Syringomyelia with Chiari 1 malformation presenting as focal hand dystonia

Abstract

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.

Keywords

• dystonia
• pseudoathetosis
• syringomyelia
• writer’s cramps