2026 Volume 49 Issue 2 Pages 81-87
During a routine physical examination, a 63-year-old male patient was found to have bilateral papilledema. The patient reported no subjective symptoms, and general physical and neurological examinations were unremarkable. His serum IgG4 level was elevated (563 mg/dL). Contrast-enhanced brain magnetic resonance imaging (MRI) demonstrated enhancement of soft tissue in the bilateral parasellar regions and paracavernous sinuses, as well as bilateral lacrimal gland enlargement and thickening of the right nasal mucosa. Biopsy of the right nasal mucosa revealed significant infiltration of IgG4-positive plasma cells and fibrosis, leading to a diagnosis of IgG4-related hypertrophic pachymeningitis. As the patient remained asymptomatic, observation was undertaken. The papilledema subsequently resolved spontaneously and serum IgG4 level decreased gradually. Space-occupying lesions in the vicinity of the cavernous sinus may give rise to congestive papilledema. These lesions are often difficult to detect on standard non-contrast brain MRI, highlighting the importance of performing contrast-enhanced MRI in patients with bilateral papilledema. Obtaining a tissue diagnosis can be challenging when IgG4-related disease lesions are localized to the head and neck region. In cases such as the one presented here, where IgG4-related disease is suspected and there is nasal mucosal thickening as well as elevation of the serum IgG4 level, nasal biopsy may provide valuable diagnostic information.
