A Case of Hypertrophic Pachymeningitis Presenting Bitemporal Hemianopia

• Published in: Neuro-Ophthalmology Japan Vol. 36; no. 1; pp. 60 - 65
• Main Authors: Fukuda, Miho, Kimura, Akiko, Masuda, Akiko, Okamoto, Mana, Mimura, Osamu, Gomi, Fumi
• Format: Journal Article
• Language: Japanese
• Published: The Japanese Neuro-Ophthalmology Society 25.03.2019
• Subjects: bitemporal hemianopia; hypertrophic pachymeningitis; rheumatoid arthritis
• ISSN: 0289-7024; 2188-2002
• DOI: 10.11476/shinkeiganka.36.60

Patients with hypertrophic pachymeningitis have onset of visual deterioration due to inflammatory change involving the visual pathway usually accompanied by severe pain. We report a 56-year-old female patient with hypertrophic pachymeningitis who initially presented chronic headache and bitemporal hemianopia. She visited our hospital complaining of the loss of vision and visual field defects of 1-month duration and exacerbated headache of few days’ duration, that was associated with chronic ocular pain and headache for 1 year. She had suffered from rheumatoid arthritis（RA）for a decade which was well controlled by immunosuppressive drugs; serological test revealed the absence of auto-antibodies and rheumatoid factor. Visual acuity was（0.4）at OD and（0.6）at OS, and there was limitation of supraduction and moderate mydriasis at OD. Static visual field examination revealed bitemporal hemianopia. Enhanced magnetic resonance imaging revealed the presence of the enhanced thickened pachymeninx at the base of the skull and pituitary gland swelling due to inflammation spread to the right cavernous sinus and pituitary gland. We diagnosis of hypertrophic pachymeningitis was made, and subsequently, steroid mini-pulse therapy was administered at our hospitals’ Department of Internal Medicine. Immediately following steroid therapy, her bilateral visual acuity improved to（1.2）； the limitation of supraduction at OD improved 2 months later and bitemporal hemianopia improved 4 months later. Our findings suggested that the patient developed bitemporal hemianopia due to compression of the pituitary gland caused by inflammatory change involving the pituitary gland with accompanied chronic mild headache since severe headache was masked by the long-term use of immunosuppressive and analgesic drugs. Patients with hypertrophic pachymeningitis with RA as underlying disease were rarely reported; this case report highlights the atypical clinical course of the disease.