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Delayed recurrent stroke in a young patient with acute posterior multifocal placoid pigment epitheliopathy
José Manuel Matamala1, Walter Feuerhake, Renato Verdugo
1Department of Neurological Science, Faculty of Medicine, Universidad de Chile, Santiago, Chile.
Abstract:
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a chorioretinal disease that causes acute binocular visual disturbance with characteristic funduscopic lesions at the level of the retinal pigment epithelium. APMPPE has been associated with multiple neurologic complications, including cerebrovascular diseases. We report a 15-year-old patient who had bilateral APMPPE, which was successfully treated with corticosteroids. One year later he presented with transient dysarthria and right hemiparesis. Brain magnetic resonance imaging (MRI) showed bilateral ischemic lesions in both lenticular nuclei and corona radiata. Brain MRI performed 3 months later revealed a new asymptomatic ischemic lesion. Cerebral angiography showed diffuse multifocal segmental vessel narrowing. The cerebrospinal fluid showed mononuclear pleocytosis in keeping with vasculitis. We started corticosteroid treatment, which lasted 10 months. Currently, after 2 years of clinical and neuroradiologic follow-up, the patient is asymptomatic and shows no worsening of the cerebrovascular lesions.
Insights
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) can cause serious neurologic issues like stroke. This case shows successful corticosteroid treatment for APMPPE and its associated cerebrovascular complications.
Area of Science:
- Ophthalmology
- Neurology
- Immunology
Background:
- Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a chorioretinal inflammatory condition.
- APMPPE is linked to neurological complications, particularly cerebrovascular diseases.
Observation:
- A 15-year-old male with bilateral APMPPE developed transient dysarthria and hemiparesis a year after initial treatment.
- Brain MRI revealed new ischemic lesions, and angiography showed multifocal segmental vessel narrowing.
- Cerebrospinal fluid analysis indicated mononuclear pleocytosis, suggesting vasculitis.
Findings:
- Corticosteroid therapy was initiated for 10 months.
- The patient experienced a full recovery and remained asymptomatic with stable cerebrovascular lesions at 2-year follow-up.
Implications:
- This case highlights the importance of monitoring for and treating cerebrovascular complications in APMPPE patients.
- Corticosteroids appear effective in managing both the ocular and neurological manifestations of APMPPE.
- Early diagnosis and intervention are crucial for favorable outcomes in APMPPE-associated vasculitis.
