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. 2024 Dec 4:14:59.
doi: 10.5334/tohm.952. eCollection 2024.

Risk of Spreading in Adult-onset Dystonia

Esra Kochan Kizilkilic  1   2 , Nursena Erener  1 , Mustafa Meric  1 , Nurten Uzun Adatepe  1 , Aysegul Gunduz  1
Affiliations

Risk of Spreading in Adult-onset Dystonia

Esra Kochan Kizilkilic et al. Tremor Other Hyperkinet Mov (N Y). .

Abstract

Background: Adult-onset dystonia can also spread to other parts of the body, although it is not as common as childhood-onset dystonia.

Objective: Our study aimed to examine the clinical factors determining spreading patterns in all adult-onset dystonia types.

Methods: We retrospectively analyzed the medical records of patients with a diagnosis of isolated dystonia followed longitudinally at our center. We included patients reporting symptom onset after 18 years. We then compared the clinical factors between groups with and without spreading.

Results: Among 434 patients (396 focal, 29 segmental, and nine generalized onset dystonia. mean follow-up of 8.6 ± 7.8 years), 48 (11.1%) experienced spread of dystonia, with 37 progressing from focal to segmental, two from focal to generalized, two from segmental to generalized, and seven from focal to segmental to generalized dystonia. Blepharospasm was the most common focal dystonia noted to spread, followed by oromandibular dystonia, cervical dystonia, laryngeal dystonia, and upper extremity dystonia, in decreasing order. A spreading pattern was observed in approximately one in 10 dystonia patients, and the spreading was more frequent in the segmental dystonia group. While there was no difference between the spreading groups regarding sensory tricks, tremor, and gender, family history was more common in the non-spreading group (p = 0.023). Older age at onset was independently associated with increased odds of spreading (hazards ratio: 1.054, p < 0.001, B = 0.053).

Conclusion: Although risk factors for spread are variable, the underlying mechanisms are not fully known. Genetic factors may be possibly related to the spread, and future studies are needed on this subject.

Keywords: adult-onset dystonia; dystonia; focal dystonia; segmental dystonia; spreading.

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Conflict of interest statement

The authors have no competing interests to declare.

Figures

A total of 579 patient files were evaluated retrospectively. We included 434 patients who met the inclusion criteria in the study
Figure 1
Flowchart of the study. CD: Cervical dystonia; BSP: blepharospasm; OMD: oromandibular dystonia; LD: laryngeal dystonia (LD).
We have performed a Kaplan Meier analysis for time to transformation and transformation, including the region involved at onset as the factor
Figure 2
Kaplan-Meier analysis shows the cumulative risk for focal and segmental dystonia over time.
See this image and copyright information in PMC

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