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Feeding dystonia in McLeod Syndrome§

Authors


  • Funding agencies: C.L. and F.T. have been supported by the EC-funded (E-Rare JTC 2009) EMINA project (European Multidisciplinary Initiative on Neuroacanthocytosis).

  • Relevant conflicts of interest/financial disclosures: Nothing to report.

  • §

    Full financial disclosures and author roles may be found in the online version of this article.

Abstract

Background:

The X-linked McLeod syndrome belongs to the group of neuroacanthocytosis syndromes and has a Huntington-disease–like phenotype with a choreatic movement disorder, cognitive alterations, and psychiatric symptoms. Another neuroacanthocytosis syndrome, the autosomal recessive chorea-acanthocytosis, has a similar presentation, but distinct clinical features, believed to be characteristic, such as tongue protrusion dystonia, feeding dystonia, and rubber-man–like appearance.

Methods:

This work comprised a case series of 3 patients with McLeod syndrome.

Results:

The 3 patients with McLeod syndrome developed severe feeding dystonia and tongue protrusion as well as rubber-man–like appearance in 1 patient during the course of the disease.

Conclusion:

These observations indicate that there is an extended phenotypic overlap between McLeod syndrome and chorea-acanthocytosis. © 2011 Movement Disorder Society

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