Bing-Neel Syndrome: An Unknown GCA Mimicker

Area of Science:

• Neurology and Ophthalmology
• Rheumatology
• Hematology

Background:

• Giant cell arteritis (GCA) is a vasculitis affecting medium and large arteries, often presenting with cranial symptoms.
• Temporal artery biopsy, the gold standard for GCA diagnosis, has limited sensitivity.
• Bing-Neel Syndrome (BNS) is a rare central nervous system complication of Waldenstrom Macroglobulinemia (WM).

Observation:

• A 77-year-old female presented with progressive vision loss.
• Ophthalmic findings included pseudophakic pseudopallor and dot blot hemorrhages.
• Temporal artery biopsy confirmed GCA spectrum.

Findings:

• Workup revealed paraproteinemia and bone marrow biopsy confirmed Waldenstrom Macroglobulinemia (WM).
• The patient's presentation was consistent with Bing-Neel Syndrome (BNS) manifesting as GCA.
• Treatment for WM led to stabilization of ophthalmic complications.

Implications:

• This case underscores the importance of considering BNS in patients presenting with biopsy-proven GCA, especially with hematologic abnormalities.
• Early diagnosis and treatment of WM are crucial for managing BNS and its associated complications, including ophthalmic manifestations.
• The findings expand the understanding of GCA's differential diagnosis and the diverse clinical spectrum of WM.