BACKGROUND: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an ocular disease of young adults with a favorable visual outcome. Up to present the etiology and pathophysiology of APMPPE have not been thoroughly understood. Serious inflammatory systemic and neurological diseases have been described in association with APMPPE. CASE REPORT: We report a single case study of a 29-year-old male patient with APMPPE. Approximately four months after diagnosis of APMPPE the patient developed a temporary tetraparesis as initial symptom of chronic inflammatory cerebral disease. We give an overview of the stated theories of the pathogenesis of APMPPE in the literature. Moreover we account on concomitant systemic symptoms and diseases. We outline neurological complications in APMPPE and discuss the reported therapies. RESULTS: Neurological complications rarely accompany APMPPE and may occur even months after the initial ocular symptoms. There have been reports of meningoencephalitis and cerebral vasculitis and two patients died of neurological complications. The recommended therapeutic regime is high-dose corticosteroids. Relapses can appear during tapering of systemic corticosteroids therapy. The role of immunsuppressive treatment remains unclear. CONCLUSION: APMPPE can be associated with serious inflammatory systemic and neurological affection. Instruction of the patient is indispensable to guarantee early and adequate treatment.
BACKGROUND: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an ocular disease of young adults with a favorable visual outcome. Up to present the etiology and pathophysiology of APMPPE have not been thoroughly understood. Serious inflammatory systemic and neurological diseases have been described in association with APMPPE. CASE REPORT: We report a single case study of a 29-year-old male patient with APMPPE. Approximately four months after diagnosis of APMPPE the patient developed a temporary tetraparesis as initial symptom of chronic inflammatory cerebral disease. We give an overview of the stated theories of the pathogenesis of APMPPE in the literature. Moreover we account on concomitant systemic symptoms and diseases. We outline neurological complications in APMPPE and discuss the reported therapies. RESULTS:Neurological complications rarely accompany APMPPE and may occur even months after the initial ocular symptoms. There have been reports of meningoencephalitis and cerebral vasculitis and two patients died of neurological complications. The recommended therapeutic regime is high-dose corticosteroids. Relapses can appear during tapering of systemic corticosteroids therapy. The role of immunsuppressive treatment remains unclear. CONCLUSION: APMPPE can be associated with serious inflammatory systemic and neurological affection. Instruction of the patient is indispensable to guarantee early and adequate treatment.