HALLERVORDEN - SPATZ DISEASE DIAGNOSED BY CLINICAL AND MRI FINDINGS : FIRST REPORT IN THAILAND
Keywords:
Hallervorden - Spatz disease, Progressive dystonia, dysarthria, Magnetic resonance imaging, “Eye of the tiger”. sign.Abstract
The diagnosis of Hallervorden-Spatz disease (HSD) has usually been made post mortem. The development of magnetic resonance imaging has increased the number of clinical diagnosis. The characteristic abnormalities in globus pallidus : iron deposition has suggested the possibility of diagnosis. The HSD could be a familial or sporadic disorder.
We present a patient of young male with progressive generalized dystonia and dysarthria ; MRI pallidal abnormalities consisted of decreased signal intensity in T2W, compatible with iron deposit, and a small area of hyper intensity in its internal segment (“Eye of the tiger” sign)
We propose that the combination of these neurological signs with these MRI findings could be considered as highly suggestive diagnosis of HSD ina living patients.
The clinical differential diagnosis is Wilson’s disease, by a normal serum copper and ceruloplasmin level.
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