Huntington's chorea.

Huntington's chorea is an hereditary disorder of the central nervous system characterized by the appearance in adult life of progressive chorea and dementia, and inherited in single autosomal dominant fashion with complete penetrance. The essential pathological features of Huntington's chorea are a primary loss of cells in the caudate nucleus and the putamen, and similar involvement of the cerebral cortex, particularly that of the frontal lobes. Often there is secondary hydrocephalus with gross dilatation of the ventricles. The clinical symptoms do not always correspond with these neuropathological features which are generally regarded as lacking specificity. I: History The disease was first recognized and described in America by Waters (I848), Lyon (I863), and others, before George Huntington gave his famous account of it. It came to be associated, however, with the name of Huntington, and rightly so, for his description of 'Hereditary Chorea' in I872 aroused the admiration of no less a personage than Sir William Osler, for its accuracy, brevity, and vividness. An excellent account of the relation of George Huntington to earlier descriptions of the disease is given by De Jong (I937). Waters recognized the disease to be 'markedly hereditary'. Huntington, with deep insight and acute observation, interpreted the mode of transmission of the disorder and wrote: