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Images in Neurology
December 2014

Hemidystonia With One Eye-of-the-Tiger Sign

Author Affiliations
  • 1Human Motor Control Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland
  • 2Department of Neurology, Govind Ballabh Pant Hospital, New Delhi, India
  • 3Department of Neurology, Penn State College of Medicine, Hershey, Pennsylvania
  • 4Department of Pediatrics, Penn State College of Medicine, Hershey, Pennsylvania
JAMA Neurol. 2014;71(12):1574-1575. doi:10.1001/jamaneurol.2014.1077

A woman in her late teens presented with dystonic posturing of her left hand for 2 years and left foot for 3 months. Dystonic posturing of her hand had started when she was 8 months pregnant, and it slowly worsened to involve her left lower limb, leading to difficulty in walking (Video). She was evaluated for left hemidystonia. Fluid-attenuated inversion recovery (FLAIR) magnetic resonance images (MRIs) of the brain gave the appearance of the eye-of-the-tiger sign in the right basal ganglia similar to what would be seen with a neuroferritinopathy, but susceptibility-weighted imaging revealed evidence of hemosiderin indicative of an old hemorrhagic infarct (Figure). The results of magnetic resonance angiography for evaluation of intracranial vessels were normal. Mutation analysis for pantothenate kinase 2 was negative. Antinuclear antibody and anti–double-stranded DNA were negative.

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