Clinical Manifestations and Treatment of Idiopathic Optic Perineuritis

Hyo Cheol Lim; Hee Young Choi; jae Hwan Choi; Jae Ho Jung

doi:10.3341/jkos.2014.55.6.891

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Lim, Choi, Choi, and Jung: Clinical Manifestations and Treatment of Idiopathic Optic Perineuritis

Original Article

J Korean Ophthalmol Soc 2014;55(6):891-897.

Published online: 30 August 2014

DOI: https://doi.org/10.3341/jkos.2014.55.6.891

Clinical Manifestations and Treatment of Idiopathic Optic Perineuritis

Hyo Cheol Lim, MD¹, Hee Young Choi, MD, PhD², jae Hwan Choi, MD, PhD³, Jae Ho Jung, MD, PhD¹

¹Department of Ophthalmology, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan, Korea

²Department of Ophthalmology, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea

³Department of Neurology, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan, Korea

Address reprint requests to Jae Ho Jung, MD, PhD, Department of Ophthalmology, Pusan National University, Yangsan Hospital, #20 Geumo-ro, Mulgeum-eup, Yangsan, 626-770, Korea, Tel: 82-55-360-2591, Fax: 82-55-360-2161, E-mail: jungjaeho@pusan.ac.kr

Received 8 July 2013 Revised 6 September 2013 Accepted 24 April 2014

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 investigate the clinical and radiologic manifestations of idiopathic optic perineuritis (OPN), and to evaluate the out-comes of steroid treatment for OPN.

Methods

We reviewed the medical records and radiologic findings of 10 patients (13 eyes) who were diagnosed with OPN and treated with steroid.

Results

The mean age was 56.5 ± 9.3 years (range, 35-77 years) and the sex ratio was equal. The main complaint was de-crease in visual acuity combined with ocular pain during extraocular eye movement in 9 patients. The median visual acuity at the first visit was 0.2 (HM-0.8) and the relative afferent papillary defect was observed in 12 eyes. Additionally, combined orbital dis-eases included posterior scleritis in 1 eye and myositis in 1 eye. Orbit magnetic resonance imaging (MRI) scans demonstrated intraorbital optic nerve sheath enhancement in all patients, occasionally with orbital fat involvement. All patients demonstrated improved visual acuity after high-dose oral steroid therapy (6 patients) or intravenous (IV) pulse steroid therapy (4 patients). Relapse occurred in 4 patients during steroid tapering.

Conclusions

The population in this study was composed predominantly of patients with OPN in their 50's. The primary symptom of OPN was visual acuity decrease combined with ocular pain during extraocular eye movement. Radiologically, orbit MRI scans demonstrated intraorbital optic nerve sheath enhancement. The patients in this study demonstrated good responses to steroid treatment, but clinicians must be aware of the high recurrence rate during steroid tapering in this condition. A combination of clin-ical and radiologic findings was helpful to diagnose OPN.

Keywords: Optic neuritis, Optic perineuritis, Orbital inflammation

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Figure 1.

Right optic disc swelling was observed in the fundus photography (A). Right visual field was seen in Humphery visu-al field test (B). Fat suppression magnetic resonance imaging scan of the orbit with contrast enhancement in axial and coronal section showed that enhancement of the right optic nerve sheath and streakiness of the surrounding orbital fat (white arrow), and posterior sclera thickening in right eye (C, D).

Figure 2.

Right visual field test was seen diffuse visual field defect at initial visit (A). Fat suppression magnetic resonance imaging scan of the orbit with contrast enhancement demonstrated that tram track sign around optic nerve sheath in axial view and donut appearance in optic nerve sheath in coronal view (white arrow) (B, C). Right visual field test revealed rapid improve-ment of visual field defect after steroid treatment, post-treatment day at 5 (D).

Table 1.

Clinical characteristics of idiopathic optic perineuritis patients

Number/Sex/Age	Initial Sx	VA	VF	Optic Disc edema	Final VA	Remarks
1/M/73	Decreased vision	0.02	Altitudinal defect	+		Pulse
	Ocular pain
2/F/42	Visual field defect	0.2	Central scotoma	-	0.4	PO
						Recurrence
3/F/69	Blurred vision	0.32	Central scotoma	−	0.63	PO
	Ocular pain					Recurrence
4/F/35	Ocular pain	0.2/0.2	Cecocentral scotoma/Altitudinal defect	+/+	0.8/0.8	PO
	Blurred vision
5/M/62	Decreased vision	0.1	Diffuse	+	0.63	PO
	Ocular pain
6/M/55	Ocular pain	0.8	Altitudinal defect	+	1.0	Pulse
	Blurred vision					Posterior Scleritis
						Recurrence
7/F/62	Decreased vision	CF	Diffuse	+	0.8	Pulse
	Ocular pain
8/M/52	Ocular pain	CF	Central scotoma	−	0.2	Pulse
	Decreased vision					Recurrence
9/M/77	Ocular pain	CF/0.5	Diffuse/Diffuse	-/-	0.2/0.63	PO
	Decreased vision
10/F/42	Ocular pain	0.6/0.6	Diffuse/Diffuse	+/ +	0.8/0.8	PO
	Decreased vision					Myositis

Sx = symptom; VA = visual acuity; VF = visual field; CF = counting finger; Pulse = intravenous (IV) pulse steroid therapy; PO = per oral high dose steroid therapy.

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