Abstract

Purpose: To evaluate the diagnostic yield of a structured systemic workup in patients presenting with acute visual symptoms who were diagnosed with isolated paracentral acute middle maculopathy (iPAMM).

Design: Retrospective cohort study.

Participants: Thirty-seven patients with new-onset visual symptoms who were diagnosed with isolated PAMM at Sheba Medical Center between 2015 and 2025.

Methods: Clinical records and multimodal imaging were reviewed. Systemic workup included brain imaging (magnetic resonance imaging [MRI] or computed tomography angiography [CTA]), carotid imaging (Doppler or CTA), echocardiography (transesophageal echocardiography or transthoracic echocardiography), and Holter monitoring in selected cases. Demographics, systemic comorbidities, ocular features, and visual outcomes were collected. Associations among baseline characteristics, PAMM subtype, and abnormal systemic findings were analyzed.

Main outcome measures: Prevalence and type of abnormal systemic findings; correlation with age, PAMM subtype, and baseline characteristics.

Results: Thirty-six patients (97%) underwent at least 1 component of systemic workup, and 24 patients completed the full evaluation. Among those with any workup, abnormal findings were detected in 21 of 33 patients (63.6%). Brain imaging showed abnormal findings in 16 of 29 patients (55%), carotid imaging showed abnormal findings in 8 of 33 patients (24%), and echocardiography showed abnormal findings in 6 of 26 patients (23.1%); Holter monitoring revealed no abnormalities. Nine patients (25%) had a documented history of stroke, but 16 patients showed abnormalities on brain imaging, indicating that approximately half of cerebrovascular disease detected was previously unrecognized. The diagnostic yield increased with age: 0% in patients aged ≤40 years, 66.7% in those aged 41-50 years, 50% in those aged 51-64 years, and 87.5% in those aged ≥65 years (chi-square = 11.75, P = 0.008; trend P = 0.002). Carotid disease was associated with globular PAMM subtype (P = 0.024).

Conclusions: Systemic workup in patients with acutely symptomatic iPAMM frequently uncovers clinically significant and often silent cerebrovascular and cardiovascular pathology, supporting targeted workup in this specific population. The diagnostic yield increased with age and was specifically high in patients over 65 years old. These findings should not be generalized to patients in whom PAMM is detected incidentally without acute symptoms. Prospective studies are needed to determine optimal imaging and evaluation criteria in broader clinical settings.