Superior oblique myokymia: diagnostic criteria and long-term outcome

Gerb, Johannes ; Zwergal, Andreas ; Lehrer, Nicole; Hansel, Nedda; Näher, Marius; Thiessen, Franziska; Dieterich, Marianne und Huppert, Doreen (2026): Superior oblique myokymia: diagnostic criteria and long-term outcome. In: Journal of Neurology, Vol. 273, No. 4, 242

Abstract

Background: Superior oblique myokymia (SOM) is a rare disorder characterized by brief episodes of high-frequency, low-amplitude monocular nystagmus frequently caused by vascular contact with the trochlear nerve. Comprehensive analyses of SOM symptomatology and long-term progression are limited. No diagnostic criteria are available.

Methods: Adult patients who were diagnosed with SOM between 2008 and 2024 were asked to complete a detailed questionnaire on disease symptomatology, time course, and medication. The impact of the disease on patient-reported functioning was assessed using a Likert scale. In addition, medical records were screened in all patients.

Results: A total of 35 patients with SOM were identified (21 females; mean age at symptom onset 42.8 ± 15.0 years). 30 patients experienced visuo-perceptual symptoms (i.e., diplopia or oscillopsia, 85.7%), 22 patients ocular motor symptoms (i.e., noticeable “eye-twitching”, 62.9%), and 15 patients dizziness or gait instability (42.9%). Of 22 patients analyzed for their long-term therapeutic outcome, 13 described symptom improvement with pharmacotherapy, and six without pharmacotherapy. Patients experienced a moderate to high impact of the disease on their daily private and professional lives. Based on these findings, specific diagnostic criteria for SOM were proposed.

Conclusions: SOM can substantially impair patients’ quality of life and daily functioning. The long-term course appears favorable, with most patients experiencing either complete remission or significant symptom reduction over time. Pharmaceutical treatment proved effective in nearly 90% of patients, and spontaneous remission was also observed. Further research should focus on larger patient cohorts defined by clear diagnostic criteria to enhance understanding of SOM’s natural history and treatment outcomes.

• Item Type: Journal article
• Faculties: Medicine > Munich Cluster for Systems Neurology (SyNergy); Medicine > Medical Center of the University of Munich > Neurological Clinic and Polyclinic with Friedrich Baur Institute; Medicine > Medical Center of the University of Munich > German Center for Vertigo and Balance Disorders
• Research Centers: Graduate School of Systemic Neurosciences (GSN)
• Subjects: 500 Science > 570 Life sciences; biology; 600 Technology > 610 Medicine and health
• ISSN: 0340-5354
• Language: English
• DFG: Gefördert durch die Deutsche Forschungsgemeinschaft (DFG) - 390857198