Neurological Manifestations of Acute Posterior Multifocal Placoid Pigment Epitheliopathy

Abstract

APMPPE should be considered among the causes of stroke and aseptic meningitis in young adults. The diagnosis is critically dependent on a thorough ophthalmologic examination. Severe neurological complications are difficult to predict at the onset of the ophthalmologic disease. The patients should be monitored closely. If investigations suggest cerebral vasculitis, immunosuppressive treatment may be helpful to prevent recurrences.

Vision Loss and Paresthesias in a Young Man

“…Others 2,5,6 have reported substantial rates of persistent visual field defects, metamorphopsia, and incomplete visual recovery while remaining equivocal regarding the role of systemic therapy. Several 1,7,8 agree that despite the paucity of high-quality clinical evidence, rapid initiation of therapy is justified in patients with central nervous system involvement and that long-term immunomodulatory therapy may be necessary.…”

Epiteliopatía pigmentaria placoide multifocal aguda asociada a parálisis del VI par craneal

“…La cefalea está en relación a la asociación de la EPPMA con una meningitis asép-tica, que no requiere tratamiento, pero es importante realizar una exploración neurológica adecuada (1). Los pacientes pueden presentar diferentes grados de vasculitis cerebral y meningoencefalitis, que pueden aparecer semanas o meses después de la EPPMA (1,3).…”

A Case of Ischemic Cerebral Infarction Associated with Acute Posterior Multifocal Placoid Pigment Epitheliopathy, CNS Vasculitis, Vitamin B₁₂ Deficiency and Homocysteinemia

“…We identified 12 reported cases of stroke in patients with APMPPE in the literature [6]. Some of these patients had cerebral vasculitis and the pathogenesis of the stroke involved irreversible tissue ischemia due to obliteration of the vascular lumen.…”

“…Although lesions usually resolve spontaneously, systemic complications and vision loss may occur [2,3], which respond to corticosteroid therapy. Reported neurological complications include: CSF pleocytosis, aseptic meningitis [4], headache, transient hearing loss [5], optic neuritis, meningioencephalitis and stroke [6].…”