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Year : 2020  |  Volume : 3  |  Issue : 3  |  Page : 173-177

Syringomyelia with Chiari 1 malformation presenting as focal hand dystonia

Department of Neurology, SMS Medical College, Jaipur, Rajasthan, India

Correspondence Address:
Dr. Vaibhav Mathur
D-176, Nirman Nagar, Kings Road, Ajmer Road, Jaipur, Rajasthan.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/AOMD.AOMD_20_20

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Syringomyelia is described as a fluid-filled cavity within the spinal cord devoid of an ependymal lining. It is best visualized on magnetic resonance imaging (MRI), with the cavity being low intensity on T1-weighted and high intensity on T2-weighted images. The association of syringomyelia with dystonia has been infrequently reported in the medical literature. We herein describe a case of syringomyelia with Chiari 1 malformation and hydrocephalus having writer’s cramp and pseudoathetosis, which as per our review, is a yet undescribed manifestation in previously published literature. Also, we emphasize on the usefulness of spinal MRI in a case of focal dystonia/writer’s cramp if cause is not apparent after initial evaluation and more so if associated with proprioceptive sensory impairment and pseudoathetosis.

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