Syringomyelia-Associated Dystonia: Case Series, Literature Review, and Novel Insights.

dystonia sensorimotor integration syringomyelia

Journal

Movement disorders clinical practice
ISSN: 2330-1619
Titre abrégé: Mov Disord Clin Pract
Pays: United States
ID NLM: 101630279

Informations de publication

Date de publication:
Jun 2019
Historique:
received: 18 11 2018
revised: 28 02 2019
accepted: 15 03 2019
entrez: 10 7 2019
pubmed: 10 7 2019
medline: 10 7 2019
Statut: epublish

Résumé

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. 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. 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. 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.

Sections du résumé

BACKGROUND 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 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 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 CONCLUSIONS
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.

Identifiants

pubmed: 31286008
doi: 10.1002/mdc3.12772
pii: MDC312772
pmc: PMC6592788
doi:

Types de publication

Case Reports

Langues

eng

Pagination

387-392

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Auteurs

Eoin Mulroy (E)

Department of Clinical and Movement Neurosciences UCL Queen Square Institute of Neurology London United Kingdom.

Bettina Balint (B)

Department of Clinical and Movement Neurosciences UCL Queen Square Institute of Neurology London United Kingdom.
Department of Neurology University Hospital Heidelberg Germany.

Anna Latorre (A)

Department of Clinical and Movement Neurosciences UCL Queen Square Institute of Neurology London United Kingdom.

Sebastian Schreglmann (S)

Department of Clinical and Movement Neurosciences UCL Queen Square Institute of Neurology London United Kingdom.

Elisa Menozzi (E)

Department of Clinical and Movement Neurosciences UCL Queen Square Institute of Neurology London United Kingdom.

Kailash P Bhatia (KP)

Department of Clinical and Movement Neurosciences UCL Queen Square Institute of Neurology London United Kingdom.

Classifications MeSH