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

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.