Neurol Sci DOI 10.1007/s10072-015-2310-2
LETTER TO THE EDITOR
Bilateral episcleritis followed by right optic perineuritis with severe visual loss: a case report Haruo Nishijima1 • Chieko Suzuki1 • Masahiko Tomiyama1
Received: 4 June 2015 / Accepted: 24 June 2015 Ó Springer-Verlag Italia 2015
Dear Editor, Persistent red eye may result in severe visual impairment. Scleritis is one possible cause of red eye that is considered to pose a significant threat to vision [1]. In contrast, episcleritis is confined to the superficial episcleral tissue and is thought to represent a mild, non-vision-threatening form of ocular inflammation, usually idiopathic in nature, that does not generally involve other ocular structures [1]. Idiopathic optic perineuritis has been reported as a type of orbital inflammatory pseudotumor [2, 3] with slow onset and often poor recovery in the event of delayed treatment [4]. Comorbid sclerotic inflammation and optic perineuritis have rarely been reported [5]. Here we describe a patient presenting with severe visual loss caused by bilateral episcleritis followed by right optic perineuritis. An 85-year-old woman was referred to our hospital with right visual loss. She had a past history of acute left visual loss and recovery with oral steroid therapy in her 50s, of which the details were unknown. Prior to referral, she had developed upper respiratory tract inflammation and visited her ophthalmologist 2 weeks later complaining of bilateral red eyes without ocular pain. At this time, her visual acuity was 20/40 with correction in each eye. She was diagnosed with episcleritis and treated with eye lotion containing corticosteroids. However, her eyes remained red 3 months later, and she noticed mild right ocular pain and decreased visual acuity in her right eye. She was finally referred to our
& Haruo Nishijima [email protected] 1
Department of Neurology, Aomori Prefectural Central Hospital, 2-1-1 Higashi-tsukurimichi, Aomori, Aomori 030-8553, Japan
department 2 months after the onset of visual impairment. On admission, her right visual acuity with correction was reduced to 20/200 and papilledema was detected in her right eye. No abnormal ocular movements were observed and her visual fields were normal. Fundus fluorescein angiography showed no apparent abnormalities. No other neurological deficit which might suggest multiple sclerosis or neuromyelitis optica spectrum disorders was detected. Other physical examinations were normal and there was no lymphadenopathy. Blood tests showed an erythrocyte sedimentation rate of 23 mm in the first hour, a white blood cell count of 7000/ll, C-reactive protein of 0.01 mg/dl, an angiotensin-converting enzyme level of 12.6 IU/l (normal 8.3–21.4 IU/l), a lysozyme level of 4.9 lg/ml (normal 5.0–10.2 lg/ml), Treponema pallidum hemagglutination assay and fluorescent treponemal antibody negativity. Antinuclear antibody was positive but rheumatoid factor and other specific autoantibodies including anti-aquaporin 4 antibody, anti-DNA antibody, and anti-neutrophil cytoplasmic antibodies were negative. Tests for bacterial, viral, fungal and tuberculous infections were all negative. Other blood studies, including renal and liver function tests, thyroid function tests, soluble interleukin-2 receptor, and adenosine deaminase, were all within the normal ranges. Chest X-ray findings were normal. Magnetic resonance imaging (MRI) of the orbit revealed high intensity at the swollen right optic nerve sheath in coronal short tau inversion recovery sequence (STIR) (Fig. 1a). The soft tissues around the right optic nerve sheath and right posterior sclera also showed high intensity in coronal and axial STIR images (Fig. 1a, b). The right optic nerve sheath and right posterior sclera were slightly enhanced in axial gadolinium-DTPA T1-weighted images using a fat-suppression technique (Fig. 1c). Systemic examination with whole-body computed tomography and 67gallium-scan
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Neurol Sci
Fig. 1 Magnetic resonance images of orbit. Short tau inversion recovery sequence of coronal (a) and axial (b) images showed highsignal intensity area at the right optic nerve sheath (arrows) and surrounding soft tissues (arrowheads). Axial gadolinium-DTPA T1-
weighted imaging using a fat-suppression technique (c) showed slightly enhanced right optic nerve sheath (arrow) and right posterior sclera (arrowhead)
scintigraphy showed no extracranial lesions. The patient was given intravenous methylprednisolone (1 g/day for 3 days) followed by oral prednisone (30 mg/day). Bilateral eye redness, ocular pain, and optic disc edema were relieved and her erythrocyte sedimentation rate was reduced to 16 mm in the first hour, though the patient’s visual acuity did not improve. After an additional course of intravenous methylprednisolone, her eye redness and pain completely disappeared. The high-signal intensity area in MRI (STIR images) and the enhancement in gadolinium-DTPA T1weighted images were reduced, though her right visual acuity remained severely impaired. The patient initially presented with bilateral episcleritis, but persistent inflammation extended to the right posterior sclera and right optic nerve sheath. Systemic immunosuppressive therapy was delayed by as long as 5 months after disease onset. Systemic immunosuppressive therapy usually has dramatic effects in patients with optic perineuritis [4], and even delayed treatment with high-dose steroids may improve visual acuity in some cases [3]. However, although the inflammation disappeared after systemic administration of high-dose corticosteroids, the patient’s visual acuity failed to improve. To the best of our knowledge, this report provides the first description of a case of episcleritis followed by optic perineuritis with severe visual loss. Very few patients have been found to progress from episcleritis to scleritis, or vice versa [1]. However, the
medical history and MRI findings in the present case suggest that persistent inflammation of the episclera may develop into posterior scleritis and orbital inflammation of the surrounding soft tissue, ultimately resulting in severe vision loss. Systemic immunosuppressive therapy should be commenced promptly to prevent permanent visual loss when optic involvement is seen in a patient with episcleritis.
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Conflict of interest We have no financial support or relationships that may pose conflict of interest.
References 1. Okhravi N, Odufuwa B, McCluskey P, Lightman S (2005) Scleritis. Surv Ophthalmol 50:351–363 2. Kennerdell JS, Dresner SC (1984) The nonspecific orbital inflammatory syndromes. Surv Ophthalmol 29:93–103 3. Tatsugawa M, Noma H, Mimura T, Funatsu H (2010) High-dose steroid therapy for idiopathic optic perineuritis: a case series. J Med Case Rep 4:404 4. Purvin V, Kawasaki A, Jacobson DM (2001) Optic perineuritis: clinical and radiographic features. Arch Ophthalmol 119:1299–1306 5. Ohtsuka K, Hashimoto M, Miura M, Nakamura Y (1997) Posterior scleritis with optic perineuritis and internal ophthalmoplegia. Br J Ophthalmol 81:514
LETTER TO THE EDITOR
Bilateral episcleritis followed by right optic perineuritis with severe visual loss: a case report Haruo Nishijima1 • Chieko Suzuki1 • Masahiko Tomiyama1
Received: 4 June 2015 / Accepted: 24 June 2015 Ó Springer-Verlag Italia 2015
Dear Editor, Persistent red eye may result in severe visual impairment. Scleritis is one possible cause of red eye that is considered to pose a significant threat to vision [1]. In contrast, episcleritis is confined to the superficial episcleral tissue and is thought to represent a mild, non-vision-threatening form of ocular inflammation, usually idiopathic in nature, that does not generally involve other ocular structures [1]. Idiopathic optic perineuritis has been reported as a type of orbital inflammatory pseudotumor [2, 3] with slow onset and often poor recovery in the event of delayed treatment [4]. Comorbid sclerotic inflammation and optic perineuritis have rarely been reported [5]. Here we describe a patient presenting with severe visual loss caused by bilateral episcleritis followed by right optic perineuritis. An 85-year-old woman was referred to our hospital with right visual loss. She had a past history of acute left visual loss and recovery with oral steroid therapy in her 50s, of which the details were unknown. Prior to referral, she had developed upper respiratory tract inflammation and visited her ophthalmologist 2 weeks later complaining of bilateral red eyes without ocular pain. At this time, her visual acuity was 20/40 with correction in each eye. She was diagnosed with episcleritis and treated with eye lotion containing corticosteroids. However, her eyes remained red 3 months later, and she noticed mild right ocular pain and decreased visual acuity in her right eye. She was finally referred to our
& Haruo Nishijima [email protected] 1
Department of Neurology, Aomori Prefectural Central Hospital, 2-1-1 Higashi-tsukurimichi, Aomori, Aomori 030-8553, Japan
department 2 months after the onset of visual impairment. On admission, her right visual acuity with correction was reduced to 20/200 and papilledema was detected in her right eye. No abnormal ocular movements were observed and her visual fields were normal. Fundus fluorescein angiography showed no apparent abnormalities. No other neurological deficit which might suggest multiple sclerosis or neuromyelitis optica spectrum disorders was detected. Other physical examinations were normal and there was no lymphadenopathy. Blood tests showed an erythrocyte sedimentation rate of 23 mm in the first hour, a white blood cell count of 7000/ll, C-reactive protein of 0.01 mg/dl, an angiotensin-converting enzyme level of 12.6 IU/l (normal 8.3–21.4 IU/l), a lysozyme level of 4.9 lg/ml (normal 5.0–10.2 lg/ml), Treponema pallidum hemagglutination assay and fluorescent treponemal antibody negativity. Antinuclear antibody was positive but rheumatoid factor and other specific autoantibodies including anti-aquaporin 4 antibody, anti-DNA antibody, and anti-neutrophil cytoplasmic antibodies were negative. Tests for bacterial, viral, fungal and tuberculous infections were all negative. Other blood studies, including renal and liver function tests, thyroid function tests, soluble interleukin-2 receptor, and adenosine deaminase, were all within the normal ranges. Chest X-ray findings were normal. Magnetic resonance imaging (MRI) of the orbit revealed high intensity at the swollen right optic nerve sheath in coronal short tau inversion recovery sequence (STIR) (Fig. 1a). The soft tissues around the right optic nerve sheath and right posterior sclera also showed high intensity in coronal and axial STIR images (Fig. 1a, b). The right optic nerve sheath and right posterior sclera were slightly enhanced in axial gadolinium-DTPA T1-weighted images using a fat-suppression technique (Fig. 1c). Systemic examination with whole-body computed tomography and 67gallium-scan
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Neurol Sci
Fig. 1 Magnetic resonance images of orbit. Short tau inversion recovery sequence of coronal (a) and axial (b) images showed highsignal intensity area at the right optic nerve sheath (arrows) and surrounding soft tissues (arrowheads). Axial gadolinium-DTPA T1-
weighted imaging using a fat-suppression technique (c) showed slightly enhanced right optic nerve sheath (arrow) and right posterior sclera (arrowhead)
scintigraphy showed no extracranial lesions. The patient was given intravenous methylprednisolone (1 g/day for 3 days) followed by oral prednisone (30 mg/day). Bilateral eye redness, ocular pain, and optic disc edema were relieved and her erythrocyte sedimentation rate was reduced to 16 mm in the first hour, though the patient’s visual acuity did not improve. After an additional course of intravenous methylprednisolone, her eye redness and pain completely disappeared. The high-signal intensity area in MRI (STIR images) and the enhancement in gadolinium-DTPA T1weighted images were reduced, though her right visual acuity remained severely impaired. The patient initially presented with bilateral episcleritis, but persistent inflammation extended to the right posterior sclera and right optic nerve sheath. Systemic immunosuppressive therapy was delayed by as long as 5 months after disease onset. Systemic immunosuppressive therapy usually has dramatic effects in patients with optic perineuritis [4], and even delayed treatment with high-dose steroids may improve visual acuity in some cases [3]. However, although the inflammation disappeared after systemic administration of high-dose corticosteroids, the patient’s visual acuity failed to improve. To the best of our knowledge, this report provides the first description of a case of episcleritis followed by optic perineuritis with severe visual loss. Very few patients have been found to progress from episcleritis to scleritis, or vice versa [1]. However, the
medical history and MRI findings in the present case suggest that persistent inflammation of the episclera may develop into posterior scleritis and orbital inflammation of the surrounding soft tissue, ultimately resulting in severe vision loss. Systemic immunosuppressive therapy should be commenced promptly to prevent permanent visual loss when optic involvement is seen in a patient with episcleritis.
123
Conflict of interest We have no financial support or relationships that may pose conflict of interest.
References 1. Okhravi N, Odufuwa B, McCluskey P, Lightman S (2005) Scleritis. Surv Ophthalmol 50:351–363 2. Kennerdell JS, Dresner SC (1984) The nonspecific orbital inflammatory syndromes. Surv Ophthalmol 29:93–103 3. Tatsugawa M, Noma H, Mimura T, Funatsu H (2010) High-dose steroid therapy for idiopathic optic perineuritis: a case series. J Med Case Rep 4:404 4. Purvin V, Kawasaki A, Jacobson DM (2001) Optic perineuritis: clinical and radiographic features. Arch Ophthalmol 119:1299–1306 5. Ohtsuka K, Hashimoto M, Miura M, Nakamura Y (1997) Posterior scleritis with optic perineuritis and internal ophthalmoplegia. Br J Ophthalmol 81:514
