Academic Commons

Articles

Update on the Non-Huntington's Disease Choreas with Comments on the Current Nomenclature

Walker, Ruth H.

Chorea can be caused by a multitude of etiologies: neurodegenerative, pharmacological, structural, metabolic, and others. In absence of other apparent causes, exclusion of Huntington's disease is often a first step in the diagnostic process. There are a number of neurodegenerative disorders whose genetic etiology has been identified in the past decade. Molecular diagnosis has enabled genetic identification of disorder subtypes which were previously grouped together, such as the neurodegeneration with brain iron accumulation disorders and the neuroacanthocytosis syndromes, as well as identification of phenotypic outliers for recognized disorders. Correct molecular diagnosis is essential for genetic counseling and, hopefully, ultimately genetic therapies. In addition, there has recently been recognition of other disorders which can mimic neurodegenerative disorders, including paraneoplastic and prion disorders. This article focuses upon recent developments in the field but is not intended to provide an exhaustive review of all causes of chorea, which is available elsewhere. I also discuss the nomenclature of these disorders which has become somewhat unwieldy, but may ultimately be refined by association with the causative gene.

Subjects

Files

Also Published In

Title
Tremor and Other Hyperkinetic Movements

More About This Work

Academic Units
Center for Parkinson's Disease and Other Movement Disorders
Published Here
July 6, 2012
Academic Commons provides global access to research and scholarship produced at Columbia University, Barnard College, Teachers College, Union Theological Seminary and Jewish Theological Seminary. Academic Commons is managed by the Columbia University Libraries.