Background: Many patients with lower cranial dystonia (LCrD) are misdiagnosed, and recognition of this condition by general practitioners and dental health professionals is limited.
Methods: We define the phenomenology and natural history of idiopathic LCrD, presenting in 41 patients with the disorder, the largest series of these patients reported to date.
Results: Phenomenology of dystonia included lower cranial and pharyngeal involvement, jaw opening and jaw closing dystonia, and tongue dystonia. Of 25 newly described patients, 72% (18) were female, average age at onset was 56 years, and delay before correct diagnosis was 3.8 years (0-25 years, median 2 years). Eleven patients (44%) reported a precipitating event, the most common of which was recent dental work. Geste antagonistes were found in 18 patients (72%). Response to treatment was mixed, indicating an unmet therapeutic need.
Conclusions: Idiopathic LCrD is often missed and institution of effective therapy is often delayed. The clinical features and natural history of LCrD are similar to other forms of focal dystonia.
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| http://dx.doi.org/10.1186/2054-7072-1-3 | DOI Listing |
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