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The chorea of McLeod syndrome: Progression to hypokinesia
Author(s) -
Miranda Marcelo,
Jung Hans H.,
Danek Adrian,
Walker Ruth H.
Publication year - 2012
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.25224
Subject(s) - chorea , hypokinesia , medicine , physical medicine and rehabilitation , neuroscience , psychology , disease
Before the identification of McLeod neuroacanthocytosis syndrome (MLS) and chorea-acanthocytosis (ChAc) as genetically distinct disorders, these two disorders, which have remarkable clinical similarities, were confusingly lumped together under the term ‘‘neuroacanthocytosis.’’ Genetic diagnosis has enabled us to identify features that initially appeared to be specific to each of the disorders. However, with increased availability of genetic and protein-based testing, it has become apparent that certain phenotypic features thought to be specific to one disorder may indeed be found in the other, such as feeding dystonia and head drops. FIG. 1. Reconstruction of the quadripolar electrode placement in a 3-dimensional atlas, based on preoperative magnetic resonance imaging and on postoperative computed tomography. A: left side; B: right side. Red nucleus, red; subthalamic nucleus (STN), transparent green; thalamic nuclei—ventral anterior (VA), ventral lateral anterior (VLa), ventral lateral posterior (VLpv or Vim).