Journal francais d'ophtalmologie · 2025
Acute posterior multifocal placoid pigment epitheliopathy complicated by a stroke, leading to the diagnoses of lupus anticoagulant and hypercysteinemia.
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Zayani M, Abada S
Аннотация
PURPOSE: To report a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) complicated by a stroke, leading to the diagnoses of lupus anticoagulant and hypercysteinemia.
METHODS: Case report.
RESULTS: A 28-year-old man complained of blurry vision and red eyes. On exam, his visual acuity was 20/25 in both eyes. Slit lamp examination demonstrated anterior uveitis, vitritis and multifocal, yellowish, subretinal, placoid lesions within the posterior pole. On fundus fluorescein angiography (FFA), the placoid lesions appeared hypofluorescent in the early phases and became hyperfluorescent in the late frames. On indocyanine green angiography (ICGA), hypofluorescent spots were present across all phases of the exam. Spectral-domain optical coherence tomography (SDOCT) demonstrated disruption of the outer retinal and ellipsoid zone. These features were consistent with the diagnosis of APMPPE. A uveitis work-up was unremarkable. Treatment with dexamethasone eye drops was initiated. Eight weeks after initial presentation, the patient was admitted on the neurology service for an acute episode of right-sided weakness as well as dysarthria. Magnetic resonance imaging (MRI) showed a small subacute infarct in the left posterior limb of the internal capsule and a small aneurysm of the right middle cerebral artery. An extensive laboratory work-up revealed hypercysteinemia with lupus anticoagulant detected by diluted Russell Viper Venom Time (dRVVT). The patient was started on anticoagulant medication and a statin. His symptoms improved, and the neurological examination was normal at a follow-up visit three months later.
CONCLUSION: APMPPE is a well-established disease, and neurological complications are rare. Cerebral vasculitis and strokes are the most commonly reported neurological lesions. The search for antiphospholipid syndrome and hypercysteinemia in the first-line work-up of APMPPE should be considered.
METHODS: Case report.
RESULTS: A 28-year-old man complained of blurry vision and red eyes. On exam, his visual acuity was 20/25 in both eyes. Slit lamp examination demonstrated anterior uveitis, vitritis and multifocal, yellowish, subretinal, placoid lesions within the posterior pole. On fundus fluorescein angiography (FFA), the placoid lesions appeared hypofluorescent in the early phases and became hyperfluorescent in the late frames. On indocyanine green angiography (ICGA), hypofluorescent spots were present across all phases of the exam. Spectral-domain optical coherence tomography (SDOCT) demonstrated disruption of the outer retinal and ellipsoid zone. These features were consistent with the diagnosis of APMPPE. A uveitis work-up was unremarkable. Treatment with dexamethasone eye drops was initiated. Eight weeks after initial presentation, the patient was admitted on the neurology service for an acute episode of right-sided weakness as well as dysarthria. Magnetic resonance imaging (MRI) showed a small subacute infarct in the left posterior limb of the internal capsule and a small aneurysm of the right middle cerebral artery. An extensive laboratory work-up revealed hypercysteinemia with lupus anticoagulant detected by diluted Russell Viper Venom Time (dRVVT). The patient was started on anticoagulant medication and a statin. His symptoms improved, and the neurological examination was normal at a follow-up visit three months later.
CONCLUSION: APMPPE is a well-established disease, and neurological complications are rare. Cerebral vasculitis and strokes are the most commonly reported neurological lesions. The search for antiphospholipid syndrome and hypercysteinemia in the first-line work-up of APMPPE should be considered.