Case Report: IgG4-related disease presenting with prominent oculomotor nerve palsy - Report - MDSpire

Case Report: IgG4-related disease presenting with prominent oculomotor nerve palsy

  • By

  • Lishi Yu

  • Weidong Huang

  • Yifei Xu

  • Chunhong Zhang

  • Honghua Lv

  • Wenhui Lei

  • July 9, 2026

  • 0 min

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Clinical Report: Prominent Oculomotor Nerve Palsy as a Presentation of IgG4-RD

Overview

This case study presents a 76-year-old man with oculomotor nerve palsy as a rare manifestation of IgG4-related disease (IgG4-RD). The diagnosis was confirmed through laboratory evaluations and histopathology.

Background

IgG4-RD is a systemic fibroinflammatory disorder that can involve various organs, but cranial nerve involvement is exceptionally rare. Oculomotor nerve palsy may be misdiagnosed due to nonspecific symptoms.

Data Highlights

Case study of a 76-year-old man with bilateral plantar numbness, nausea, vomiting, and headache leading to the diagnosis of IgG4-RD. Laboratory tests showed elevated immunoglobulins, and histopathology confirmed IgG4-RD.

Key Findings

  • Oculomotor nerve palsy is a rare presentation of IgG4-RD, with an incidence of less than 1% among patients.
  • Histopathology revealed reactive lymphoid hyperplasia with abundant IgG4+ plasma cell infiltration.
  • Contrast-enhanced MRI showed no structural abnormalities.
  • Treatment with immunosuppressive therapy was initiated.
  • IgG4-RD should be considered in the differential diagnosis of unexplained cranial neuropathies.

Clinical Implications

IgG4-RD should be considered in patients presenting with unexplained cranial nerve palsies.

Conclusion

This case highlights the need for recognition of IgG4-RD as a potential cause of oculomotor nerve palsy.

Related Resources & Content

  1. Frontiers in Immunology, 2026 -- Case Report: Presenting as optic neuritis—a biopsy-proven IgG4 anti-NF155–positive combined central and peripheral demyelination syndrome
  2. Frontiers in Immunology, 2026 -- Case report: The masquerading spectrum: a pediatric case series of IgG4-related disease
  3. Hypertrophic Pachymeningitis Associated with IgG4-Related Disease Featuring Tumor-like Lesions in the Brain
  4. Frontiers in Neurology, 2026 -- Case Report: A rare case of ocular giant cell arteritis with bilateral orbital inflammation and diagnostic challenges
  5. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease | Annals of the Rheumatic Diseases
  6. Inebilizumab for Treatment of IgG4-Related Disease - PubMed
  7. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease
  8. Inebilizumab for Treatment of IgG4-Related Disease - PubMed

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