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Huntington Disease and Huntington Disease-Like Syndromes: An Overview

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Chorea

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Abstract

The differential diagnosis of chorea syndromes may be complex. It includes inherited forms, the most important of which is autosomal dominant Huntington disease (HD). In addition there are disorders mimicking HD, the so-called HD-like syndromes, and molecular workup revealed that they account for about 1 % of suspected HD cases. The aim of this review is to summarize the main characteristics of these rare conditions in order to familiarize clinicians with them. While treatment remains symptomatic, advances have been made with genetic delineation. Hopefully with better understanding of their pathophysiology, we will also move towards mechanistic therapies.

The authors have no conflicts of interest.

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Authors and Affiliations

  1. Department of Neurology, Christian-Albrechts-University Kiel, University-Hospital-Schleswig-Holstein, Kiel Campus, Schittenhelmstr. 10, Kiel, 24105, Germany

    Susanne A. Schneider

  2. Department of Neurology, University of Kiel, Kiel, Germany

    Felix Gövert

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  1. Susanne A. Schneider
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  2. Felix Gövert
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Correspondence to Susanne A. Schneider .

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  1. Parkinson Disease and Movement Disorders Program and Neurology Department, Hospital de Clínicas, José de San Martín, Buenos Aires, Argentina

    Federico E. Micheli

  2. Parkinson's Disease and Movement Disorders Program, Henry Ford West Bloomfield Hospital, West Bloomfield, Michigan, USA

    Peter A. LeWitt

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Schneider, S.A., Gövert, F. (2014). Huntington Disease and Huntington Disease-Like Syndromes: An Overview. In: Micheli, F., LeWitt, P. (eds) Chorea. Springer, London. https://doi.org/10.1007/978-1-4471-6455-5_7

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