Clinical Neurology and Neurosurgery 132 (2015) 12–15
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case Report
Idiopathic optic perineuritis: Disguised as recurrent optic neuritis Syed Baqer Sadiq a,b,∗ , James J. Corbett b,1 , Abuhuzeifa Abubakr b,1 a b
Department of Anesthesiology, 2500 North State Street, Jackson MS, USA Department of Neurology, 2500 North State Street, Jackson 39216-4505, USA
a r t i c l e
i n f o
Article history: Received 20 June 2014 Received in revised form 14 January 2015 Accepted 28 January 2015 Available online 7 February 2015 Keywords: Vision loss Optic neuritis Perineuritis
1. Initial presentation A 53-year-old woman with history of mitral valve prolapse and gastroesophageal reflux, presented to the Emergency Department with right eye pain and blurry vision for 2 days. She had acute onset of retro-orbital pain and headache, eye pain with eye movement but no nausea or vomiting. She also reported seeing gray spots and had ‘blurry vision’ in the right eye. She reported that the temporal visual field was entirely gray in the right eye. She had no prior visual symptoms and no family history of eye disease. She had no other neurological complaints. Her neurological examination showed extra ocular movements to be intact. She had a relative afferent pupillary defect in the right eye. On Humphrey 24-2 visual field, she had an enlarged blind spot and constriction peripherally. On ophthalmoscopic examination the patient had disk swelling in the right eye (Fig. 1). There were no hemorrhages or exudates. There was pain on eye movement. The remainder of her neurological and general physical examination was normal. Patient was admitted for evaluation of what was thought to be optic neuritis. MRI brain with and without contrast did not reveal any lesions suggestive of multiple sclerosis or other demyelinating disease. Lumbar puncture was normal. She was started on high dose
∗ Corresponding author. Tel.: +1 601 984 5501. E-mail addresses: [email protected] (S.B. Sadiq), [email protected] (J.J. Corbett), [email protected] (A. Abubakr). 1 Tel.: +1 601 984 5501; fax: +1 601 984 5503. http://dx.doi.org/10.1016/j.clineuro.2015.01.027 0303-8467/© 2015 Elsevier B.V. All rights reserved.
intravenous corticosteroids with rapid resolution of the pain and her vision improved remarkably. The right temporal visual field defect improved. 2. Relapse Four months after her initial attack of suspected optic neuritis she had an analogous episode of visual loss in the left eye and a repeat evaluation looking for an etiology. MRI of the brain and spine did not reveal any lesions suggestive of multiple sclerosis (MS). CSF and serum studies including neuromyelitis optica (NMO) antibody, were all negative. MRI of orbits revealed hyperintensity on FLAIR of the meninges surrounding the affected optic nerve which responded to high dose corticosteroids. She later on had two subsequent relapses affecting the left eye at 4 and 8 month’s interval following the first initial episode which affected the right eye. With each attack she had severe eye pain, worse with eye movement and blurring of vision, but no other neurologic symptoms. She had no diplopia, injection of sclera or conjunctiva during any attack. She became asymptomatic after treatment of each flare up with intravenous corticosteroids and the exam after her last exacerbation revealed visual acuity of 20/20 in both eyes, and no residual relative afferent pupillary defect (Fig. 2). MR imaging of the orbits revealed slight enhancement of the left intraorbital optic nerve sheath which led to the diagnosis of perioptic neuritis with the inflammation surrounding the optic nerve rather than causing the inflammation of the nerve itself [Image 1].
S.B. Sadiq et al. / Clinical Neurology and Neurosurgery 132 (2015) 12–15
Fig. 1. The right eye has a .3 cup to disk ratio and shows a pink neuroretinal rim. The left eye has .25 cup to disk ratio with nerve fiber layer drop out from the 1 o’ clock to 5 o’ clock position. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
13
Fig. 2. The right eye has a cup to disk ratio of .5 with neuroretinal rim pallor. The left eye has .25 cup to disk ratio with pink neuro retinal rim. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
3. Discussion Optic perineuritis (OPN) also known as perioptic neuritis, is a rare orbital inflammatory disorder of the optic nerve dural sheath and is distinct from demyelinating optic neuritis. It has been mostly described as an isolated condition of unknown cause; however, it has also been reported to have occurred with other infectious and inflammatory conditions. OPN usually does not have a recurrence. In the past two decades, reports with MRI have begun to clearly demarcate this disease from optic neuritis (ON). This differentiation stems primarily from the differences in the clinical course, the MRI appearance and the response to treatment of these two diseases. OPN, more often than not, presents as an isolated idiopathic disorder. Cases have been reported where it has presented as a manifestation of an underlying pathology such as Wegener’s granulomatosis, giant cell arteritis [1], Crohn’s disease [2], during the acute phase of (secondary) syphilis [3] as well as a manifestation of neurosyphilis [4], and with pre-B-cell lymphocytic leukemia [5]. ON, meanwhile, is associated with multiple sclerosis (MS) and in most cases is a predictor of MS in at least 50% of the cases. It can occur secondary to autoimmune disorders such as systemic lupus erythematosus (SLE), sarcoidosis, or, due to infections such as syphilis, Lyme disease or tuberculosis.
Image 1. MRI of the orbits, T2 axial image of the left eye in the posterior orbit preoptic canal sheath shows a tram track sign consistent with diagnosis of optic perineuritis.
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S.B. Sadiq et al. / Clinical Neurology and Neurosurgery 132 (2015) 12–15
OPN has been known to present in two forms: an exudative form with a focal suppurative pachymeningitis and as an exudative form with leptomeningitis and involvement of the subarachnoid space around the optic nerve. The initiating pathologic processes ranges from lymphocytic infiltrate, to foci of degenerating necrobiotic collagen, to granulomatous inflammation of the sheath. This has led to the belief that OPN is not a single pathology, but rather represents a response to a spectrum of disorders. Regardless of the initial inflammatory mechanism, the common reaction is the marked thickening of the optic nerve sheath involving fibrosis. This leads to secondary demyelination and/or importantly ischemic infarction of the optic nerve due to compression of blood vessels by the thickened optic nerve sheath. Clinically, OPN is difficult to differentiate from ON. Both conditions present with an acute monocular visual loss, pain with eye movement and, either a normal or swollen optic disk. Indeed, it is now thought that many of the previously labeled “corticosteroid responsive optic neuritis” cases were actually cases of OPN. Thus, radiographic findings become important in such a scenario as they dictate the further course of treatment. Whether or not steroids are administered to patients with ON 95% improve to VA of 20/40 or better. It should be noted however, that the visual loss in ON is usually central and/or altitudinal and progresses over days, while in OPN the visual loss tends to be peripheral or arcuate and may progress over weeks. The visual natural history and treatment of these two conditions, however is different. OPN typically does not naturally resolve. There may be a mild motility disturbance (due to extra-ocular muscle inflammation). Other manifestations may also be seen but not limited to diplopia, ptosis and conjunctival chemosis [6]. It is, therefore, important to identify any clinical and radiographic features that might help to distinguish ON from OPN. OPN occurs in a broad age distribution with the majority of patients being over 50 years of age. ON tends to affect young adults more often, with an increased occurrence amongst women. The age distribution here tends to be 15–49 years, with 15% of the patients over the age of 50 years. Both disorders present with similar symptoms of acute to sub-acute monocular visual loss, pain with eye movement and, a swollen or more often a normal-appearing optic disk. However, there are subtle differences that may help. Color vision defects occur in both. Other symptoms, indicating a more generalized underlying pathology, may be present, such as progressive weakness of the lower limbs or ataxia (indicating MS). Visual loss, in ON occurs over several days; this may show up as a central and/or altitudinal or arcuate defect in the visual field. Most often, the visual loss in a patient with ON may spontaneously resolve in a few weeks. Pathologically, OPN occurs as a result of varied processes such as lymphocytic infiltration, granulomatous inflammation of the sheath [7] or, necrobiotic degeneration of collagen in the dura. Regardless of the initiating mechanism, fibrotic thickening of the dural nerve sheath ensues. This may lead to secondary demyelination and/or infarction of the optic nerve as a result of compression by the thickened nerve sheath. ON, on the other hand, occurs due to a type IV hypersensitivity reaction. Cytokines released by an inflammatory process cross the blood brain barrier, and cause myelin degeneration and axonal death. The differentiation of OPN from ON, is of primary importance given the different prognosis for both the diseases. Along with the clinical picture, neuroimaging can help diagnose a suspected case of OPN. MRI findings are typical with a characteristic pattern of enhancement around the optic nerve, a donut on coronal cuts [Image 2] and is a “tram track” on FLAIR axial cuts along with streaky enhancement of the orbital fat [Image 1]. By contrast, ON tends to show enhancement of the optic nerve itself along with other features of demyelinating disease such as spinal lesions (in MS and NMO), white matter changes in the brain (as in MS) [6].
Image 2. MRI of the orbits, T1 coronal view shows thin donuts sign consistent with diagnosis of optic perineuritis.
Oral prednisone administration at 80 mg/day in patients with OPN can be both therapeutic, as well as diagnostic. In OPN, initiating treatment with prednisone provides for excellent recovery. Vision loss, however, may recur when treatment is discontinued. In ON, corticosteroid therapy has not been shown to influence the visual outcome and low dose 60–80 mg doses in patients with ON predispose the patient to recurrent ON. In summary, diagnosing OPN requires a nuanced approach. The clinical picture, where the patient is over 50 years old, with visual loss sparing the central visual field, progressing over weeks, and resulting in recurrent attacks after steroids are tapered is strongly suspicious of OPN. Neuro-imaging of OPN with MRI typically shows a characteristic enhancement pattern around the optic nerve (“tram-track” on axial and “doughnut” on coronal) and OPN may exhibit a “streaky” enhancement of the orbital fat. Any delay in initiating therapy with oral corticosteroids in OPN can result in visual loss. At the same time, ON has to be excluded as its presence is strongly indicative of an underlying demyelinating process like MS or NMO, which again require different forms of intervention and long term monitoring. 4. Conclusions Optic perineuritis (OPN) is difficult to differentiate from optic neuritis (ON). Both conditions present with an acute monocular visual loss, pain with eye movement and, a normal or swollen optic disk. In contrast to those younger patients with optic neuritis, patients with optic perineuritis are often older at onset and are more likely to show sparing of central vision. Magnetic resonance imaging scans demonstrate enhancement around, rather than within, the optic nerve. Response to corticosteroids is more dramatic in OPN than in patients with ON, and OPN patients are more likely to experience recurrence after stopping treatment with corticosteroids. In case of recurrence of visual loss, ON should be reconsidered. However, recent advances in MRI imaging have helped identify such a possibility as the radiographic findings of OPN are highly specific.
S.B. Sadiq et al. / Clinical Neurology and Neurosurgery 132 (2015) 12–15
References [1] Morotti A, Liberini P, Padovani A. Bilateral optic perineuritis as the presenting feature of giant cell arteritis. BMJ Case Rep 2013;2013. [2] McClelland C, Zaveri M, Walsh R, Fleisher J, Galetta S. Optic perineuritis as the presenting feature of Crohn disease. J Neuroophthalmol 2012;32: 345–7. [3] O’Connell K, Marnane M, McGuigan C. Bilateral ocular perineuritis as the presenting feature of acute syphilis infection. J Neurol 2012;259:191–2.
15
[4] Parker SE, Pula JH. Neurosyphilis presenting as asymptomatic optic perineuritis. Case Rep Ophthalmol Med 2012;2012:621872. [5] 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. [6] Purvin V, Kawasaki A, Jacobson DM. Optic perineuritis: clinical and radiographic features. Arch Ophthalmol 2001;119:1299–306. [7] Dutton JJ, Anderson RL. Idiopathic inflammatory perioptic neuritis simulating optic nerve sheath meningioma. Am J Ophthalmol 1985;100:424–30.
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case Report
Idiopathic optic perineuritis: Disguised as recurrent optic neuritis Syed Baqer Sadiq a,b,∗ , James J. Corbett b,1 , Abuhuzeifa Abubakr b,1 a b
Department of Anesthesiology, 2500 North State Street, Jackson MS, USA Department of Neurology, 2500 North State Street, Jackson 39216-4505, USA
a r t i c l e
i n f o
Article history: Received 20 June 2014 Received in revised form 14 January 2015 Accepted 28 January 2015 Available online 7 February 2015 Keywords: Vision loss Optic neuritis Perineuritis
1. Initial presentation A 53-year-old woman with history of mitral valve prolapse and gastroesophageal reflux, presented to the Emergency Department with right eye pain and blurry vision for 2 days. She had acute onset of retro-orbital pain and headache, eye pain with eye movement but no nausea or vomiting. She also reported seeing gray spots and had ‘blurry vision’ in the right eye. She reported that the temporal visual field was entirely gray in the right eye. She had no prior visual symptoms and no family history of eye disease. She had no other neurological complaints. Her neurological examination showed extra ocular movements to be intact. She had a relative afferent pupillary defect in the right eye. On Humphrey 24-2 visual field, she had an enlarged blind spot and constriction peripherally. On ophthalmoscopic examination the patient had disk swelling in the right eye (Fig. 1). There were no hemorrhages or exudates. There was pain on eye movement. The remainder of her neurological and general physical examination was normal. Patient was admitted for evaluation of what was thought to be optic neuritis. MRI brain with and without contrast did not reveal any lesions suggestive of multiple sclerosis or other demyelinating disease. Lumbar puncture was normal. She was started on high dose
∗ Corresponding author. Tel.: +1 601 984 5501. E-mail addresses: [email protected] (S.B. Sadiq), [email protected] (J.J. Corbett), [email protected] (A. Abubakr). 1 Tel.: +1 601 984 5501; fax: +1 601 984 5503. http://dx.doi.org/10.1016/j.clineuro.2015.01.027 0303-8467/© 2015 Elsevier B.V. All rights reserved.
intravenous corticosteroids with rapid resolution of the pain and her vision improved remarkably. The right temporal visual field defect improved. 2. Relapse Four months after her initial attack of suspected optic neuritis she had an analogous episode of visual loss in the left eye and a repeat evaluation looking for an etiology. MRI of the brain and spine did not reveal any lesions suggestive of multiple sclerosis (MS). CSF and serum studies including neuromyelitis optica (NMO) antibody, were all negative. MRI of orbits revealed hyperintensity on FLAIR of the meninges surrounding the affected optic nerve which responded to high dose corticosteroids. She later on had two subsequent relapses affecting the left eye at 4 and 8 month’s interval following the first initial episode which affected the right eye. With each attack she had severe eye pain, worse with eye movement and blurring of vision, but no other neurologic symptoms. She had no diplopia, injection of sclera or conjunctiva during any attack. She became asymptomatic after treatment of each flare up with intravenous corticosteroids and the exam after her last exacerbation revealed visual acuity of 20/20 in both eyes, and no residual relative afferent pupillary defect (Fig. 2). MR imaging of the orbits revealed slight enhancement of the left intraorbital optic nerve sheath which led to the diagnosis of perioptic neuritis with the inflammation surrounding the optic nerve rather than causing the inflammation of the nerve itself [Image 1].
S.B. Sadiq et al. / Clinical Neurology and Neurosurgery 132 (2015) 12–15
Fig. 1. The right eye has a .3 cup to disk ratio and shows a pink neuroretinal rim. The left eye has .25 cup to disk ratio with nerve fiber layer drop out from the 1 o’ clock to 5 o’ clock position. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
13
Fig. 2. The right eye has a cup to disk ratio of .5 with neuroretinal rim pallor. The left eye has .25 cup to disk ratio with pink neuro retinal rim. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
3. Discussion Optic perineuritis (OPN) also known as perioptic neuritis, is a rare orbital inflammatory disorder of the optic nerve dural sheath and is distinct from demyelinating optic neuritis. It has been mostly described as an isolated condition of unknown cause; however, it has also been reported to have occurred with other infectious and inflammatory conditions. OPN usually does not have a recurrence. In the past two decades, reports with MRI have begun to clearly demarcate this disease from optic neuritis (ON). This differentiation stems primarily from the differences in the clinical course, the MRI appearance and the response to treatment of these two diseases. OPN, more often than not, presents as an isolated idiopathic disorder. Cases have been reported where it has presented as a manifestation of an underlying pathology such as Wegener’s granulomatosis, giant cell arteritis [1], Crohn’s disease [2], during the acute phase of (secondary) syphilis [3] as well as a manifestation of neurosyphilis [4], and with pre-B-cell lymphocytic leukemia [5]. ON, meanwhile, is associated with multiple sclerosis (MS) and in most cases is a predictor of MS in at least 50% of the cases. It can occur secondary to autoimmune disorders such as systemic lupus erythematosus (SLE), sarcoidosis, or, due to infections such as syphilis, Lyme disease or tuberculosis.
Image 1. MRI of the orbits, T2 axial image of the left eye in the posterior orbit preoptic canal sheath shows a tram track sign consistent with diagnosis of optic perineuritis.
14
S.B. Sadiq et al. / Clinical Neurology and Neurosurgery 132 (2015) 12–15
OPN has been known to present in two forms: an exudative form with a focal suppurative pachymeningitis and as an exudative form with leptomeningitis and involvement of the subarachnoid space around the optic nerve. The initiating pathologic processes ranges from lymphocytic infiltrate, to foci of degenerating necrobiotic collagen, to granulomatous inflammation of the sheath. This has led to the belief that OPN is not a single pathology, but rather represents a response to a spectrum of disorders. Regardless of the initial inflammatory mechanism, the common reaction is the marked thickening of the optic nerve sheath involving fibrosis. This leads to secondary demyelination and/or importantly ischemic infarction of the optic nerve due to compression of blood vessels by the thickened optic nerve sheath. Clinically, OPN is difficult to differentiate from ON. Both conditions present with an acute monocular visual loss, pain with eye movement and, either a normal or swollen optic disk. Indeed, it is now thought that many of the previously labeled “corticosteroid responsive optic neuritis” cases were actually cases of OPN. Thus, radiographic findings become important in such a scenario as they dictate the further course of treatment. Whether or not steroids are administered to patients with ON 95% improve to VA of 20/40 or better. It should be noted however, that the visual loss in ON is usually central and/or altitudinal and progresses over days, while in OPN the visual loss tends to be peripheral or arcuate and may progress over weeks. The visual natural history and treatment of these two conditions, however is different. OPN typically does not naturally resolve. There may be a mild motility disturbance (due to extra-ocular muscle inflammation). Other manifestations may also be seen but not limited to diplopia, ptosis and conjunctival chemosis [6]. It is, therefore, important to identify any clinical and radiographic features that might help to distinguish ON from OPN. OPN occurs in a broad age distribution with the majority of patients being over 50 years of age. ON tends to affect young adults more often, with an increased occurrence amongst women. The age distribution here tends to be 15–49 years, with 15% of the patients over the age of 50 years. Both disorders present with similar symptoms of acute to sub-acute monocular visual loss, pain with eye movement and, a swollen or more often a normal-appearing optic disk. However, there are subtle differences that may help. Color vision defects occur in both. Other symptoms, indicating a more generalized underlying pathology, may be present, such as progressive weakness of the lower limbs or ataxia (indicating MS). Visual loss, in ON occurs over several days; this may show up as a central and/or altitudinal or arcuate defect in the visual field. Most often, the visual loss in a patient with ON may spontaneously resolve in a few weeks. Pathologically, OPN occurs as a result of varied processes such as lymphocytic infiltration, granulomatous inflammation of the sheath [7] or, necrobiotic degeneration of collagen in the dura. Regardless of the initiating mechanism, fibrotic thickening of the dural nerve sheath ensues. This may lead to secondary demyelination and/or infarction of the optic nerve as a result of compression by the thickened nerve sheath. ON, on the other hand, occurs due to a type IV hypersensitivity reaction. Cytokines released by an inflammatory process cross the blood brain barrier, and cause myelin degeneration and axonal death. The differentiation of OPN from ON, is of primary importance given the different prognosis for both the diseases. Along with the clinical picture, neuroimaging can help diagnose a suspected case of OPN. MRI findings are typical with a characteristic pattern of enhancement around the optic nerve, a donut on coronal cuts [Image 2] and is a “tram track” on FLAIR axial cuts along with streaky enhancement of the orbital fat [Image 1]. By contrast, ON tends to show enhancement of the optic nerve itself along with other features of demyelinating disease such as spinal lesions (in MS and NMO), white matter changes in the brain (as in MS) [6].
Image 2. MRI of the orbits, T1 coronal view shows thin donuts sign consistent with diagnosis of optic perineuritis.
Oral prednisone administration at 80 mg/day in patients with OPN can be both therapeutic, as well as diagnostic. In OPN, initiating treatment with prednisone provides for excellent recovery. Vision loss, however, may recur when treatment is discontinued. In ON, corticosteroid therapy has not been shown to influence the visual outcome and low dose 60–80 mg doses in patients with ON predispose the patient to recurrent ON. In summary, diagnosing OPN requires a nuanced approach. The clinical picture, where the patient is over 50 years old, with visual loss sparing the central visual field, progressing over weeks, and resulting in recurrent attacks after steroids are tapered is strongly suspicious of OPN. Neuro-imaging of OPN with MRI typically shows a characteristic enhancement pattern around the optic nerve (“tram-track” on axial and “doughnut” on coronal) and OPN may exhibit a “streaky” enhancement of the orbital fat. Any delay in initiating therapy with oral corticosteroids in OPN can result in visual loss. At the same time, ON has to be excluded as its presence is strongly indicative of an underlying demyelinating process like MS or NMO, which again require different forms of intervention and long term monitoring. 4. Conclusions Optic perineuritis (OPN) is difficult to differentiate from optic neuritis (ON). Both conditions present with an acute monocular visual loss, pain with eye movement and, a normal or swollen optic disk. In contrast to those younger patients with optic neuritis, patients with optic perineuritis are often older at onset and are more likely to show sparing of central vision. Magnetic resonance imaging scans demonstrate enhancement around, rather than within, the optic nerve. Response to corticosteroids is more dramatic in OPN than in patients with ON, and OPN patients are more likely to experience recurrence after stopping treatment with corticosteroids. In case of recurrence of visual loss, ON should be reconsidered. However, recent advances in MRI imaging have helped identify such a possibility as the radiographic findings of OPN are highly specific.
S.B. Sadiq et al. / Clinical Neurology and Neurosurgery 132 (2015) 12–15
References [1] Morotti A, Liberini P, Padovani A. Bilateral optic perineuritis as the presenting feature of giant cell arteritis. BMJ Case Rep 2013;2013. [2] McClelland C, Zaveri M, Walsh R, Fleisher J, Galetta S. Optic perineuritis as the presenting feature of Crohn disease. J Neuroophthalmol 2012;32: 345–7. [3] O’Connell K, Marnane M, McGuigan C. Bilateral ocular perineuritis as the presenting feature of acute syphilis infection. J Neurol 2012;259:191–2.
15
[4] Parker SE, Pula JH. Neurosyphilis presenting as asymptomatic optic perineuritis. Case Rep Ophthalmol Med 2012;2012:621872. [5] 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. [6] Purvin V, Kawasaki A, Jacobson DM. Optic perineuritis: clinical and radiographic features. Arch Ophthalmol 2001;119:1299–306. [7] Dutton JJ, Anderson RL. Idiopathic inflammatory perioptic neuritis simulating optic nerve sheath meningioma. Am J Ophthalmol 1985;100:424–30.
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