Mulroy, E; Balint, B; Latorre, A; Schreglmann, S; Menozzi, E; Bhatia, KP; Syringomyelia-Associated Dystonia: Case Series, Literature Review, and Novel Insights. Movement Disorders Clinical Practice 10.1002/mdc3.12772. (In press).

Text Bhatia Syrinx Dystonia MDCPrevised submission NOTC.pdf - Accepted Version Access restricted to UCL open access staff Download (468kB)

Abstract

Background Syringomyelia has previously been suggested as a potential trigger of secondary dystonia. However, a definite causal relationship between the conditions remains to be established. We describe 4 cases of syrinx‐associated dystonia, review past literature on the subject, and propose novel pathophysiological insights into this association. Methods We reviewed demographic, clinical, and neuroradiological features in 4 cases of syrinx‐associated dystonia. A retrospective review of previously published literature on the subject was also conducted. Results Patients with syrinx‐associated dystonia were younger than those with primary dystonia. None had sensory gestes. Syringomyelia frequently involved the cervical cord. Arnold‐Chiari type 1 malformation was a common finding. Some patients responded to botulinum toxin and syrinx decompression. Conclusion Further work is needed to clearly establish the correlation between syringomyelia and dystonia. However, plausibly, syrinx‐related disruption of the dystonia network (through alterations in sensory inputs, loss of inhibition and cerebellar abnormalities) could explain the association.

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