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Recurrent, alternating orbital inflammation
Journal of the Neurological Sciences 357 (2015) 288–289
Contents lists available at ScienceDirect
Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns
Short communication
Recurrent, alternating orbital inflammation Akiyuki Hiraga a,⁎, Satoshi Kuwabara b a b
Department of Neurology, Chiba Rosai Hospital, Chiba, Japan Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan
a r t i c l e
i n f o
Article history: Received 12 April 2015 Received in revised form 29 July 2015 Accepted 30 July 2015 Available online 1 August 2015 Keywords: Idiopathic orbital inflammation Orbital myositis Computed tomography Magnetic resonance imaging Alternating Painful ophthalmoplegia
1. Background Idiopathic orbital inflammation (IOI) describes a group of benign non-infectious inflammatory disorders within the orbit. The subtype of IOI includes isolated dacryoadenitis, orbital myositis, orbital apex syndrome and others [1]. Typically, IOI presents with a unilateral acute clinical course and bilateral presentation is rare in adults [2]. In this report, we present the case of a woman with a history of over 50 years of relapsing–remitting and alternating attacks of orbital pain or painful ophthalmoplegia due to orbital inflammation, mainly myositis of extraorbital muscles.
2. Case presentation The patient had no relevant medical history until the age of 30 years, when she had attacks of recurrent unilateral orbital pain, occurring as episodes of active pain lasting for 4 weeks. Attacks occurred irregularly, on an average 2 to 3 times per year, with pain-free periods ranging from 1 month to 3 years. The pain was unilateral, restricted to the right or left orbit and was accompanied by ptosis on the same side on which the pain occurred. Prior to coming to our institution, she had multiple clinic
visits during the pain attacks; however, no definitive diagnosis was made. At the age of 73, the patient was referred to our outpatient clinic complaining of acute right orbital pain. On clinical examination, she had right ptosis; however, both eyes had a full motion range. Brain magnetic resonance imaging (MRI) revealed no cranial abnormalities. The symptoms resolved within 1 month without any treatment. Two months later, she presented with left orbital pain and left ptosis. On examination, apart from left ptosis, there were no other neurological deficits. Brain computed tomography (CT) at that time showed no intracranial abnormalities and symptoms gradually resolved. One year later, she presented with left orbital pain and diplopia. On examination, she had mild limitation in abduction of the left eye movement and left ptosis. Brain CT showed no intracranial abnormalities. Although a diagnosis had not been established, treatment with oral prednisolone (20 mg/day) was initiated and symptoms dramatically improved within one day. At the age of 77 and at the age of 79, she relapsed presenting with right orbital pain and right ptosis, and corticosteroid therapy again led to resolution of symptoms. At the age of 80, she presented with right orbital pain. She had right ptosis with severe limitation in abduction of the right eye movement. Orbital CT revealed thickening of the right medial rectus muscle with normal thickness of the other extraocular muscles (Fig. 1). MRI scans also revealed enlargement of the belly of the right medial rectus muscle. Reassessment of the cranial MRI and CT scans performed during previous attacks revealed slight thickening of the unilateral medial or lateral rectus muscle, which had not been noticed earlier (Fig. 1). Therefore, a diagnosis of recurrent orbital inflammation was established. Serum levels of free triiodothyronine, free thyroxine and thyroid stimulating hormone (TSH) were 2.94 pg/mL (2.3–4.0), 0.95 ng/dL (1.00–1.80) and 5.18 μIU/mL (0.50–5.00), respectively. TSH receptor antibody and TSH receptor stimulating antibody were negative; however, values of anti-thyroid peroxidase antibodies of 227 IU/mL (0–16) and anti-thyroglobulin antibodies of 371 U/L (0–28) were elevated. There was marked clinical improvement after starting prednisolone treatment. At the age of 81, she presented with right orbital pain and ptosis but there was no ophthalmoplegia. Orbital CT showed no enlargement of the extraocular muscles and corticosteroids again resolved her symptoms. 3. Discussion
⁎ Corresponding author at: Department of Neurology, Chiba Rosai Hospital, 2-16 Tatsumidai-Higashi, Ichihara-shi, Chiba 290-0003, Japan. E-mail address: [email protected] (A. Hiraga).
http://dx.doi.org/10.1016/j.jns.2015.07.049 0022-510X/© 2015 Elsevier B.V. All rights reserved.
Here, we report a case of relapsing–remitting orbital inflammation affecting various extraocular muscles with changing laterality and a
A. Hiraga, S. Kuwabara / Journal of the Neurological Sciences 357 (2015) 288–289
289
Fig. 1. (A) Coronal computed tomography (CT) scan of the orbits showed gross thickening of the right medial rectus muscle (arrow) with normal thickness of the other extraocular muscles. (B) Axial and (C) coronal post-enhanced magnetic resonance imaging scans showed an enlargement of the belly of the right medial rectus muscle. There was no tendon involvement. (D–I) Serial findings of extraocular muscles. The side of the pain attack is indicated by arrowheads. (D) At the age of 73, the first examination of the patient, who presented with right orbital pain and ptosis, was conducted at our institution. T2-weighted imaging scans indicated an enlarged belly of the right medial rectus muscle (arrow). (E) At the age of 73, two months after the first visit, the patient presented with left orbital pain. Computed tomography showed normal extraocular muscles. (F) At the age of 75, the patient had left orbital pain with limitation in abduction of the left eye. CT showed an enlarged belly of the left lateral rectus muscle (arrow). (G) At the age of 77, the patient presented with right orbital pain. CT showed an enlarged belly of the right lateral rectus muscle (arrow). (H) At the age of 80, the patient had right orbital pain with limitation in abduction of the right eye. CT showed an isolated enlarged belly of the right inferior rectus muscle (arrow). (I) At the age of 81, the patient had right orbital pain. CT showed normal extraocular muscles.
long clinical course of over 50 years. By definition, IOI excludes lesions with identifiable local or systemic aetiology [1]. The differential diagnosis of orbital inflammation includes systemic disease, such as vasculitis, thyroid orbitopathy and neoplasm. Our case showed no systemic inflammatory disease, and the long time-course did not suggest infection or malignancy. Thyroid orbitopathy was a potential diagnosis in this case because thyroid peroxidase autoantibodies were elevated. Thyroid orbitopathy has characteristic manifestations such as eyelid retraction, ptosis and extraocular myopathy, and pain is not a prominent feature. In addition, radiological findings include enlargement of extraocular muscles and an increase in orbital fat volume, typically bilateral [1]. The clinical features including acute-onset unilateral pain with ptosis and marked improvement by corticosteroid therapy indicate IOI; however, thyroid orbitopathy cannot be completely ruled out. Although imaging of the extraocular muscles by brain CT (which did not focus on the orbit) was difficult, reassessment of previous serial MRI and CT scans revealed alternating orbital muscle enlargement. Our case indicates that orbital inflammation can recur in different extraocular muscles and in the contralateral orbit after long periods of time. IOI is known to recur in the same eye and the rate of relapse has been reported as 0.18/person-year [2]. However, recurrence in the contralateral eye, namely alternating IOI, is very rare. There have been recent reports on six cases of alternating IOI. Four cases had two episodes of relapse and the other two cases had three episodes of relapse. All the relapses were at different sites and the time to recurrence ranged from 1 week to 26 months [3]. There are also older reports on alternating IOI [4–7]; however, there has been no report on relapsing–remitting and alternating IOI with such an extensive clinical course, as in our case. The reversible enlargement of the extraocular muscles over such long periods indicated a benign inflammatory pathogenesis in this case without sclerosis or fibrosis. Although the exact cause of IOI is unknown [1], alternating attacks
involving various extraocular muscles, with rapid and reversible response to corticosteroid therapy, as in our case, are suggestive of an immunemediated pathophysiology. IOI is one of the most important differential diagnoses of ‘painful ophthalmoplegia’, ranking with Tolosa–Hunt syndrome or vascular conditions such as an aneurysm; most of these conditions are unilateral. Our case emphasises the importance of considering orbital inflammation in the differential diagnosis of recurrent and alternating painful ophthalmoplegia. Although brain MRI or CT scans are mandatory in the diagnostic work-up of painful ophthalmoplegia, thickening of the extraocular muscles may be missed when the focus of imaging is centred on other structures. Disclosure The authors declare that they have no competing interests. References [1] S.J.A. Yuen SJ, P.A.D. Rubin, Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome, Arch. Ophthalmol. 121 (2003) 491–499. [2] B.N. Swamy, P. McCluskey, A. Nemet, et al., Idiopathic orbital inflammatory syndrome: clinical features and treatment outcomes, Br. J. Ophthalmol. 91 (2007) 1667–1670. [3] N. Avni-Zauberman, D. Tripathy, N. Rosen, G.J. Ben Simon, Relapsing migratory idiopathic orbital inflammation: six new cases and review of the literature, Br. J. Ophthalmol. 96 (2012) 276–280. [4] J.R. Keane, Alternating proptosis: a case report of acute orbital myositis defined by the computerized tomographic scan, Arch. Neurol. 34 (1977) 642–643. [5] I. Ludwig, R.L. Tomsak, Acute recurrent orbital myositis, J. Clin. Neuroophthalmol. 3 (1983) 41–47. [6] I. Mombaerts, L. Koornneef, Current status in the treatment of orbital myositis, Ophthalmology 104 (1997) 402–408. [7] I. Maurer, S. Zierz, Recurrent orbital myositis: report of a familial incidence, Arch. Neurol. 56 (1999) 1407–1409.
Contents lists available at ScienceDirect
Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns
Short communication
Recurrent, alternating orbital inflammation Akiyuki Hiraga a,⁎, Satoshi Kuwabara b a b
Department of Neurology, Chiba Rosai Hospital, Chiba, Japan Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan
a r t i c l e
i n f o
Article history: Received 12 April 2015 Received in revised form 29 July 2015 Accepted 30 July 2015 Available online 1 August 2015 Keywords: Idiopathic orbital inflammation Orbital myositis Computed tomography Magnetic resonance imaging Alternating Painful ophthalmoplegia
1. Background Idiopathic orbital inflammation (IOI) describes a group of benign non-infectious inflammatory disorders within the orbit. The subtype of IOI includes isolated dacryoadenitis, orbital myositis, orbital apex syndrome and others [1]. Typically, IOI presents with a unilateral acute clinical course and bilateral presentation is rare in adults [2]. In this report, we present the case of a woman with a history of over 50 years of relapsing–remitting and alternating attacks of orbital pain or painful ophthalmoplegia due to orbital inflammation, mainly myositis of extraorbital muscles.
2. Case presentation The patient had no relevant medical history until the age of 30 years, when she had attacks of recurrent unilateral orbital pain, occurring as episodes of active pain lasting for 4 weeks. Attacks occurred irregularly, on an average 2 to 3 times per year, with pain-free periods ranging from 1 month to 3 years. The pain was unilateral, restricted to the right or left orbit and was accompanied by ptosis on the same side on which the pain occurred. Prior to coming to our institution, she had multiple clinic
visits during the pain attacks; however, no definitive diagnosis was made. At the age of 73, the patient was referred to our outpatient clinic complaining of acute right orbital pain. On clinical examination, she had right ptosis; however, both eyes had a full motion range. Brain magnetic resonance imaging (MRI) revealed no cranial abnormalities. The symptoms resolved within 1 month without any treatment. Two months later, she presented with left orbital pain and left ptosis. On examination, apart from left ptosis, there were no other neurological deficits. Brain computed tomography (CT) at that time showed no intracranial abnormalities and symptoms gradually resolved. One year later, she presented with left orbital pain and diplopia. On examination, she had mild limitation in abduction of the left eye movement and left ptosis. Brain CT showed no intracranial abnormalities. Although a diagnosis had not been established, treatment with oral prednisolone (20 mg/day) was initiated and symptoms dramatically improved within one day. At the age of 77 and at the age of 79, she relapsed presenting with right orbital pain and right ptosis, and corticosteroid therapy again led to resolution of symptoms. At the age of 80, she presented with right orbital pain. She had right ptosis with severe limitation in abduction of the right eye movement. Orbital CT revealed thickening of the right medial rectus muscle with normal thickness of the other extraocular muscles (Fig. 1). MRI scans also revealed enlargement of the belly of the right medial rectus muscle. Reassessment of the cranial MRI and CT scans performed during previous attacks revealed slight thickening of the unilateral medial or lateral rectus muscle, which had not been noticed earlier (Fig. 1). Therefore, a diagnosis of recurrent orbital inflammation was established. Serum levels of free triiodothyronine, free thyroxine and thyroid stimulating hormone (TSH) were 2.94 pg/mL (2.3–4.0), 0.95 ng/dL (1.00–1.80) and 5.18 μIU/mL (0.50–5.00), respectively. TSH receptor antibody and TSH receptor stimulating antibody were negative; however, values of anti-thyroid peroxidase antibodies of 227 IU/mL (0–16) and anti-thyroglobulin antibodies of 371 U/L (0–28) were elevated. There was marked clinical improvement after starting prednisolone treatment. At the age of 81, she presented with right orbital pain and ptosis but there was no ophthalmoplegia. Orbital CT showed no enlargement of the extraocular muscles and corticosteroids again resolved her symptoms. 3. Discussion
⁎ Corresponding author at: Department of Neurology, Chiba Rosai Hospital, 2-16 Tatsumidai-Higashi, Ichihara-shi, Chiba 290-0003, Japan. E-mail address: [email protected] (A. Hiraga).
http://dx.doi.org/10.1016/j.jns.2015.07.049 0022-510X/© 2015 Elsevier B.V. All rights reserved.
Here, we report a case of relapsing–remitting orbital inflammation affecting various extraocular muscles with changing laterality and a
A. Hiraga, S. Kuwabara / Journal of the Neurological Sciences 357 (2015) 288–289
289
Fig. 1. (A) Coronal computed tomography (CT) scan of the orbits showed gross thickening of the right medial rectus muscle (arrow) with normal thickness of the other extraocular muscles. (B) Axial and (C) coronal post-enhanced magnetic resonance imaging scans showed an enlargement of the belly of the right medial rectus muscle. There was no tendon involvement. (D–I) Serial findings of extraocular muscles. The side of the pain attack is indicated by arrowheads. (D) At the age of 73, the first examination of the patient, who presented with right orbital pain and ptosis, was conducted at our institution. T2-weighted imaging scans indicated an enlarged belly of the right medial rectus muscle (arrow). (E) At the age of 73, two months after the first visit, the patient presented with left orbital pain. Computed tomography showed normal extraocular muscles. (F) At the age of 75, the patient had left orbital pain with limitation in abduction of the left eye. CT showed an enlarged belly of the left lateral rectus muscle (arrow). (G) At the age of 77, the patient presented with right orbital pain. CT showed an enlarged belly of the right lateral rectus muscle (arrow). (H) At the age of 80, the patient had right orbital pain with limitation in abduction of the right eye. CT showed an isolated enlarged belly of the right inferior rectus muscle (arrow). (I) At the age of 81, the patient had right orbital pain. CT showed normal extraocular muscles.
long clinical course of over 50 years. By definition, IOI excludes lesions with identifiable local or systemic aetiology [1]. The differential diagnosis of orbital inflammation includes systemic disease, such as vasculitis, thyroid orbitopathy and neoplasm. Our case showed no systemic inflammatory disease, and the long time-course did not suggest infection or malignancy. Thyroid orbitopathy was a potential diagnosis in this case because thyroid peroxidase autoantibodies were elevated. Thyroid orbitopathy has characteristic manifestations such as eyelid retraction, ptosis and extraocular myopathy, and pain is not a prominent feature. In addition, radiological findings include enlargement of extraocular muscles and an increase in orbital fat volume, typically bilateral [1]. The clinical features including acute-onset unilateral pain with ptosis and marked improvement by corticosteroid therapy indicate IOI; however, thyroid orbitopathy cannot be completely ruled out. Although imaging of the extraocular muscles by brain CT (which did not focus on the orbit) was difficult, reassessment of previous serial MRI and CT scans revealed alternating orbital muscle enlargement. Our case indicates that orbital inflammation can recur in different extraocular muscles and in the contralateral orbit after long periods of time. IOI is known to recur in the same eye and the rate of relapse has been reported as 0.18/person-year [2]. However, recurrence in the contralateral eye, namely alternating IOI, is very rare. There have been recent reports on six cases of alternating IOI. Four cases had two episodes of relapse and the other two cases had three episodes of relapse. All the relapses were at different sites and the time to recurrence ranged from 1 week to 26 months [3]. There are also older reports on alternating IOI [4–7]; however, there has been no report on relapsing–remitting and alternating IOI with such an extensive clinical course, as in our case. The reversible enlargement of the extraocular muscles over such long periods indicated a benign inflammatory pathogenesis in this case without sclerosis or fibrosis. Although the exact cause of IOI is unknown [1], alternating attacks
involving various extraocular muscles, with rapid and reversible response to corticosteroid therapy, as in our case, are suggestive of an immunemediated pathophysiology. IOI is one of the most important differential diagnoses of ‘painful ophthalmoplegia’, ranking with Tolosa–Hunt syndrome or vascular conditions such as an aneurysm; most of these conditions are unilateral. Our case emphasises the importance of considering orbital inflammation in the differential diagnosis of recurrent and alternating painful ophthalmoplegia. Although brain MRI or CT scans are mandatory in the diagnostic work-up of painful ophthalmoplegia, thickening of the extraocular muscles may be missed when the focus of imaging is centred on other structures. Disclosure The authors declare that they have no competing interests. References [1] S.J.A. Yuen SJ, P.A.D. Rubin, Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome, Arch. Ophthalmol. 121 (2003) 491–499. [2] B.N. Swamy, P. McCluskey, A. Nemet, et al., Idiopathic orbital inflammatory syndrome: clinical features and treatment outcomes, Br. J. Ophthalmol. 91 (2007) 1667–1670. [3] N. Avni-Zauberman, D. Tripathy, N. Rosen, G.J. Ben Simon, Relapsing migratory idiopathic orbital inflammation: six new cases and review of the literature, Br. J. Ophthalmol. 96 (2012) 276–280. [4] J.R. Keane, Alternating proptosis: a case report of acute orbital myositis defined by the computerized tomographic scan, Arch. Neurol. 34 (1977) 642–643. [5] I. Ludwig, R.L. Tomsak, Acute recurrent orbital myositis, J. Clin. Neuroophthalmol. 3 (1983) 41–47. [6] I. Mombaerts, L. Koornneef, Current status in the treatment of orbital myositis, Ophthalmology 104 (1997) 402–408. [7] I. Maurer, S. Zierz, Recurrent orbital myositis: report of a familial incidence, Arch. Neurol. 56 (1999) 1407–1409.