The supplementary motor area syndrome and the cerebellar mutism syndrome: a pathoanatomical relationship?

Abstract

The supplementary motor area (SMA) syndrome affects adults after tumour resection in SMA neighbouring motor cortex. Cerebellar mutism syndrome (CMS) affects children after tumour resection in the posterior fossa. Both syndromes include disturbances in speech and motor function. The causes of the syndromes are unknown; however, surgical damage to the dentato-thalamo-cortical pathway (DTCP) has been associated with CMS. Thus, an anatomical link between the areas associated with the syndromes is possible. We discuss the syndromes and their possible relationship through the DTCP. We identified 61 articles (cohort studies, case reports and reviews) in MEDLINE and Embase searching for CMS, SMA syndrome or DTCP or synonyms and reviewed for evidence linking CMS and SMA. We found that SMA syndrome and CMS are similar regarding (1) surgical causation; (2) symptoms including speech impairment, disturbance in motor function and facial dysfunction; (3) delayed onset; (4) the courses of the syndromes are transient; and (5) long-term sequelae are seen in both. Relevant differences include age predominance of adults in SMA syndrome versus children in CMS. The similarities of the two syndromes could be traced back to their mutual connection through the DTCP and their membership to a cerebro-cerebellar circuit. The connectivity network could explain the emotional changes and speech reduction in CMS. The difference in time of post-surgical onset may be related to the anatomical distance between the surgical damage to the cerebellum and the SMA, respectively, and the effector neural loop underpinning symptoms.