Anti-IgLON5 disease presenting with myokymia and favourable response to FcRN-antagonist combined with oral glucocorticoids: case report
By
Tianling Shang
Bo Ma
Shumei Wen
Zuohong Tang
Xiaoliang Mao
Keping Jiao
June 29, 2026
Clinical Scorecard: Case Report: Myokymia as a Presentation of Anti-IgLON5 Disease with Positive Response to FcRN Antagonist and Oral Glucocorticoids
At a Glance
Category Detail
Condition Anti-IgLON5 disease Key Mechanisms Autoimmune disorder characterized by anti-neuronal surface autoantibodies targeting IgLON5 protein. Target Population Adults with autoimmune neurological disorders. Care Setting Neurology clinic or hospital setting.
Key Highlights
Patient presented with generalized multi-site myokymia. Positive anti-IgLON5 IgG antibodies in serum and CSF. Treatment included FcRn antagonist and oral glucocorticoids. Symptoms alleviated rapidly after treatment. Antibody titers declined gradually to negative. Guideline-Based Recommendations
Diagnosis
Diagnosis based on clinical symptoms and positive anti-IgLON5 antibodies in serum and CSF. Management
Combined immunosuppressive therapy with FcRn antagonist and glucocorticoids. Monitoring & Follow-up
Monitor antibody titers and symptomatology during follow-up. Risks
Consider potential side effects of glucocorticoids and immunotherapy. Patient & Prescribing Data
53-year-old male with anti-IgLON5 disease.
Initial treatment with baclofen was ineffective; combined therapy led to symptom control.
Clinical Best Practices
Screen for neuronal autoantibodies in patients presenting with myokymia. Consider FcRn antagonists as a treatment option for anti-IgLON5 disease. Related Resources & Content
