manitoba, (Canada) on May 24–26, 2017. The main theme of the 8th International Symposium was “Development of the Cerebellum and Neurodevelopmental Disorders.” Advances in genetics, epigenetic, cerebellar neurogenesis, axonogenesis and gliogenesis, cerebellar developmental disorders including autism spectrum disorders (ASD), neuroimaging, cerebellar ataxias, medulloblastoma, and clinical investigation of cerebellar diseases were presented. The goal of this symposium was to provide a platform to discuss cutting-edge knowledge while allowing researchers and trainees the opportunity to share and discuss their front-line research and ideas with others in the field, make connections, and strengthen international collaborations. The Ferdinando Rossi lecture was delivered by Dr. Richard Hawkes on the topic of patterning of the cerebellar cortex. This symposium emphasized the major importance of the involvement of the cerebellum in neurodevelopmental diseases from the clinical, radiological, biological, and genetic standpoint.     

Analysis of cerebellar dysmetria associated with lithium intoxication

Abstract

By contrast to the permanent cerebellar sequelae developing over the weeks following a lithium intoxication, the cerebellar dysfunction occurring in the acute phase is poorly documented. In this study, we analysed the fast and accurate wrist flexion movements and the associated electromyographic activities in 6 patients as soon as possible after a lithium intoxication and three months later. Movements were recorded before and after increasing the inertial load of the moving hand. During the acute phase of the disease; three patients performed dysmetric movements; which became hypermetric when the inertia was increased. In the three other patients; the overshooting of the target which was already observed in the basal condition (no load), was even larger when masses were added. In all the patients; addition of loads increased the onset latency of the antagonist activity during the acute stage of the intoxication. Three of the six patients who presented fever, renal failure and did not undergo hemodialysis developed permanent cerebellar deficits. The three others who presented no fever, no renal failure and underwent hemodialysis recovered completely. [Neurol Res 1996; 18: 416^24]     

Kinematics of fast wrist movements in manic-depressive illness chronically treated with lithium carbonate

Abstract

Lithium salts have been shown to impair kinematics of fast voluntary movements during acute intoxication. The aim of the present study was to determine whether lithium carbonate affected the kinematics of fast movements in patients chronically treated and who did not exhibit signs of neurotoxicity. We analysed fast wrist flexion movements in 6 healthy subjects, in 5 patients presenting a manic-depressive illness without treatment, and in 8 patients receiving lithium carbonate for a manic-depressive disease. The mean duration of treatment was 3.9±4.1 years, the mean daily dose 837±341 mg and the mean serum level 0.95±0. 15 mEq/l. Although mean movement amplitudes were similar in the 3 groups, the variability of fast movements was increased in patients receiving lithium salts. The ratio of maximum to average velocities (VmNaveJ was significantly higher in patients treated, and their movements were temporally asymmetrical, with a ratio ofacceleration duration divided by deceleration duration being lower than in the 2 other groups. These kinematic abnormalities show that a chronic treatment with lithium salts is associated with an impairment of the cerebellar control of fast single-joint movements. [Neural Res 1998; 20: 320-326]     

Is essential tremor a Purkinjopathy? The role of the cerebellar cortex in its pathogenesis

Essential tremor (ET) encompasses a group of progressive neurological diseases in which the primary clinical feature is kinetic tremor of the arms. There is accumulating evidence to suggest that the cerebellum is involved in the pathogenesis of ET; the clinical presentation, neurophysiological data, and functional and metabolic abnormalities revealed by neuroimaging studies all point toward the dysregulation of cerebellar circuits. Recent neuropathological findings at postmortem demonstrate that Purkinje neurons, and some brainstem neurons, play an integral role in the pathogenesis of this common neurological disorder. The assessment of Purkinje cell linear density shows that Purkinje density is abnormal in ET brains. Specific efforts need be devoted to understanding the molecular and cellular events occurring in the Purkinje neurons of the cerebellar cortex, which are emerging as being of particular importance in the pathogenesis of ET in a subgroup of patients. © 2013 International Parkinson and Movement Disorder Society.     

Diagnostic Criteria for Primary Autoimmune Cerebellar Ataxia—Guidelines from an International Task Force on Immune-Mediated Cerebellar Ataxias

Aside from well-characterized immune-mediated ataxias with a clear trigger and/or association with specific neuronal antibodies, a large number of idiopathic ataxias are suspected to be immune mediated but remain undiagnosed due to lack of diagnostic biomarkers. Primary autoimmune cerebellar ataxia (PACA) is the term used to describe this later group. An International Task Force comprising experts in the field of immune ataxias was commissioned by the Society for Research on the Cerebellum and Ataxias (SRCA) in order to devise diagnostic criteria aiming to improve the diagnosis of PACA. The proposed diagnostic criteria for PACA are based on clinical (mode of onset, pattern of cerebellar involvement, presence of other autoimmune diseases), imaging findings (MRI and if available MR spectroscopy showing preferential, but not exclusive involvement of vermis) and laboratory investigations (CSF pleocytosis and/or CSF-restricted IgG oligoclonal bands) parameters. The aim is to enable clinicians to consider PACA when encountering a patient with progressive ataxia and no other diagnosis given that such consideration might have important therapeutic implications.

     

Mechanisms of human cerebellar dysmetria: experimental evidence and current conceptual bases

The human cerebellum contains more neurons than any other region in the brain and is a major actor in motor control. Cerebellar circuitry is unique by its stereotyped architecture and its modular organization. Understanding the motor codes underlying the organization of limb movement and the rules of signal processing applied by the cerebellar circuits remains a major challenge for the forthcoming decades. One of the cardinal deficits observed in cerebellar patients is dysmetria, designating the inability to perform accurate movements. Patients overshoot (hypermetria) or undershoot (hypometria) the aimed target during voluntary goal-directed tasks. The mechanisms of cerebellar dysmetria are reviewed, with an emphasis on the roles of cerebellar pathways in controlling fundamental aspects of movement control such as anticipation, timing of motor commands, sensorimotor synchronization, maintenance of sensorimotor associations and tuning of the magnitudes of muscle activities. An overview of recent advances in our understanding of the contribution of cerebellar circuitry in the elaboration and shaping of motor commands is provided, with a discussion on the relevant anatomy, the results of the neurophysiological studies, and the computational models which have been proposed to approach cerebellar function.