Neurological Manifestations of Acute Posterior Multifocal Placoid Pigment Epitheliopathy

Abstract

Background and purpose: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an immune-mediated chorioretinal disease that causes acute visual symptoms with characteristic ophthalmoscopic findings. Neurological complications are rarely reported in the literature. Here we report two new cases of APMPPE that presented with neurological manifestations, one of which was associated with peripheral neuropathy, which has not been described before.

Methods: A retrospective database review of all patients with a diagnosis of APMPPE was performed. Clinical, ophthalmological, and neurological data were analyzed, and only cases of APMPPE with neurological complications were included. A literature review of several databases was also performed, and previous case reports were reviewed and analyzed in detail.

Results: In total, 56 cases of APMPPE-associated neurological complications were included in the analyses: 54 from the literature and 2 from our own practice. The most common complication was cerebral vasculitis, which affected 28 patients (50%), followed by headaches in 15 patients (26.8%). The other complications include sixth-cranial-nerve palsy, transient hearing loss, meningoencephalitis, cavernous sinus thrombosis, and viral meningitis.

Conclusions: This report adds to the literature of a novel association of APMPPE with peripheral neuropathy, and comprehensively reviews the neurological manifestations of this disease. A high level of suspicion should be applied when dealing with a case of APMPPE. We recommend applying detailed clinical neurological examinations and magnetic resonance imaging to APMPPE patients, and then early steroid treatment if the examination is positive or even suspicious. Early treatment with steroids and long-term treatment with immunosuppressive azathioprine with interval neurological evaluations will contribute positively to the outcomes and avoid fatal complications, namely strokes.

Keywords: acute posterior multifocal placoid pigment epitheliopathy; corticosteroids; neurological complications; peripheral neuropathy.

Fig. 1. Both posterior poles of the first case showing multiple creamy yellow patches involving the retinal pigment epithelium.

Fig. 2. MRI of the brain showing infarcts at the posterior limb of the left internal capsule extending into the putamen, corona radiata, pericallosal white matter, and posterior frontal centrum semiovale.

Fig. 3. Fundus photography of the second case showing multiple creamy yellow patches involving the retinal pigment epithelium before and after acute posterior multifocal placoid pigment epitheliopathy treatment.