Cerebellar motor syndrome from children to the elderly

Handb Clin Neurol. 2018;154:151-166. doi: 10.1016/B978-0-444-63956-1.00009-6.

Abstract

More than a century after the description of its cardinal components, the cerebellar motor syndrome (CMS) remains a cornerstone of daily clinical ataxiology, in both children and adults. Anatomically, motor cerebellum involves lobules I-V, VI, and VIII. CMS is typically associated with errors in the metrics of voluntary movements and a lack of coordination. Symptoms and motor signs consist of speech deficits, impairments of limb movements, and abnormalities of posture/gait. Ataxic dysarthria has a typical scanning (explosive with staccato) feature, voice has a nasal character, and speech is slurred. Cerebellar mutism is most common in children and occurs after resection of a large midline cerebellar tumor. Ataxia of limbs includes at various degrees dysmetria (hypermetria: overshoot, hypometria: undershoot), dysdiadochokinesia, cerebellar tremor (action tremor, postural tremor, kinetic tremor, some forms of orthostatic tremor), isometrataxia, disorders of muscle tone (both hypotonia and cerebellar fits), and impaired check and rebound. Handwriting is irregular and some patients exhibit megalographia. Cerebellar patients show an increased body sway with a broad-based stance (ataxia of stance). Gait is irregular and staggering. Delayed learning of complex motor skills may be a prominent feature in children. CMS is currently explained by the inability of the cerebellum to handle feedback signals during slow movements and to create, store, select, and update internal models during fast movements. The cerebellum is embedded in large-scale brain networks and is essential to perform accurate motor predictions related to body dynamics and environmental stimuli. Overall, the observations in children and adults exhibiting a CMS fit with the hypothesis that the cerebellum contains neural representations reproducing the dynamic properties of body, and generates and calibrates sensorimotor predictions. Therapies aiming at a reinforcement or restoration of internal models should be implemented to cancel CMS in cerebellar ataxias. The developmental trajectory of the cerebellum, the immature motor behavior in children, and the networks implicated in CMS need to be taken into account.

Keywords: cerebellum; coordination; internal models; motor; timing.

Publication types

  • Review

MeSH terms

  • Aging*
  • Cerebellar Diseases / physiopathology*
  • Cerebellum / pathology*
  • Extremities / physiopathology
  • Humans
  • Movement Disorders / physiopathology*
  • Posture
  • Psychomotor Performance / physiology
  • Speech Disorders / etiology
  • Writing