Teaching NeuroImages: Cerebral white matter involvement in a patient with Vogt-Koyanagi-Harada syndrome
A 27-year-old man presented with decreased vision bilaterally. Ophthalmologic examination revealed panuveitis with bilateral serous retinal detachments (figure 1) characteristic of Vogt-Koyanagi-Harada (VKH) syndrome. Analysis of CSF revealed mild pleocytosis. No oligoclonal bands were detected. On MRI, there was bilateral abnormal thickening of the choroid with retinal detachment (figure 2, A and B), with hyperintense lesion on fluid-attenuated inversion recovery (FLAIR) image in the periventricular deep white matter (figure 2C). VKH syndrome is one of the causes of uveo-meningeal syndromes. Differential diagnosis of VKH syndrome includes Wegener granulomatosis, sarcoidosis, and Behçet disease.1 The main disorders showing association with hyperintense plaques on deep white matter FLAIR images are inflammatory, metabolic, vascular, degenerative, autoimmune, and demyelinating diseases. MRI is a helpful tool in diagnosing VKH syndrome; in addition to the typical bilateral ocular findings, scattered periventricular white matter lesions on FLAIR are also described.2

Fundus photography (A, B) reveals exudative retinal detachment of the macula in both eyes on presentation. Fundus fluorescein angiography (C, D) shows subretinal fluorescein pooling. Optical coherence tomography displays exudative macular detachment in both eyes (E, F). It also shows reduction of subretinal fluid after corticosteroid therapy in both eyes (G, H).

Axial T1-weighted MRI (A, B) demonstrates symmetric choroidal thickening with retinal detachment (arrows). Axial fluid-attenuated inversion recovery MRI shows hyperintense lesion in the periventricular left parietal deep white matter (C).
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REFERENCES
1.
Brazis PW, Stewart M, Lee AG. The uveo-meningeal syndromes. Neurologist 2004;10:171–184.
2.
Lohman BD, Gustafson CA, McKinney AM, Sarikaya B, Silbert SC. MR imaging of Vogt-Koyanagi-Harada syndrome with leptomeningeal enhancement. AJNR Am J Neuroradiol 2011;32:E169–E171.
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© 2013 American Academy of Neurology.
Publication History
Published online: September 9, 2013
Published in issue: September 10, 2013
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The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.
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No targeted funding reported.
Authors
Author Contributions
Dr. Sadullah Keles: study concept and design and acquisition of data. Dr. Hayri Ogul: analysis and interpretation. Dr. Lokman Can Pinar: acquisition of data. Dr. Mecit Kantarci: critical revision of the manuscript for important intellectual content.
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Cited By
- Cochleitis in Vogt-Koyanagi-Harada disease, Radiopaedia.org, (2023).https://doi.org/10.53347/rID-164312
- Vogt-Koyanagi-Harada disease, Radiopaedia.org, (2023).https://doi.org/10.53347/rID-159969
- Unusual neurologic manifestations of Vogt-Koyanagi-Harada disease: a systematic literature review, BMC Neurology, 22, 1, (2022).https://doi.org/10.1186/s12883-022-02569-6
- Concurrent presence of retinal hemorrhages in the setting of acute Vogt-Koyanagi-Harada syndrome - an unusual presentation, Journal of Ophthalmic Inflammation and Infection, 10, 1, (2020).https://doi.org/10.1186/s12348-020-00203-5
- Unique clinical spectrum with distinguishing diagnostic features in Vogt-Koyanagi-Harada syndrome, BMJ Case Reports, 12, 12, (e231397), (2019).https://doi.org/10.1136/bcr-2019-231397
- Diagnostic Testing in Uveitis, Uveitis, (143-162), (2019).https://doi.org/10.1007/978-981-15-0331-3_5
- F-18 FDG PET Findings for Vogt-Koyanagi-Harada Disease, Nuclear Medicine and Molecular Imaging, 51, 2, (190-192), (2015).https://doi.org/10.1007/s13139-015-0384-7
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