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

Aran Cho; So Young Kim

doi:10.3341/jkos.2011.52.7.887

Journal List > J Korean Ophthalmol Soc > v.52(7) > 1009092

Go to TopGo to Top Go to BottomGo to Bottom

TOOLS

Cho and Kim: A Case of Moyamoya Disease Initially Presenting as Anterior Ischemic Optic Neuropathy

Original Article

J Korean Ophthalmol Soc 2011;52(7):887-892.

Published online: 7 September 2011

DOI: https://doi.org/10.3341/jkos.2011.52.7.887

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

Aran Cho, MD¹, So Young Kim, MD, PhD^1,²

¹Department of Ophthalmology, Soonchunhyang University College of Medicine, Seoul, Korea

²Department of Ophthalmology, Soonchunhyang University Cheonan Hospital, Cheonan, Korea

Address reprint requests to So Young Kim, MD, PhD Department of Ophthalmology, Soonchunhyang University Cheonan Hospital #23-20 Bongmyeong-dong, Dongnam-gu, Cheonan 330-720, Korea Tel: 82-41-530-2260, Fax: 82-41-576-2262, E-mail: ophdrkim@schch.ac.kr

Received 17 March 2010 Revised 12 January 2011 Accepted 12 April 2011

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Keywords: Moyamoya disease, Optic neuropathy, Steroid pulse therapy

References

1. David CA, Nottmeier E. Intracranial occlusion disease: Moyamoya. In: Winn HR ed. Youmans Neurological Surgery. 5th ed.Philadelphia: Saunders;2004. 2:chap. 103.

2. Noda S, Hayasaka S, Setogawa T, Matsumoto S. Ocular symptoms of moyamoya disease. Am J Ophthalmol. 1987; 103:812–6.

3. Takeuchi K. Occlusive diseases of the carotid artery. Shinkei Shimpo. 1961; 5:511–43.

4. Suzuki J, Takaku A. Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol. 1969; 20:288–99.

5. Kuriyama S, Kusaka Y, Fujimura M, et al. Prevalence and clin-icoepidemiological features of moyamoya disease in Japan: findings from a nationwide epidemiological survey. Stroke. 2008; 39:42–7.

6. Phi JH, Wang KC, Cho BK, Kim SK. Pediatric cerebrovascular disease. Korean J Pediatr. 2008; 51:1282–9.

7. Robertson RL, Burrows PE, Barnes PD, et al. Angiographic changes after pial synangiosis in childhood moyamoya disease. AJNR Am J Neuroradiol. 1997; 18:837–45.

8. Miyamoto S, Kikuchi H, Karasawa J, et al. Study of the posterior circulation in moyamoya disease. Part 2: Visual disturbances and surgical treatment. J Neurosurg. 1986; 65:454–60.

9. Chen CS, Lee AW, Kelman S, Wityk R. Anterior ischemic optic neuropathy in moyamoya disease: a first case report. Eur J Neurol. 2007; 14:823–5.

10. Barrall JL, Summers CG. Ocular ischemic syndrome in a child with moyamoya disease and neurofibromatosis. Surv Ophthalmol. 1996; 40:500–4.

11. Ushimura S, Mochizuki K, Ohashi M, et al. Sudden blindness in the fourth month of pregnancy led to diagnosis of moyamoya disease. Ophthalmologica. 1993; 207:169–73.

12. Harissi-Dagher M, Sebag M, Dagher JH, Moumdjian R. Chorioretinal atrophy in a patient with moyamoya disease. Case report. J Neurosurg. 2004; 101:843–5.

13. Chace R, Hedges TR 3rd. Retinal artery occlusion due to moyamoya disease. J Clin Neuroophthalmol. 1984; 4:31–4.

14. Lee YW, Lee SN. A case of bilateral upgaze palsy associated with unilateral midbrain hemorrhage in moyamoya disease. J Korean Ophthalmol Soc. 2004; 45:1772–6.

15. Khan N, Schinzel A, Shuknecht B, et al. Moyamoya angiopathy with dolichoectatic internal carotid arteries, patent ductus arterio-sus and pupillary dysfunction: a new genetic syndrome? Eur Neurol. 2004; 51:72–7.

16. Krishnan C, Roy A, Traboulsi E. Morning glory disk anomaly, choroidal coloboma, and congenital constrictive malformations of the internal carotid arteries (moyamoya disease). Ophthalmic Genet. 2000; 21:21–4.

Figure 1.

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

Figure 2.

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

Figure 3.

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

Figure 4.

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

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.

Figure 6.

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

TOOLS

Similar articles