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A case of acute posterior multifocal placoid pigment epitheliopathy with recurrent stroke
Katie Luneau1, Nancy J Newman, Sunil Srivastava
1Departments of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia 30322, USA.
Abstract:
A 43-year-old man who had visual loss from acute posterior multifocal placoid pigment epitheliopathy (APMPPE) developed a right middle cerebral artery territory infarction a few weeks after the visual loss occurred and shortly after corticosteroid therapy was tapered. He was then treated continuously with oral low-dose prednisone and cyclophosphamide but presented with recurrent cerebral infarction 6 months later, shortly after cyclophosphamide was replaced with azathioprine. Neurologic complications of APMPPE are exceedingly rare, with only 18 other well-documented cases of APMPPE in the English and French literature. Cerebral vasculitis was the presumed mechanism in most patients, but only 2 patients had pathologic confirmation. There have been only 3 reported cases of recurrent cerebral infarction, all occurring during corticosteroid taper. Because neurologic complications of APMPPE are rare, it is reasonable to reserve neuroimaging for patients who have unusual headaches or other new neurologic manifestations.
Insights
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) can rarely cause neurologic complications like cerebral infarction. This case highlights recurrent infarction risk, even with immunosuppressive therapy.
Area of Science:
- Ophthalmology
- Neurology
- Immunology
Background:
- Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare inflammatory condition affecting the retina.
- Neurologic complications, particularly cerebral infarction, are exceedingly rare manifestations of APMPPE.
Observation:
- A 43-year-old male with APMPPE experienced a cerebral infarction shortly after corticosteroid taper.
- Recurrent cerebral infarction occurred 6 months later, coinciding with azathioprine substitution for cyclophosphamide.
Findings:
- Cerebral vasculitis is the presumed mechanism for APMPPE-associated neurologic events.
- Recurrent cerebral infarction is uncommon, with only three prior reported cases, all during corticosteroid taper.
- Pathologic confirmation of cerebral vasculitis in APMPPE is rare, with only two documented instances.
Implications:
- Neuroimaging should be reserved for APMPPE patients with new neurologic symptoms due to the rarity of these complications.
- Careful monitoring and management of immunosuppressive therapy are crucial in APMPPE patients with neurologic involvement.
- This case underscores the potential for recurrent cerebral infarction in APMPPE, necessitating a nuanced approach to treatment adjustments.
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