Purpose: Identifying the etiology of orbital apex syndrome is important because of its diverse causes. However, no studies on this disease have been conducted with a large sample size. Therefore, we have described the disease etiology, treatment, and rate of recurrence in a case series from our department. Method: Nineteen patients with orbital apex syndrome visited our department from 2014 to 2018 (men/women: 6/13; average age: 68.6 ± 13.4 years). Diagnoses were made based on the clinical course, visual acuity, visual field, and magnetic resonance imaging findings by a neuroophthalmologist. The clinical characteristics were determined from medical records. Results: The causes of the disease were idiopathic; antineutrophil cytoplasmic antibody-associated vasculitis, such as polyangiitis granulomatosis; fungal infection; immunoglobulin G4-related disease; oncogenicity; and other autoimmune diseases in 7, 5, 3, 2, 1 and 1 case, respectively. Steroid pulse therapy was used in 14 of the 19 cases, with 8 showing improvement to a corrected visual acuity of 1.0 or more. Cases of fungal infections or neoplasms showed no improvement. Conclusion: In case of orbital apex syndrome, a definitive diagnosis is often difficult at the initial examination, and many cases are associated with systemic diseases, necessitating cooperation with specialists from different medical fields to reliably identify the underlying etiology.
- Antineutrophil cytoplasmic antibody associated vasculitis
- Hypertrophic pachymeningitis
- Orbital apex syndrome
ASJC Scopus subject areas
- Clinical Neurology