Acute posterior multifocal placoid pigment epitheliopathy

Prior to onset of the condition, about a third of people have flu-like or viral symptoms such as fever, swollen lymph glands, nausea, vomiting, joint pain and/or tenderness. Headaches may also be present. Rarely, there may be neurological signs such as temporary loss of speech (aphasia) and/or weakness of the arms and legs.^[1][2]

In the early stages of APMPPE, affected people may notice areas of visual blotchiness; flashes of light (photopsia) caused by irritation of the retina; distortion of shapes (metamorphopsia); increased sensitivity to light (photophobia); and/or conjunctivitis. Later, affected people usually develop impairment of vision. In rare cases, vision impairment may be severe.^[1][2]

In most cases, the disorder resolves within a few weeks without permanent loss of visual acuity. However, in some cases, visual acuity does not improve.^[2] Neurological symptoms develop in some affected people and should be evaluated and treated promptly, as neurological involvement can result in severe complications.^[3][1]

There are rare cases of chronic or recurrent APMPPE; it has been suggested that these cases represent relentless placoid chorioretinitis.^[4] This is a rare condition characterized by the development of multiple inflammatory lesions resembling those seen in APMPPE. However, unlike in APMPPE, the lesions continue to expand in size and number, with a relentless course over many months.^[5]

While we are not aware of the specific symptom of memory loss being reported in people with APMPPE, the association between APMPPE and central nervous system (CNS) complications is well known. There have been reports of various neurological signs and symptoms in people with APMPPE including headaches (the most common symptom); confusion; hemiplegia; hemiparesis; dysarthria; numbness; ataxia; incontinence; hearing loss; optic neuritis; meningoencephalitis; transient ischemic attacks; and strokes.^[3][6] Cerebrovascular lesions (abnormalities in the blood vessels supplying the brain) in people with APMPPE have been documented, and cerebral vasculitis associated with APMPPE has been reported to cause permanent and severe neurological complications in some cases.^[3][6]

If there is suspected neurological involvement in a person with APMPPE, MRI and lumbar puncture (spinal tap) may be used to identify CNS involvement. If CNS involvement is found, treatment should begin immediately.^[3] People with APMPPE who are concerned about neurological signs or symptoms should speak with their health care provider promptly and consult with a neurologist and/or a neurovascular specialist.

The treatment of APMPPE is somewhat controversial, but the general consensus is that no treatment seems to alter the course of the ocular lesions. In cases complicated by subretinal neovascularization (growth of new blood vessels), laser photocoagulation may be useful.^[1]

In most cases, the lesions resolve spontaneously and no therapy is required. Some clinicians have used corticosteroids (which suppress inflammation) to treat the ocular findings and any severe systemic involvement. However, there is no evidence that treatment with corticosteroids affects the visual outcome.^[1] The use of steroids has also been suggested when treating cases where the macula is involved.^[7] Cycloplegics may be useful for severe iritis, which is an uncommon finding.^[1]

It is recommended that people with questions about treatment options for themselves or family members speak with their health care provider.

There have been few reports about the long-term effects of APMPPE on macular function. It has been suggested that the prognosis relating to macular function may be good, unless lesions in the retina or retinal pigment epithelium persist.^[8] Because not all people with APMPPE are affected the same way, those who have specific questions about their own prognosis should speak with their ophthalmologist.