Peripheral and Autonomic Neuropathies with Involvement of the Central Nervous System

Abstract

This chapter discusses the disorders affecting the peripheral and autonomic nervous systems, often accompanied with lesions in the CNS. Charcot–Marie–Tooth disease comprises a morphologically and genetically heterogeneous group of disorders, and is subdivided into two: neuropathic form and neuronal form. Giant axonal neuropathy is described as a slowly progressive peripheral neuropathy. Involvement of the CNS has been repeatedly observed. Nervous control of gastrointestinal motility is extremely complex, regulated by the enteric system, the “brain of the gut,” and modulated by extrinsic nerves. This system, with its multiplicity of transmitters and receptors, does not always allow a clear interpretation of experimental data, especially with compounds lacking specificity. Hirschsprung's disease is characterized by the absence of enteric neurons from the distal colon and rectum. Total intestinal aganglionosis is a rare uniformly fatal condition, with an absence of ganglia from the duodenum to the rectum. Hypoganglionosis differs from neuronal colon dysplasia. While aganglionosis usually requires surgical therapy, in dysganglionosis, a distinct differentiation is necessary between patients sufficiently treated by conservative methods and others requiring surgery.