Neuro-Ophthalmic Manifestations of Hypertrophic Cranial Pachymeningitis Associated With Antineutrophil Cytoplasmic Antibody for Myeloperoxidase

Abstract

Hypertrophic cranial pachymeningitis is a rare inflammatory disease characterised by localised or diffuse thickening of the dura mater. We report a 68-year-old woman with hypertrophic cranial pachymeningitis presenting with subacute progressive bilateral visual loss and left abducens nerve palsy. On repeat fundoscopy, no remarkable findings were found, the patient was referred to our hospital 2 months after the onset of the ocular manifestations. Magnetic resonance imaging revealed thickening of the dura mater in the middle and anterior cranial fossae, which extended bilaterally to the vicinity of the optic canals and the superior orbital fissures. On immunological testing, elevated titres for antineutrophil cytoplasmic antibody for myeloperoxidase were found. The patient was immediately treated with high-dose methylprednisolone. After treatment, visual loss in the right eye and the left abducens nerve palsy improved significantly, but visual loss in the left eye remained unchanged. Careful systemic and radiological observations may be required when ophthalmic manifestations are seen in patients with positive antineutrophil cytoplasmic antibody for myeloperoxidase. The early initiation of treatment could prevent permanent damage to the optic nerve.

KEYWORDS::

• antineutrophil cytoplasmic antibody against myeloperoxydase (MPO-ANCA)
• cranial nerve palsy
• headache
• hypertrophic cranial pachymeningitis
• optic neuropathy

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

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