J Korean Ophthalmol Soc.  2011 Jul;52(7):887-892. 10.3341/jkos.2011.52.7.887.

A Case of Moyamoya Disease Initially Presenting as Anterior Ischemic Optic Neuropathy

• Cho A
• Kim SY

• KMID: 2214717
• DOI: http://doi.org/10.3341/jkos.2011.52.7.887

Abstract

PURPOSE
To present a case of a patient with decreased visual acuity and anterior ischemic optic neuropathy diagnosed with advanced Moyamoya disease.
CASE SUMMARY
A 46-year-old woman presented sudden episodes of headache and decreased visual acuity. On her first visit, her best corrected visual acuity was 20/25 in the right eye and 20/70 in the left eye. The left eye pupil was dilated with a relative afferent papillary defect. Fundus examinations revealed disc swelling in the left eye. After being admitted, steroid pulse therapy was started and Magnetic Resonance Imaging (MRI) studies revealed Moyamoya disease. The diagnosis was confirmed via Magnetic Resonance Angiography (MRA). After steroid pulse therapy, the headaches and visual acuity improved and the patient is continuing follow-up visits at neurosurgery and ophthalmology clinics.
CONCLUSIONS
Patients with Moyamoya disease may initially present symptoms and signs of anterior ischemic optic neuropathy.

Keyword

Moyamoya disease; Optic neuropathy; Steroid pulse therapy

MeSH Terms

Eye
Female
Follow-Up Studies
Headache
Humans
Magnetic Resonance Angiography
Magnetic Resonance Imaging
Middle Aged
Moyamoya Disease
Neurosurgery
Ophthalmology
Optic Nerve Diseases
Optic Neuropathy, Ischemic
Pupil
Visual Acuity

Figure

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  Figure 1. Fundus photographs show marked disc swelling in the left eye and mild disc swelling in the right eye.

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  Figure 2. The visual field exams on first visit show pericentral scotomas in the right eye and diffuse scotoma in the left eye.

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  Figure 3. The left eye VEP shows a decrease in amplitude. (A) Pattern VEP. (B) Flash VEP.

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  Figure 4. T2-weighted brain MRI shows increased tiny signal voids in both middle cerebral artery trunks (R/O Moyamoya disease).

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  Figure 5. Brain MRA and cerebral angiography show stenosis of both internal carotid arteries and development of abnormal vascular networks (moyamoya vessels) at the base of the brain.

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  Figure 6. The visual field exams at last visit show improved field defects in both eyes.

Reference

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