# Intracranial Major Artery Stenosis Associated With Acute Posterior Multifocal Placoid Pigment Epitheliopathy

## TL;DR

A 16-year-old girl with APMPPE developed cerebral infarctions and artery stenosis, which improved with immunotherapy and antiplatelet drugs.

## Contribution

Highlights the association between APMPPE and intracranial artery stenosis in young patients.

## Key findings

- APMPPE can cause cerebral infarctions and intracranial artery stenosis in juveniles.
- Immunotherapy and antiplatelet drugs resolved arterial stenosis and neurological symptoms.
- Vascular stenosis may progress despite immunotherapy, requiring close monitoring.

## Abstract

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an immune-mediated chorioretinal disease that can cause cerebral infarctions in young individuals. We report the case of a 16-year-old girl who developed intracranial major artery stenosis. She presented with eye pain and microcerebral infarctions, and CSF examination showed lymphocytosis and elevated interleukin-6 (IL-6). Ophthalmoscopy revealed multiple white posterior pole lesions; hence, she was diagnosed with cerebral infarction due to APMPPE. Five weeks after the initiation of prednisolone (PSL) treatment, she developed asymptomatic intracranial arterial stenosis. The addition of intravenous methylprednisolone (IVMP) and antiplatelet drugs resulted in the complete resolution of arterial stenosis and neurological symptoms.

It is important to recognize APMPPE as a potential cause of juvenile cerebral infarction accompanied by ocular symptoms. In addition, careful monitoring for intracranial major artery stenosis is warranted, as vascular stenosis may progress despite immunotherapy.