J Korean Ophthalmol Soc.  2011 Jul;52(7):893-896. 10.3341/jkos.2011.52.7.893.

Acute Bilateral Visual Loss with Idiopathic Hypertrophic Pachymeningitis

• Lee YH
• Kim JY
• Nam KY

• KMID: 2214718
• DOI: http://doi.org/10.3341/jkos.2011.52.7.893

Abstract

PURPOSE
To report a case of bilateral optic neuropathy related with idiopathic hypertropic pachymeningitis.
CASE SUMMARY
A 66-year old woman presented with acute visual loss that developed 6 days previously in the right eye and 3 days prior in the left eye. During the initial evaluation, her visual acuity was light perception in the right eye and counting fingers at 30 cm in the left eye. A relative afferent papillary defect (RAPD) was noted in the patient's right eye. On brain MRI, the duramater was thickened and enhanced by contrast. The erythrocyte Sedimentation Rate (ESR) and C-reactive protein (CRP) level were increased and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) as positive. Other neurological and medical abnormalities were not found. Under the suspicion of bilateral optic neuropathy due to idiopathic hypertrophic pachymeningitis, the patient was treated with methylprednisolone pulse therapy. Two days after the treatment, the visual acuity was hand movements at 20 cm in the right eye and 0.6 in the left eye. Three months after the treatment, the visual acuity was counting fingers at 20 cm in the right eye, and 0.7 in the left eye. The right eye showed optic disc pallor and diffuse retinal nerve fiber layer (RNFL) defect. The left eye showed a suspicious RNFL defect, and was otherwise normal.
CONCLUSIONS
The authors report a rare case of optic neuropathy related with idiopathic hypertrophic pachymeningitis. Idiopathic hypertrophic pachymeningitis should be considered as one of the various causes of optic neuropathy.

Keyword

Idiopathic hypertrophic pachymeningitis; Optic neuropathy

MeSH Terms

Blood Sedimentation
Brain
C-Reactive Protein
Cytoplasm
Eye
Female
Fingers
Hand
Humans
Light
Meningitis
Methylprednisolone
Nerve Fibers
Optic Nerve Diseases
Pallor
Patient Rights
Retinaldehyde
Visual Acuity
C-Reactive Protein
Methylprednisolone
Retinaldehyde

Figure

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  Figure 1. Brain MRI of the patient at initial visit. Thickening of dura mater (black arrow) is seen on T2 FLAIR image (A) and enhancement of dura mater (black arrow) is noted on T1 enhanced image (B).

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  Figure 2. The fundus photographs at three months after initial presentation. The photographs show the pale optic disc, diffuse retinal nerve fiber layer (RNFL) defect and Roth's spot nasal to the optic disc in the right eye (A), and suspious superotemporal RNFL defect in the left eye (B).

Reference

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