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J Neurol (2010) 257:405409 DOI 10.1007/s00415-009-5334-9
ORIGINAL COMMUNICATION
The thalamic ultrastructural abnormalities in paroxysmal kinesigenic choreoathetosis: a diffusion tensor imaging study
Bo Zhou Qin Chen Qiyong Gong
Hehan Tang Dong Zhou
Received: 17 July 2009 / Revised: 8 September 2009 / Accepted: 16 September 2009 / Published online: 11 December 2009 Springer-Verlag 2009
Abstract Paroxysmal kinesigenic choreoathetosis (PKC) is a rare neurologic disorder. There are not apparent morphological changes in patients with idiopathic PKC. The purpose of this study is to determine whether ultrastructural changes are in the brain of patients with idiopathic PKC using diffusion tensor imaging. From May 2007 to August 2008, seven patients with idiopathic PKC were included. The mean age at initial onset was 11.7 3.1 (range 817) years, and the mean disease duration was 6.9 5.1 (range 114) years. Seven subjects of an age- and sex-matched control group were recruited. DTI data were obtained with a 3-T scanner. Fractional anisotropy (FA) and mean diffusivity (MD) were obtained in eight brain regions of interest. Patients with idiopathic PKC had signicantly higher FA values than controls in the right thalamus (P \ 0.05 Bonferroni corrected). Patients also had lower
MD values than controls in the left thalamus (P \ 0.05 Bonferroni corrected). FA and MD values were not signicantly correlated with age of onset, gender, frequency of attack and duration of the disease. The results showed that in patients with idiopathic PKC, diffusion tensor imaging discloses distinct ultrastructural abnormalities in the thalamus. DTI is a sensitive neuroradiologic technique
for detecting cerebral alterations in patients even without visible lesions on conventional MRI.
Keywords Paroxysmal kinesigenic choreoathetosis
Diffusion tensor imaging Thalamus
Fractional anisotropy
Introduction
Paroxysmal dyskinesias are a group of movement disorders characterized by transient attacks of choreoathetosis or dystonia that recur in brief episodes [1]. Paroxysmal kinesigenic choreoathetosis (PKC) is a term used to describe one of the paroxysmal dyskinesias and is a relatively rare neurological condition that is characterised by sudden, brief (seconds to 5 min) attacks of involuntary movements that are precipitated by sudden voluntary movement [2]. There are not conventional magnetic resonance imaging (MRI) abnormalities in patients with idiopathic PKC. The pathophysiology of PKC may involve ion channel dys-function [3] or abnormalities in the basal ganglia-thalamocortical circuits [46], but it...