Abstract

The cerebellum has a well-established role in maintaining motor coordination and studies of cerebellar learning suggest that it does this by recognizing neural patterns, which it uses to predict optimal movements. Serious damage to the cerebellum impairs this learning and results in a set of motor disturbances called ataxia. However, recent work implicates the cerebellum in cognition and emotion, and it has been argued that cerebellar dysfunction contributes to non-motor conditions such as autism spectrum disorders (ASD). Based on human and animal model studies, two major questions arise. Does the cerebellum contribute to non-motor as well as motor diseases, and if so, how does altering its function contribute to such diverse symptoms? The architecture and connectivity of cerebellar circuits may hold the answers to these questions. An emerging view is that cerebellar defects can trigger motor and non-motor neurological conditions by globally influencing brain function. Furthermore, during development cerebellar circuits may play a role in wiring events necessary for higher cognitive functions such as social behavior and language. We discuss genetic, electrophysiological, and behavioral evidence that implicates Purkinje cell dysfunction as a major culprit in several diseases and offer a hypothesis as to how canonical cerebellar functions might be at fault in non-motor as well as motor diseases.

Highlights

  • The cerebellum is essential for smooth, purposeful movement

  • We discuss the etiology of cerebellar disease in the context of how circuits are organized, and present evidence that cerebellar connectivity may be altered in ataxia, dystonia, and autism spectrum disorders ASD

  • Keeping in mind that a lively debate continues as to whether the cerebellum is involved in non-motor function (Lemon and Edgley, 2010), we present recent evidence that has unveiled unexpected roles for the cerebellum in conditions that are historically “non-cerebellar” (Table 1)

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Summary

Introduction

The cerebellum is essential for smooth, purposeful movement. Recently, human neuroimaging and animal behavior studies have implicated the cerebellum in the processing of signals for perception, cognition, and emotion (Schmahmann, 2010; Bastian, 2011; D’Angelo and Casali, 2012), in circumstances involving predictions or timing. Recent work implicates the cerebellum in cognition and emotion, and it has been argued that cerebellar dysfunction contributes to non-motor conditions such as autism spectrum disorders (ASD). Electrophysiological, and behavioral evidence that implicates Purkinje cell dysfunction as a major culprit in several diseases and offer a hypothesis as to how canonical cerebellar functions might be at fault in non-motor as well as motor diseases.

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