Brain & Development 37 (2015) 635–637 www.elsevier.com/locate/braindev
Case Report
A unilateral optic perineuritis in a teenager – A case report Ahmad Ameilia a, Ismail Shatriah a,⇑, Wan Hazabbah Wan-Hitam a, Rohaizan Yunus b a
Department of Ophthalmology, School of Medical Sciences, Universiti Sains Malaysia, 16150 Kubang Kerian, Kelantan, Malaysia b International Medical University Clinical School, Jalan Rasah, 70300 Seremban, Negeri Sembilan, Malaysia Received 5 August 2014; received in revised form 29 September 2014; accepted 30 September 2014
Abstract Optic perineuritis is an uncommon inflammatory disorder that involves optic nerve sheath. Numerous case reports have been published on optic perineuritis in adults, the majority of whom had bilateral presentation. There are limited data on optic perineuritis occurring in pediatric patients. We report a teenager who presented with a unilateral sign that mimicked the presentation of optic neuritis. The orbit and brain magnetic resonance imaging confirmed features of unilateral optic perineuritis. She was treated with a high dose of corticosteroids for 2 weeks, and her final visual outcome was satisfactory. No signs of relapse were noted during follow-up visits. Ó 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
Keywords: Optic perineuritis; Pediatric patients
1. Introduction Optic perineuritis is an uncommon form of orbital inflammatory disease and typically affects the optic nerve sheath. The majority of cases are isolated and idiopathic [1,2]. Syphilis, Wegener’s granulomatosis, sarcoidosis, Crohn disease and leukemia have been reported as causing optic perineuritis in adult patients [3–8]. Based on a PubMed literature search, limited information is available regarding optic perineuritis in pediatric patients. Recently, Grosso et al. was the first to report a case of optic perineuritis in a pediatric patient [9]. We describe here another case of optic perineuritis in a teenager with unilateral presentation. The clinical signs were fairly similar to optic neuritis. However, the magnetic resonance (MR) images of orbit and brain were consistent with right optic perineuritis, and our ⇑ Corresponding author. Tel.: +60 97676354.
E-mail address: [email protected] (I. Shatriah).
patient showed dramatic response to a high dose of systemic corticosteroid. 2. Case report A 15-year-old girl presented with blurred vision over her right eye that had lasted 2 weeks. It was associated with mild discomfort on ocular movement. No eye pain, redness or double vision was noted. She had no history of a similar illness. She denied any recent history of upper respiratory infection, fever, joint pain or recent immunization. Her visual acuity was 1/60 and improved to 4/60 with a pinhole in the right eye and 6/6 in the left eye. A relative afferent pupillary defect was noted in the affected eye. Other anterior segment examination was within normal. Fundoscopy examination revealed a mild swollen optic disk, particularly at the superior and nasal edges. The macula was normal. Examination of the fellow eye revealed no observed abnormality. Humphrey visual field showed inferotemporal field
http://dx.doi.org/10.1016/j.braindev.2014.09.011 0387-7604/Ó 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
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A. Ameilia et al. / Brain & Development 37 (2015) 635–637
Fig. 1. Humphrey visual field shows inferotemporal defect of the right eye.
defect in the right eye (Fig. 1). Color vision was affected in the involved eye. Laboratory tests revealed normal results for blood count, erythrocyte sedimentation rate, venereal disease research laboratory test, antinuclear antibody, angiotensin converting enzyme, C-reactive protein, complement test, and renal and liver function tests. The chest radiography was normal. The MR images of orbit and brain showed a tram-track sign on axial and doughnut appearance on coronal views (Fig. 2), which are features in line with right optic perineuritis. Cerebral spinal fluid analysis was not performed due to the parents’ refusal. The patient was treated with a high dose of intravenous methylprednisolone for 3 days, followed by oral prednisolone 1.2 mg/kg/body weight/day for another 11 days. She showed a dramatic improvement after 10 days of treatment. Her symptoms improved; visual acuity of the affected eye improved to 6/7.5 and optic disk swelling resolved. The patient was monitored closely during follow-up visits. Her right visual acuity returned 6/6 with normal appearance of the optic disk, visual field and color vision test. She was last reviewed at one and a half years after the attack, and reported no symptom of recurrence. 3. Discussion Purvin et al. described features of optic perineuritis that were observed in 14 adult patients aged 24–60 years [10]. The features included occurrence in an older age group, progress over a few weeks, often having arcuate or paracentral visual field defect, showing prompt and dramatic response and relapsing after brief treatment [10]. Our patient and the child reported by Grosso et al. [9] showed few similar clinical characteristics. Both patients were females, displayed no identified possible
etiology, had gradual onset of the symptom and showed prompt improvement in visual acuity after the initiation of systemic corticosteroid treatment. Our patient had a unilateral presentation of optic perineuritis. The majority of published optic perineuritis had bilateral involvement [1–4,9]. Two cases of unilateral optic perineuritis were described previously in adults, and both of which were identified as occurring due to syphilis infection [5]. The etiology remained obscured in our patient despite exhaustive investigations. Our patient presented with clinical manifestations mimicking optic neuritis of the right eye. The diagnosis of optic perineuritis in our patient was confirmed using MR imaging. In optic perineuritis, MR imaging demonstrates sign of perineural enhancement instead of intraneural enhancement in demyelinating optic neuritis. The tram-track sign on axial view and doughnut appearance on coronal view are suggestive. Streakiness of orbital fat with or without extraocular muscle enhancement may also be observed [10]. However Grosso et al. [9] reported slightly different signs on MR imaging; their patient displayed evidence of T2-weighted hyperintensity without contrast enhancement around the optic nerve and mild flattening of posterior globes. In contrast to optic neuritis, no large clinical trial has been described on a standard treatment for patients with optic perineuritis. The various treatment regimens described for adult patients include oral prednisolone, indomethacin, azathioprine, intravenous methylprednisolone and radiation therapy [10]. The duration of treatment was also variable. Poor final visual outcome has been reported in adult patients, and was attributed to delayed treatment [10]. As mentioned earlier, there is very limited information regarding the treatment of pediatric optic perineuritis. Grosso et al. [9] treated their patient with
A. Ameilia et al. / Brain & Development 37 (2015) 635–637
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Fig. 2. MR images in axial T2 with fat suppression (a), T1 (b), T1 post gadolinium with fat suppression (d) and coronal T1 post gadolinium with fat suppression (c). The signal intensity changes of the right optic nerve on image a and b (arrow). Post gadolinium it shows doughnut sign on coronal (image c) (arrow) and tram-track sign on axial (image d) (arrow).
intravenous methylprednisolone for 5 days, followed by a slow tapering of oral prednisolone for 1 month. Our patient was treated with intravenous methylprednisolone and oral prednisolone for 2 weeks. The final visual outcome was good, and our patient had no signs of relapse over the subsequent 18 months of monitoring. In conclusion, pediatric optic perineuritis is uncommon and can be easily mistaken for optic neuritis. MR imaging is mandatory to confirm the diagnosis. It is important to differentiate between pediatric optic perineuritis and optic neuritis cases; the management and prognosis of these two entities are different. Little is known about the treatment regime, final outcome and risk of recurrence or relapse of this disease in the pediatric population. More case reports or large case series publications are essential to provide comprehensive knowledge to ophthalmologists when treating cases of pediatric optic perineuritis.
Acknowledgement This study was supported by a Research University Grant from Universiti Sains Malaysia (Grant No. 1001/PPSP/812104).
References [1] Margo CE, Levy MH, Beck RW. Bilateral idiopathic inflammation of the optic nerve sheaths. Light and electron microscopic findings. Ophthalmology 1989;96:200–6. [2] Hykin PG, Spalton DJ. Bilateral perineuritis of the optic nerves. J Neurol Neurosurg Psychiatry 1991;54:375–6. [3] Parker SE, Pula JH. Neurosyphilis presenting as asymptomatic optic perineuritis. Case Rep Ophthalmol Med 2012;2012:621872. [4] O’Connell K, Marnane M, McGuigan C. Bilateral ocular perineuritis as the presenting feature of acute syphilis infection. J Neurol 2012;259:191–2. [5] McBurney J, Rosenberg ML. Unilateral syphilitic optic perineuritis presenting as the big blind spot syndrome. J Clin Neuroophthalmol 1987;7:167–9. [6] Takazawa T, Ikeda K, Nagaoka T, Hirayama T, Yamamoto T, Yanagihashi M, et al. Wegener granulomatosis-associated optic perineuritis. Orbit 2014;33:13–6. [7] Purvin V, Kawasaki A. Optic perineuritis secondary to Wegener’s granulomatosis. Clin Exp Ophthalmol 2009;37:712–7. [8] Townsend JH, Dubovy SR, Pasol J, Lam BL. Transient optic perineuritis as the initial presentation of central nervous system involvement by pre-B cell lymphocytic leukemia. J Neuroophthalmol 2013;33:162–4. [9] Grosso S, Cornacchione S, Romano D, Bertrando S, Franceschini R, Balestri P. Optic perineuritis: a further cause of visual loss and disc edema in children. Brain Dev 2014;36:932–5. doi: . [Epub ahead of print]. [10] Purvin V, Kawasaki A, Jacobson DM. Optic perineuritis: clinical and radiographic features. Arch Ophthalmol 2001;119:1299–306.
Case Report
A unilateral optic perineuritis in a teenager – A case report Ahmad Ameilia a, Ismail Shatriah a,⇑, Wan Hazabbah Wan-Hitam a, Rohaizan Yunus b a
Department of Ophthalmology, School of Medical Sciences, Universiti Sains Malaysia, 16150 Kubang Kerian, Kelantan, Malaysia b International Medical University Clinical School, Jalan Rasah, 70300 Seremban, Negeri Sembilan, Malaysia Received 5 August 2014; received in revised form 29 September 2014; accepted 30 September 2014
Abstract Optic perineuritis is an uncommon inflammatory disorder that involves optic nerve sheath. Numerous case reports have been published on optic perineuritis in adults, the majority of whom had bilateral presentation. There are limited data on optic perineuritis occurring in pediatric patients. We report a teenager who presented with a unilateral sign that mimicked the presentation of optic neuritis. The orbit and brain magnetic resonance imaging confirmed features of unilateral optic perineuritis. She was treated with a high dose of corticosteroids for 2 weeks, and her final visual outcome was satisfactory. No signs of relapse were noted during follow-up visits. Ó 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
Keywords: Optic perineuritis; Pediatric patients
1. Introduction Optic perineuritis is an uncommon form of orbital inflammatory disease and typically affects the optic nerve sheath. The majority of cases are isolated and idiopathic [1,2]. Syphilis, Wegener’s granulomatosis, sarcoidosis, Crohn disease and leukemia have been reported as causing optic perineuritis in adult patients [3–8]. Based on a PubMed literature search, limited information is available regarding optic perineuritis in pediatric patients. Recently, Grosso et al. was the first to report a case of optic perineuritis in a pediatric patient [9]. We describe here another case of optic perineuritis in a teenager with unilateral presentation. The clinical signs were fairly similar to optic neuritis. However, the magnetic resonance (MR) images of orbit and brain were consistent with right optic perineuritis, and our ⇑ Corresponding author. Tel.: +60 97676354.
E-mail address: [email protected] (I. Shatriah).
patient showed dramatic response to a high dose of systemic corticosteroid. 2. Case report A 15-year-old girl presented with blurred vision over her right eye that had lasted 2 weeks. It was associated with mild discomfort on ocular movement. No eye pain, redness or double vision was noted. She had no history of a similar illness. She denied any recent history of upper respiratory infection, fever, joint pain or recent immunization. Her visual acuity was 1/60 and improved to 4/60 with a pinhole in the right eye and 6/6 in the left eye. A relative afferent pupillary defect was noted in the affected eye. Other anterior segment examination was within normal. Fundoscopy examination revealed a mild swollen optic disk, particularly at the superior and nasal edges. The macula was normal. Examination of the fellow eye revealed no observed abnormality. Humphrey visual field showed inferotemporal field
http://dx.doi.org/10.1016/j.braindev.2014.09.011 0387-7604/Ó 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
636
A. Ameilia et al. / Brain & Development 37 (2015) 635–637
Fig. 1. Humphrey visual field shows inferotemporal defect of the right eye.
defect in the right eye (Fig. 1). Color vision was affected in the involved eye. Laboratory tests revealed normal results for blood count, erythrocyte sedimentation rate, venereal disease research laboratory test, antinuclear antibody, angiotensin converting enzyme, C-reactive protein, complement test, and renal and liver function tests. The chest radiography was normal. The MR images of orbit and brain showed a tram-track sign on axial and doughnut appearance on coronal views (Fig. 2), which are features in line with right optic perineuritis. Cerebral spinal fluid analysis was not performed due to the parents’ refusal. The patient was treated with a high dose of intravenous methylprednisolone for 3 days, followed by oral prednisolone 1.2 mg/kg/body weight/day for another 11 days. She showed a dramatic improvement after 10 days of treatment. Her symptoms improved; visual acuity of the affected eye improved to 6/7.5 and optic disk swelling resolved. The patient was monitored closely during follow-up visits. Her right visual acuity returned 6/6 with normal appearance of the optic disk, visual field and color vision test. She was last reviewed at one and a half years after the attack, and reported no symptom of recurrence. 3. Discussion Purvin et al. described features of optic perineuritis that were observed in 14 adult patients aged 24–60 years [10]. The features included occurrence in an older age group, progress over a few weeks, often having arcuate or paracentral visual field defect, showing prompt and dramatic response and relapsing after brief treatment [10]. Our patient and the child reported by Grosso et al. [9] showed few similar clinical characteristics. Both patients were females, displayed no identified possible
etiology, had gradual onset of the symptom and showed prompt improvement in visual acuity after the initiation of systemic corticosteroid treatment. Our patient had a unilateral presentation of optic perineuritis. The majority of published optic perineuritis had bilateral involvement [1–4,9]. Two cases of unilateral optic perineuritis were described previously in adults, and both of which were identified as occurring due to syphilis infection [5]. The etiology remained obscured in our patient despite exhaustive investigations. Our patient presented with clinical manifestations mimicking optic neuritis of the right eye. The diagnosis of optic perineuritis in our patient was confirmed using MR imaging. In optic perineuritis, MR imaging demonstrates sign of perineural enhancement instead of intraneural enhancement in demyelinating optic neuritis. The tram-track sign on axial view and doughnut appearance on coronal view are suggestive. Streakiness of orbital fat with or without extraocular muscle enhancement may also be observed [10]. However Grosso et al. [9] reported slightly different signs on MR imaging; their patient displayed evidence of T2-weighted hyperintensity without contrast enhancement around the optic nerve and mild flattening of posterior globes. In contrast to optic neuritis, no large clinical trial has been described on a standard treatment for patients with optic perineuritis. The various treatment regimens described for adult patients include oral prednisolone, indomethacin, azathioprine, intravenous methylprednisolone and radiation therapy [10]. The duration of treatment was also variable. Poor final visual outcome has been reported in adult patients, and was attributed to delayed treatment [10]. As mentioned earlier, there is very limited information regarding the treatment of pediatric optic perineuritis. Grosso et al. [9] treated their patient with
A. Ameilia et al. / Brain & Development 37 (2015) 635–637
637
Fig. 2. MR images in axial T2 with fat suppression (a), T1 (b), T1 post gadolinium with fat suppression (d) and coronal T1 post gadolinium with fat suppression (c). The signal intensity changes of the right optic nerve on image a and b (arrow). Post gadolinium it shows doughnut sign on coronal (image c) (arrow) and tram-track sign on axial (image d) (arrow).
intravenous methylprednisolone for 5 days, followed by a slow tapering of oral prednisolone for 1 month. Our patient was treated with intravenous methylprednisolone and oral prednisolone for 2 weeks. The final visual outcome was good, and our patient had no signs of relapse over the subsequent 18 months of monitoring. In conclusion, pediatric optic perineuritis is uncommon and can be easily mistaken for optic neuritis. MR imaging is mandatory to confirm the diagnosis. It is important to differentiate between pediatric optic perineuritis and optic neuritis cases; the management and prognosis of these two entities are different. Little is known about the treatment regime, final outcome and risk of recurrence or relapse of this disease in the pediatric population. More case reports or large case series publications are essential to provide comprehensive knowledge to ophthalmologists when treating cases of pediatric optic perineuritis.
Acknowledgement This study was supported by a Research University Grant from Universiti Sains Malaysia (Grant No. 1001/PPSP/812104).
References [1] Margo CE, Levy MH, Beck RW. Bilateral idiopathic inflammation of the optic nerve sheaths. Light and electron microscopic findings. Ophthalmology 1989;96:200–6. [2] Hykin PG, Spalton DJ. Bilateral perineuritis of the optic nerves. J Neurol Neurosurg Psychiatry 1991;54:375–6. [3] Parker SE, Pula JH. Neurosyphilis presenting as asymptomatic optic perineuritis. Case Rep Ophthalmol Med 2012;2012:621872. [4] O’Connell K, Marnane M, McGuigan C. Bilateral ocular perineuritis as the presenting feature of acute syphilis infection. J Neurol 2012;259:191–2. [5] McBurney J, Rosenberg ML. Unilateral syphilitic optic perineuritis presenting as the big blind spot syndrome. J Clin Neuroophthalmol 1987;7:167–9. [6] Takazawa T, Ikeda K, Nagaoka T, Hirayama T, Yamamoto T, Yanagihashi M, et al. Wegener granulomatosis-associated optic perineuritis. Orbit 2014;33:13–6. [7] Purvin V, Kawasaki A. Optic perineuritis secondary to Wegener’s granulomatosis. Clin Exp Ophthalmol 2009;37:712–7. [8] Townsend JH, Dubovy SR, Pasol J, Lam BL. Transient optic perineuritis as the initial presentation of central nervous system involvement by pre-B cell lymphocytic leukemia. J Neuroophthalmol 2013;33:162–4. [9] Grosso S, Cornacchione S, Romano D, Bertrando S, Franceschini R, Balestri P. Optic perineuritis: a further cause of visual loss and disc edema in children. Brain Dev 2014;36:932–5. doi: . [Epub ahead of print]. [10] Purvin V, Kawasaki A, Jacobson DM. Optic perineuritis: clinical and radiographic features. Arch Ophthalmol 2001;119:1299–306.
