Mitoxantrone Therapy for Acute Posterior Multifocal Placoid Pigment Epitheliopathy with Cerebral Vasculitis

Abstract

Purpose. To report favorable outcome of a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) associated with cerebral vasculitis after treatment with immunosuppressive therapy by mitoxantrone. Design. Single case report. Method. A 22-year-old man presented with acute isolated bilateral loss of vision revealing APMPPE. Corticosteroid therapy was initiated and visual acuity gradually improved. Seventeen days later, visual function deteriorated again, associated with flu-like syndrome and severe headaches. A relapse of APMPPE was diagnosed, complicated with lymphocytic meningitis and cerebral ischemia. Intravenous therapy with mitoxantrone was performed in combination with methylprednisolone. Results. Headaches disappeared in a few days whereas visual acuity gradually improved and stabilized at 20/40 in the right eye and 20/32 in the left eye. No adverse event was observed. Clinical improvement was confirmed by magnetic resonance imaging. Conclusion. Cerebral vasculitis is the most severe complication of the extraocular manifestations of APMPEE. This diagnosis should be evoked when severe headaches or behavior disorder are associated with APMPEE.

Fatal Ischemic Stroke Complicating Acute Multifocal Placoid Pigment Epitheliopathy

“…An estimated 50% of AMPPE patients have headaches with or without neck stiffness (6,18). Possibly, severe headaches are the earliest indication of cerebral inflammation and can predate the onset of more severe neurologic involvement by several weeks to months (6,8,19,21,22).…”

“…To judge if a patient is in danger of severe neurological complications, the severity of the ophthalmological disease has not been a reliable marker (18). In these cases, there may be elevated erythrocyte sedimentation rate (22) and CSF pleocytosis (6,17,21). In these cases, there may be elevated erythrocyte sedimentation rate (22) and CSF pleocytosis (6,17,21).…”

“…Neurological involvement associated with AMPPE includes headaches (6,9,18), strokes (6,7,17,19), cerebral venous sinus thrombosis (20), seizures (5,6), and CNS vasculitis (6,17,21). These neurological complications tend to occur early in the clinical course and are usually self-limited (18).…”

Recurring meningoencephalitis in sinusitis-associated acute posterior multifocal placoid pigment epitheliopathy under prednisone tapering

“…Although extremely rare, neurological complications of APMPPE had proved to be severe4 5 9–12 16 or even fatal 2 15. Attempts to control neurological complications in patients with recurrences under corticoid tapering have been made with azathioprine,4 9 14 cyclophosphamide12 14 or mitoxantrone 17. Considering the side-effects of all long-term immunosuppressive medication for this young adult, we chose azathioprine under which no further relapses were observed.…”

Neurological complications of acute multifocal placoid pigment epitheliopathy

“…Patients who are already receiving corticosteroids when cerebrovascular complications develop may require more intensive immunosuppression with azathioprine, cyclophosphamide or mitoxantrone [16,17,23,24].…”