Purpose: To report the case of a patient with acute posterior multifocal placoid pigment epitheliopathy (APMPPE) associated with cerebral infarction, and its associated literature review from overseas.
Case: A 29-year-old man suffered from APMPPE after a fever and subsequent general fatigue for 2 months. On conducting OCT angiography imaging of the inner choroid, hypoperfusion was found at the lesions. He developed an acute brainstem infarction in the pontine 1 month after spontaneous disappearance of APMPPE. Magnetic resonance angiography showed focal stenosis of the right posterior cerebral artery, suggesting vasculitis. The cerebrospinal fluid examination showed lymphocytic pleocytosis and increased concentrations of protein and IL-6. The patient was treated with oral corticosteroid for 10 months and recovered without sequelae. We reviewed 14 case reports describing APMPPE associated with cerebral infarction. The mean latency from the onset of APMPPE to cerebral infarction was approximately 2 months. The common neurological complications included headache (66.7%), cerebrovascular stenosis (90.9%), and cerebrospinal pleocytosis (83.3%). In a majority of cases, new-onset cerebral infarctions occurred during the corticosteroid treatment.
Conclusions: When severe headaches or neurological symptoms are associated with APMPPE, MRI and CSF examinations for suspected lesions in the central nervous system should be proactively initiated. The long-term use of high-dose corticosteroid should be considered for such patients.
Nippon Ganka Gakkai Zasshi (J Jpn Ophthalmol Soc) 123: 969-976, 2019.