The contribution of mamillary body damage to Wernicke's encephalopathy and Korsakoff's syndrome.

Histopathological alterations of the mamillary bodies are the most conspicuous and the most consistent neuropathological features of several disorders that occur after severe thiamine deficiency, such as Wernicke's encephalopathy and Korsakoff's syndrome. Moreover, they are among the few abnormalities that are visible to the naked eye in these disorders. With a lifetime prevalence of approximately 1.3%, Wernicke's encephalopathy is by far the most frequent cause of damage to the mamillary bodies in humans. Still, there is a persisting uncertainty with regard to the development and the clinical consequences of this damage, because it is virtually impossible to study in isolation. As a rule, it always occurs alongside neuropathology in other subcortical gray matter structures, notably the medial thalamus. Converging evidence from other pathologies and animal experiments is needed to assess the clinical impact of mamillary body damage and to determine which functions can be attributed to these structures in healthy subjects. In this chapter, we describe the history and the current state of knowledge with regard to thiamine deficiency disorders and the contribution of mamillary body damage to their clinical presentations.