Orbital apex-like syndrome with multiple cranial neuropathies secondary to chronic inflammatory demyelinating polyneuropathy.

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder characterized by progressive demyelination of peripheral nerves, leading to motor and sensory deficits. Ophthalmologic involvement, though uncommon, may manifest as ophthalmoplegia, papilledema, optic neuropathy, or proptosis. This report presents a 49-year-old male with refractory CIDP and extensive orbital involvement, including bilateral proptosis and enlargement of multiple cranial nerves, resulting in an orbital apex-like syndrome and compressive optic neuropathy. The patient's condition was refractory to medical management with intravenous immunoglobulin, corticosteroids, plasma exchange, and rituximab, and he ultimately required surgical decompression. Improvement in visual symptoms was noted in the early post-operative period. This case highlights the importance of a multidisciplinary approach in the management of complex CIDP cases with significant cranial nerve involvement and underscores the need for further research into its pathophysiology and treatment optimization.