Neurol Sci DOI 10.1007/s10072-015-2093-5
LETTER TO THE EDITOR
Eyelid ptosis enhanced after steroid pulse therapy in ocular myasthenia gravis: a case report Haruo Nishijima • Tatsuya Ueno • Chieko Suzuki Masayuki Baba • Masahiko Tomiyama
•
Received: 5 November 2014 / Accepted: 21 January 2015 Ó Springer-Verlag Italia 2015
Dear Editor, Eyelid ptosis is a frequent symptom in myasthenia gravis and severity of ptosis fluctuates along with the disease course [1]. Here, we describe a patient with ocular myasthenia gravis, presenting with ptosis in the contralateral side to primary affected side after edrophonium (Tensilon) test and steroid pulse therapy. A 39-year-old Japanese woman presented with a 3-month history of diplopia and right eyelid ptosis (Fig. 1a). Her symptoms had diurnal variation, worsened in the evening, and improved after rest. There were no symptoms or signs of weakness of facial, neck, bulbar, limb, or respiratory muscles. The edrophonium test disclosed improvement of the right ptosis and induced minor left ptosis (Fig. 1b). Repetitive nerve stimulation of the deltoid muscles and abductor digiti minimi muscles showed no significant amplitude decrements. Serum acetylcholine receptor antibodies and muscle-specific tyrosine kinase antibodies were both negative. Chest computed tomography was negative for thymoma. Magnetic resonance imaging of the orbits revealed no abnormalities. Ambenonium chloride (20 mg/day) resulted in improvement of ptosis and diplopia. We diagnosed her illness as double-seronegative ocular myasthenia gravis. Her H. Nishijima (&) T. Ueno C. Suzuki M. Baba M. Tomiyama Department of Neurology, Aomori Prefectural Central Hospital, 2-1-1 Higashi-tsukurimichi, Aomori City, Aomori 030-8553, Japan e-mail: [email protected] H. Nishijima T. Ueno M. Tomiyama Department of Neurophysiology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Hirosaki City, Aomori, Japan
symptoms gradually worsened in the following 2 months. Oral prednisolone therapy (5 mg/day) was started and her symptoms slightly improved. She still had a difficulty in her daily activities, but refused to continue oral prednisolone therapy, because concerned about adverse effect due to long-term treatment with steroid. Then, she was admitted to our hospital, stopped to taking oral steroid, and intravenous methylprednisolone therapy (1 g/day for 3 days) was commenced. After three courses of intravenous methylprednisolone therapy with 4-day intervals between the courses, right eyelid ptosis satisfactorily improved; however, left eyelid ptosis appeared during the second course of the therapy (Fig. 1c). Left eyelid ptosis did not have diurnal variation. The edrophonium test was negative and repetitive nerve stimulation of the left orbicularis oculi showed no significant amplitude decrements. After 1-month observation without additional immunosuppressive treatment, left ptosis completely disappeared (Fig. 1d). Diplopia completely improved after the first course of the therapy and did not recur in this observation period. We consider that our patient’s left ptosis might have been induced by rapid improvement of right eyelid ptosis with steroid pulse therapy. Hering’s law of equal innervations as applied to the elevator palpebrae superioris could be a likely explanation of this phenomenon, as in the case with surgical treatment-induced contralateral ptosis [2, 3]. Central nervous system compensates for peripheral weakness of the extraocular muscles in a patient with ocular myasthenia gravis. To keep the dominant right eyelid in the primary position, an increased innervation to the right elevator muscle is needed. The same increase of innervation is provided to the non-paretic contralateral synergist of the left eyelid. Rapid improvement of right ptosis with steroid pulse therapy may unmask this central adaptation,
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Neurol Sci
that is, induce decrement of these innervations, which results in relatively hypo-innervation to left eyelid elevator [4, 5]. Enhanced ptosis contralateral to the primary affected side after steroid therapy in a patient with myasthenia gravis was rarely reported. Steroid pulse therapy is not common in treatment of myasthenia gravis, considering well-known frequent complication, initial exacerbation. However, it has been reported that intravenous methylprednisolone therapy for ocular myasthenia gravis induced more rapid induction of disease remission than oral steroid therapy [5]. Central adaptation may be lost after gradual recovery, which could explain the absence of a similar phenomenon in a treatment with oral steroid therapy. Conflict of interest We have no financial support or relationships that may pose conflict of interest.
References 1. Jayam Trouth A, Dabi A, Solieman N, Kurukumbi M, Kalyanam J (2012) Myasthenia gravis: a review. Autoimmune Dis 2012:874680. doi:10.1155/2012/874680 2. Lepore FE (1988) Unilateral ptosis and Hering’s law. Neurology 38:319–322 3. Erb MH, Kersten RC, Yip CC, Hudak D, Kulwin DR, McCulley TJ (2004) Effect of unilateral blepharoptosis repair on contralateral eyelid position. Ophthal Plast Reconstr Surg 20:418–422 4. Oosterhuis HJGH (1982) The ocular signs and symptoms of myasthenia gravis. Doc Ophthalmol 52:363–378 5. Komiyama A, Arai H, Kijima M, Hirayama K (2000) Extraocular muscle responses to high dose intravenous methylprednisolone in myasthenia gravis. J Neurol Neurosurg Psychiatry 68:214–217
Fig. 1 Ptosis in a patient with ocular myasthenia gravis. The patient showed right ptosis when referred to our hospital (a). Edrophonium injection improved right ptosis and induced minor left ptosis (b). After intravenous methylprednisolone therapy, right ptosis was satisfactorily relieved, however, left ptosis got appeared (c). Left ptosis was resolved after 1-month observation without additional immunosuppressive therapy (d)
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LETTER TO THE EDITOR
Eyelid ptosis enhanced after steroid pulse therapy in ocular myasthenia gravis: a case report Haruo Nishijima • Tatsuya Ueno • Chieko Suzuki Masayuki Baba • Masahiko Tomiyama
•
Received: 5 November 2014 / Accepted: 21 January 2015 Ó Springer-Verlag Italia 2015
Dear Editor, Eyelid ptosis is a frequent symptom in myasthenia gravis and severity of ptosis fluctuates along with the disease course [1]. Here, we describe a patient with ocular myasthenia gravis, presenting with ptosis in the contralateral side to primary affected side after edrophonium (Tensilon) test and steroid pulse therapy. A 39-year-old Japanese woman presented with a 3-month history of diplopia and right eyelid ptosis (Fig. 1a). Her symptoms had diurnal variation, worsened in the evening, and improved after rest. There were no symptoms or signs of weakness of facial, neck, bulbar, limb, or respiratory muscles. The edrophonium test disclosed improvement of the right ptosis and induced minor left ptosis (Fig. 1b). Repetitive nerve stimulation of the deltoid muscles and abductor digiti minimi muscles showed no significant amplitude decrements. Serum acetylcholine receptor antibodies and muscle-specific tyrosine kinase antibodies were both negative. Chest computed tomography was negative for thymoma. Magnetic resonance imaging of the orbits revealed no abnormalities. Ambenonium chloride (20 mg/day) resulted in improvement of ptosis and diplopia. We diagnosed her illness as double-seronegative ocular myasthenia gravis. Her H. Nishijima (&) T. Ueno C. Suzuki M. Baba M. Tomiyama Department of Neurology, Aomori Prefectural Central Hospital, 2-1-1 Higashi-tsukurimichi, Aomori City, Aomori 030-8553, Japan e-mail: [email protected] H. Nishijima T. Ueno M. Tomiyama Department of Neurophysiology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Hirosaki City, Aomori, Japan
symptoms gradually worsened in the following 2 months. Oral prednisolone therapy (5 mg/day) was started and her symptoms slightly improved. She still had a difficulty in her daily activities, but refused to continue oral prednisolone therapy, because concerned about adverse effect due to long-term treatment with steroid. Then, she was admitted to our hospital, stopped to taking oral steroid, and intravenous methylprednisolone therapy (1 g/day for 3 days) was commenced. After three courses of intravenous methylprednisolone therapy with 4-day intervals between the courses, right eyelid ptosis satisfactorily improved; however, left eyelid ptosis appeared during the second course of the therapy (Fig. 1c). Left eyelid ptosis did not have diurnal variation. The edrophonium test was negative and repetitive nerve stimulation of the left orbicularis oculi showed no significant amplitude decrements. After 1-month observation without additional immunosuppressive treatment, left ptosis completely disappeared (Fig. 1d). Diplopia completely improved after the first course of the therapy and did not recur in this observation period. We consider that our patient’s left ptosis might have been induced by rapid improvement of right eyelid ptosis with steroid pulse therapy. Hering’s law of equal innervations as applied to the elevator palpebrae superioris could be a likely explanation of this phenomenon, as in the case with surgical treatment-induced contralateral ptosis [2, 3]. Central nervous system compensates for peripheral weakness of the extraocular muscles in a patient with ocular myasthenia gravis. To keep the dominant right eyelid in the primary position, an increased innervation to the right elevator muscle is needed. The same increase of innervation is provided to the non-paretic contralateral synergist of the left eyelid. Rapid improvement of right ptosis with steroid pulse therapy may unmask this central adaptation,
123
Neurol Sci
that is, induce decrement of these innervations, which results in relatively hypo-innervation to left eyelid elevator [4, 5]. Enhanced ptosis contralateral to the primary affected side after steroid therapy in a patient with myasthenia gravis was rarely reported. Steroid pulse therapy is not common in treatment of myasthenia gravis, considering well-known frequent complication, initial exacerbation. However, it has been reported that intravenous methylprednisolone therapy for ocular myasthenia gravis induced more rapid induction of disease remission than oral steroid therapy [5]. Central adaptation may be lost after gradual recovery, which could explain the absence of a similar phenomenon in a treatment with oral steroid therapy. Conflict of interest We have no financial support or relationships that may pose conflict of interest.
References 1. Jayam Trouth A, Dabi A, Solieman N, Kurukumbi M, Kalyanam J (2012) Myasthenia gravis: a review. Autoimmune Dis 2012:874680. doi:10.1155/2012/874680 2. Lepore FE (1988) Unilateral ptosis and Hering’s law. Neurology 38:319–322 3. Erb MH, Kersten RC, Yip CC, Hudak D, Kulwin DR, McCulley TJ (2004) Effect of unilateral blepharoptosis repair on contralateral eyelid position. Ophthal Plast Reconstr Surg 20:418–422 4. Oosterhuis HJGH (1982) The ocular signs and symptoms of myasthenia gravis. Doc Ophthalmol 52:363–378 5. Komiyama A, Arai H, Kijima M, Hirayama K (2000) Extraocular muscle responses to high dose intravenous methylprednisolone in myasthenia gravis. J Neurol Neurosurg Psychiatry 68:214–217
Fig. 1 Ptosis in a patient with ocular myasthenia gravis. The patient showed right ptosis when referred to our hospital (a). Edrophonium injection improved right ptosis and induced minor left ptosis (b). After intravenous methylprednisolone therapy, right ptosis was satisfactorily relieved, however, left ptosis got appeared (c). Left ptosis was resolved after 1-month observation without additional immunosuppressive therapy (d)
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