Cortex
Volume 44, Issue 8 , Pages 1037-1066, September 2008

Disconnection syndromes of basal ganglia, thalamus, and cerebrocerebellar systems

  • Jeremy D. Schmahmann

      Affiliations

    • Ataxia Unit, Cognitive/Behavioral Neurology Unit, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA
    • Corresponding Author InformationCorresponding author. Department of Neurology, Massachusetts General Hospital, Suite 340, Charles River Plaza South, 55 Fruit Street, Boston, MA 02114, USA.
  • ,
  • Deepak N. Pandya

      Affiliations

    • Department of Anatomy and Neurobiology, Boston University School of Medicine, Boston, MA 02118, USA

Received 24 January 2008; received in revised form 4 March 2008 and 13 April 2008; accepted 14 April 2008. published online 09 July 2008.

Abstract 

Disconnection syndromes were originally conceptualized as a disruption of communication between different cerebral cortical areas. Two developments mandate a re-evaluation of this notion.

First, we present a synopsis of our anatomical studies in monkey elucidating principles of organization of cerebral cortex. Efferent fibers emanate from every cortical area, and are directed with topographic precision via association fibers to ipsilateral cortical areas, commissural fibers to contralateral cerebral regions, striatal fibers to basal ganglia, and projection subcortical bundles to thalamus, brainstem and/or pontocerebellar system. We note that cortical areas can be defined by their patterns of subcortical and cortical connections. Second, we consider motor, cognitive and neuropsychiatric disorders in patients with lesions restricted to basal ganglia, thalamus, or cerebellum, and recognize that these lesions mimic deficits resulting from cortical lesions, with qualitative differences between the manifestations of lesions in functionally related areas of cortical and subcortical nodes.

We consider these findings on the basis of anatomical observations from tract tracing studies in monkey, viewing them as disconnection syndromes reflecting loss of the contribution of subcortical nodes to the distributed neural circuits. We introduce a new theoretical framework for the distributed neural circuits, based on general, and specific, principles of anatomical organization, and on the architecture of the nodes that comprise these systems. We propose that neural architecture determines function, i.e., each architectonically distinct cortical and subcortical area contributes a unique transform, or computation, to information processing; anatomically precise and segregated connections between nodes define behavior; and association fiber tracts that link cerebral cortical areas with each other enable the cross-modal integration required for evolved complex behaviors. This model enables the formulation and testing of future hypotheses in investigations using evolving magnetic resonance imaging techniques in humans, and in clinical studies in patients with cortical and subcortical lesions.

Keywords: Cognition, Anatomy, Connections, Fiber tracts, Distributed neural systems

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PII: S0010-9452(08)00119-6

doi:10.1016/j.cortex.2008.04.004

Cortex
Volume 44, Issue 8 , Pages 1037-1066, September 2008