Objective: To describe a group of patients with LP regarding clinical presentation, etiology, and treatment.
Background: Lingual dystonia (LD) is a rare, disabling and difficult to treat form of focal dystonia with a limited number of etiologies described in the literature. In spite of being classically associated with heredo-degenerative diseases, idiopathic and tardive syndromes seem to be common causes of LD in clinical practice.
Method: Retrospective longitudinal study based on an adult movement disorders consult in a tertiary center. We selected LD from all oromandibular dystonia (OMD) patients and reviewed clinical/imagological/genetic/treatment data. Descriptive statistical analysis was made.
Results: Twenty seven of the 73 OMD patients had LD: 21 females with a median age of 52±27 years at onset. Eighteen out of 27 presented in late adulthood. Dystonia was classified as focal in 12, multifocal in 8 and generalized in 6. LD presented as isolated dystonia in 10 and associated with other movement disorders in 8. Regarding the etiology, in most patients (n=17) LD were acquired: tardive dyskinesia (n=9), cerebral palsy/perinatal brain injury (n=2), metabolic disturbance (hyperthyroidism, renal failure) (n=2), peripheral lesion (dental procedure) (n=2), acquired hepatocerebral degeneration (n=1), post-anoxic and iatrogenic encephalopathy (n=1); in 3 patients a genetic etiology was identified (rapid-onset dystonia-parkinsonism – DYT12, YY1-related dystonia – DYT-THAP1, and CANVAS – Cerebellar ataxia with neuropathy and vestibular areflexia syndrome); in the remaining 7, the cause was considered idiopathic or undetermined. Nine patients were treated with botulinum toxin injections (submental complex muscles and/or genioglossus), the majority with subjective clinical and/or functional improvement and without significant adverse effects.
Conclusion: Our series expands the range of pathologies in which LD can be present. As described in the literature, botulinum toxin was an effective treatment for LD patients.
To cite this abstract in AMA style:
J. Lopes, M. Magalhães. Lingual dystonia etiology spectrum in a tertiary center [abstract]. Mov Disord. 2022; 37 (suppl 2). https://www.mdsabstracts.org/abstract/lingual-dystonia-etiology-spectrum-in-a-tertiary-center/. Accessed November 21, 2024.« Back to 2022 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/lingual-dystonia-etiology-spectrum-in-a-tertiary-center/