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Moyamoya Vasculopathy with Atypical Cogan's Syndrome: A Case Report
Amy Phu1, Erica Chao1, Ranjana Sood2
1Department of Translational Research, College of Osteopathic Medicine of the Pacific, Western University of Health Sciences, Pomona, California 91766 USA.
Archives of Clinical and Medical Case Reports
|August 8, 2026
Summary
This case report details a rare instance of Cogan's syndrome (CS) associated with Moyamoya syndrome. It highlights the potential for autoimmune vascular injury in CS to trigger progressive cerebrovascular disease, even with controlled inflammation.
Area of Science:
- Neurology
- Immunology
- Vascular Medicine
Background:
- Cogan's syndrome (CS) is a rare autoimmune disorder characterized by audio-vestibular and ocular inflammation.
- Systemic vasculitis is a known complication of CS, but its association with Moyamoya-type cerebrovascular remodeling is undocumented.
- This report presents a unique case linking these two conditions.
Purpose of the Study:
- To report the first known case of concurrent Cogan's syndrome and Moyamoya vasculopathy.
- To investigate the potential link between chronic autoimmune vascular injury in CS and the development of Moyamoya syndrome.
- To raise clinical awareness of Moyamoya syndrome as a differential diagnosis in CS patients with new neurological symptoms.
Main Methods:
- Case report of a 19-year-old Asian woman diagnosed with atypical Cogan's syndrome.
- Documentation of clinical presentation, immunosuppressive treatment, and subsequent development of neurological symptoms.
- Neuroimaging (revealing internal carotid artery occlusion and Moyamoya changes), genetic testing (SLC37A4 variant), and surgical intervention (STA-MCA bypass).
Main Results:
- The patient presented with hyperpyrexia, weight loss, and hearing loss, diagnosed as atypical CS.
- Five years post-immunosuppressive treatment, she developed headaches, hypertension, and hemiparesis, despite normal inflammatory markers.
- Neuroimaging confirmed Moyamoya syndrome; surgical bypass resolved neurological symptoms.
Conclusions:
- Late-onset Moyamoya syndrome can develop secondary to chronic autoimmune-mediated vascular injury in Cogan's syndrome.
- Clinicians should consider Moyamoya syndrome in CS patients presenting with new neurological deficits, irrespective of systemic inflammation markers.
- This case underscores the complex interplay between autoimmune disorders and cerebrovascular remodeling.
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