Ocular and Neuro-Ophthalmic Manifestations of Chronic Inflammatory Demyelinating Polyneuropathy

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated neuropathy characterized by demyelination and axonal injury of peripheral nerves. While its hallmark presentation involves progressive sensorimotor deficits, an underrecognized subset of patients develop ocular and neuro-ophthalmic manifestations. These include optic neuritis, optic atrophy, vision loss, ophthalmoplegia, extraocular muscle enlargement with proptosis, intracranial hypertension, and corneal small fiber pathology. Electrophysiologic and imaging studies, including visual evoked potentials, optical coherence tomography, and corneal confocal microscopy, have further revealed subclinical involvement of the optic nerve and retina, underscoring an expanded disease spectrum beyond classic peripheral neuropathy. The pathophysiology is multifactorial, involving demyelination, inflammatory cell infiltration, and immune-mediated axonal injury, with evidence suggesting potential central nervous system and cranial nerve involvement. These atypical manifestations often mimic disorders such as multiple sclerosis, neuromyelitis optica spectrum disorders, or thyroid eye disease, complicating early diagnosis. Importantly, most cases respond to standard immunomodulatory therapies, including intravenous immunoglobulin, corticosteroids, and plasma exchange. Advances in ocular imaging and biomarker discovery provide promising avenues for earlier detection and monitoring of neuro-ophthalmic involvement. Recognition of ocular features in CIDP is essential for timely diagnosis and treatment. Interdisciplinary collaboration between neurologists and ophthalmologists can prevent vision-threatening complications, refine diagnostic accuracy, and optimize long-term outcomes. Future studies are warranted to elucidate the mechanisms underlying ocular involvement and to evaluate emerging targeted therapies that may improve prognosis in these atypical but clinically significant phenotypes.