The underpinnings of cerebellar ataxias

The human cerebellum contains more than 60% of all neurons of the brain. Anatomically, the cerebellum is divided into 10 lobules (I-X). The cerebellar cortex is arranged into three layers: the molecular layer (external), the Purkinje cell layer and the granular layer (internal). Purkinje neurons and interneurons are inhibitory, except for granule cells. The layer of Purkinje neurons inhibit cerebellar nuclei, the sole output of the cerebellar circuitry, as well as vestibular nuclei. The cerebellum is arranged into a series of olivo-cortico-nuclear modules arranged longitudinally in the rostro-caudal plane. The cerebro-cerebellar connectivity is organized into multiple loops running in parallel. From the clinical standpoint, it is now considered that cerebellar symptoms can be gathered into 3 cerebellar syndromes: a cerebellar motor syndrome (CMS), a vestibulocerebellar syndrome (VCS) and a cerebellar cognitive affective syndrome/Schmahmann syndrome (CCAS/SS). CMS remains a cornerstone of modern clinical ataxiology, and relevant lesions involve lobules I-V, VI and VIII. The core feature of cerebellar symptoms is dysmetria, covering motor dysmetria (errors in the metrics of motion) and dysmetria of thought. The cerebellar circuitry plays a key-role in the generation and maintenance of internal models which correspond to neural representations reproducing the dynamic properties of the body. These models allow predictive computations for motor, cognitive, social, and affective operations. Cerebellar circuitry is endowed with noticeable plasticity properties.