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Published on: March 26, 2019
Choroiditis and choroidal neovascularization in acute disseminated encephalomyelitis
Nikol Panou1, Ivana K Kim, Lucia Sobrin
1*Department of Ophthalmology, Zoodochos Pigi Wing, Amalia Fleming Prefectoral General Hospital, Athens, Greece †Retina ‡Uveitis Services, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts.
Insights
This case study highlights a patient with bilateral choroidal neovascularization (CNV) secondary to acute disseminated encephalomyelitis (ADEM)-associated choroiditis. Immunomodulatory therapy successfully prevented CNV recurrence, suggesting a role for systemic inflammation.
Area of Science:
- Ophthalmology
- Neurology
- Immunology
Background:
- Acute disseminated encephalomyelitis (ADEM) is a rare, immune-mediated demyelinating disease.
- Choroiditis and secondary choroidal neovascularization (CNV) are uncommon complications of ADEM.
- Recurrent CNV poses a significant challenge in managing ADEM-associated ocular manifestations.
Purpose of the Study:
- To report a unique case of bilateral CNV secondary to ADEM-associated choroiditis.
- To evaluate the efficacy of immunomodulatory therapy in preventing CNV recurrence.
- To discuss the role of systemic inflammation in the pathogenesis of ADEM-related CNV.
Main Methods:
- A 57-year-old male patient with a history of ADEM was diagnosed with bilateral CNV 6 years post-neurologic event.
- Initial treatment involved intravitreal bevacizumab and oral prednisone.
- Recurrence upon steroid tapering led to the initiation of mycophenolate mofetil as steroid-sparing immunomodulatory therapy.
Main Results:
- Intravitreal bevacizumab and oral prednisone initially controlled the CNV.
- CNV recurred during oral steroid tapering.
- Mycophenolate mofetil achieved sustained remission of CNV for 1.5 years without further antiangiogenic therapy.
Conclusions:
- This is the first reported case of choroiditis and secondary CNV associated with ADEM.
- Systemic immunomodulatory therapy, in conjunction with antiangiogenic agents, should be considered for recurrent CNV in ADEM.
- The findings support the hypothesis that ongoing inflammation plays a crucial role in the pathogenesis of ADEM-associated CNV.
Purpose:
To present a case with bilateral choroidal neovascularization (CNV) secondary to acute disseminated encephalomyelitis-associated choroiditis requiring immunomodulatory therapy for prevention of recurrence.
Methods:
The clinical course of a patient diagnosed with acute disseminated encephalomyelitis, who developed bilateral choroiditis at the time of his neurologic diagnosis and bilateral CNV 6 years later, is reviewed.
Patient:
A 57-year-old man developed CNV in both eyes, 6 years after the initial diagnosis of acute disseminated encephalomyelitis-associated choroiditis. The patient was initially treated successfully with intravitreal bevacizumab injections and oral prednisone, but CNV recurred with steroid tapering. Mycophenolate mofetil was initiated as steroid-sparing immunomodulatory therapy.
Results:
There was no CNV recurrence for 1.5 years without the need for additional antiangiogenic therapy.
Conclusion:
To our best knowledge, this is the first report of choroiditis and secondary CNV associated with acute disseminated encephalomyelitis. In cases of recurrent CNV associated with choroiditis, systemic therapy should be strongly considered in conjunction with antiangiogenic therapy. The recurrence of CNV with tapering of oral steroids and the remission of CNV with steroid-sparing immunomodulatory therapy support the role of ongoing inflammation in the pathogenesis.
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