To discuss the clinical presentation and management of idiopathic hypertrophic pachymeningitis that presented with multiple cranial neuropathies.

This is a case report.

A 37-year-old female presented with right-sided headache and ipsilateral cranial nerve (CN) I, II, III, IV, and V deficits which improved with nonsteroidal anti-inflammatory drugs (NSAIDs). Six months later she developed bilateral blurring of vision with pain on eye movement, which progressed to severe bilateral affectation that prompted admission and treatment with high-dose methylprednisolone therapy followed by prolonged oral steroid treatment which was gradually tapered. Recurrence was treated with oral steroids and azathioprine. Diagnostic modalities included an initial cranial contrast computed tomography (CT) scan which was inconclusive; contrast magnetic resonance imaging (MRI) clinched the diagnosis, as well as demonstrated thickening of the optic nerve perineurium. Visual field analysis 30-2 as well as optic nerve head optical coherence tomography (OCT) were used to support the diagnosis and document optic nerve affectation. Biologic testing was used to rule out tuberculosis, syphilis, fungal infection, granulomatosis with polyangiitis, polyarteritis nodosa, and rheumatoid arthritis. The patient had complete vision recovery in the left eye but only partial vision recovery in the right eye.

Pachymeningitis should be a diagnostic consideration in patients with headache and multiple cranial neuropathies. Clinicians should always perform independent evaluation of diagnostic modalities with the patient’s clinical presentation in mind. Pachymeningitis can involve the perineurium through contiguous spread of the lesion and present as optic perineuritis as well, with more insidious progression and lasting deficits than isolated optic perineuritis.