High-dose steroid therapy for idiopathic optic perineuritis: a case series
© Tatsugawa et al; licensee BioMed Central Ltd. 2010
Received: 22 December 2009
Accepted: 10 December 2010
Published: 10 December 2010
It has been reported that the prognosis of optic perineuritis may be poor when initiation of treatment is delayed. Here we report the successful treatment of three patients with idiopathic optic perineuritis, including two in whom initiation of therapy was delayed.
Three Japanese patients (two women aged 73 and 66 years, and one man aged 27 years) presented with loss of vision (for five months, several months, and two months respectively) and pain on eye movement in the third case only, and were diagnosed as having idiopathic optic perineuritis. Fat-suppressed T2-weighted magnetic resonance images showed high signal intensity areas around the affected optic nerves, suggesting the presence of optic perineuritis. Two patients received steroid pulse therapy and the third was given high-dose steroid therapy. The visual acuity improved in all three cases.
High-dose steroid therapy may be effective for idiopathic perineuritis in patients without optic nerve atrophy, even if initial treatment (including moderate-dose steroids) has failed.
Idiopathic optic perineuritis has been reported as a type of orbital inflammatory pseudotumor [1–3]. Currently, the diagnosis of optic perineuritis is most commonly based on magnetic resonance image (MRI) findings along with the clinical characteristics. Although some reported cases have been diagnosed by pathologic examinations, the distinction between optic neuritis and optic perineuritis is generally radiographic . The characteristic differences between idiopathic optic perineuritis and idiopathic optic neuritis are as follows : The age distribution of the former is wide and it particularly affects elderly patients, and a paracentral scotoma or an arcuate defect are frequent findings. The onset is slow (usually over several weeks), and recovery is often poor in patients with optic perineuritis when treatment is delayed. The response to corticosteroids is often dramatic, although recurrence is common with tapering of therapy. Here, we report the successful treatment of three patients with idiopathic optic perineuritis who received high-dose steroid therapy.
The prognosis of optic perineuritis has been reported to be poor when initiation of treatment is delayed . However, our first two cases both responded well to steroid therapy and achieved a good visual prognosis, despite the interval between the onset of symptoms and initiation of treatment being longer than six months. Concerning the steroid dose, recurrence was observed in Case 2 after treatment with prednisolone at a daily dose of 40 mg in the early stage of her illness, and Case 1 showed recurrence after receiving prednisolone at a dose of 30 mg/day at her previous hospital. After we performed steroid pulse therapy at our hospital for Cases 1 and 3, there was no recurrence in Case 3, and no subsequent recurrence in Case 1. Purvin et al. reported recurrence of optic perineuritis in 4 out of 14 patients treated with oral steroids at doses of 60-80 mg/day .
Perimetry was performed up to isopter I-1e in all of our patients. The innermost isopter of the central visual field that showed a response was isopter I-4e in Case 1, isopter I-2e in Case 2, and isopter I-1e in Case 3. Thus, Cases 1 and 2 did not respond to isopter I-1e, suggesting the presence of central depression. Perimetry of the peripheral visual fields including the paracentral field revealed arcuate constriction on the downside of isopter V-4e in Case 1, who showed a generalized decrease of sensitivity. In Case 2, depression was seen on the upside of Mariotte's blind spot in isopter I-4e, indicating a paracentral scotoma. In Case 3, scotomata were observed at three sites on the upside of Mariotte's blind spot in isopter III-4e. These results suggest that reduced vision was at least partly ascribable to a decrease of central visual field sensitivity in Cases 1 and 2, whereas vision was reduced despite the lack of a central scotoma or reduction of central field sensitivity in Case 3. Therefore, the reduced visual acuity was related to central or generalized depression of vision due to optic perineuritis in Cases 1 and 2. In Case 3, vision may have been reduced because scotomata involved the fixation point.
This case series had the following limitation. The best diagnostic sequence for optic perineuritis is post-contrast fat-suppressed T1-weighted images. On other images, the area of hyperintensity around the optic nerve could represent an increase of cerebrospinal fluid that would occur if there was optic atrophy. However, we did not obtain fat-suppressed T1-weighted images in all three cases, although such images are required for the definite diagnosis of optic perineuritis.
Two patients with optic perineuritis who underwent steroid pulse therapy showed no recurrence, as did one patient receiving high-dose prednisolone. Our results suggest that steroid pulse therapy or high-dose prednisolone may be effective for idiopathic optic perineuritis in patients without optic nerve atrophy, even if initial treatment (including moderate-dose steroids) has failed.
Written informed consent was obtained from the patients for publication of this case series and any accompanying images. Copies of the written consents are available for review by the Editor-in-Chief of this journal.
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