Quality of life in purely ocular myasthenia in Japan
Tóm tắt
Since there has been no conclusive evidence regarding the treatment of ocular myasthenia, treatment guidelines were recently issued by the European Federation of Neurological Societies/European Neurological Society (EFNS/ENS). However, the therapeutic outcomes concerning the quality-of-life (QOL) of patients with ocular myasthenia are not yet fully understood. We investigated the therapeutic outcomes of patients with purely ocular myasthenia in a multicenter cross-sectional survey in Japan. To evaluate the severity of ocular symptoms, we used the ocular-quantitative MG (QMG) score advocated by Myasthenia Gravis Foundation of America. We used the Japanese translated version of the MG-QOL15, a self-appraised scoring system. Of 607 myasthenia gravis (MG) patients with an observation-duration of illness ≥ 2 years, the cases of 123 patients (20%) were limited to ocular muscles (purely ocular myasthenia). During the entire clinical course, 81 patients experienced both ptosis and diplopia, 36 had ptosis alone, and six had diplopia alone. Acetyl-cholinesterase inhibitors and prednisolone were used in 98 and 52 patients, respectively. Treatment improved ocular symptoms, with the mean reduction in ocular-QMG score of 2.3 ± 1.8 points. However, 47 patients (38%) failed to gain minimal manifestation or a better status. Patients with unfavorable outcomes also self-reported severe QOL impairment. Multivariate analyses showed that the pretreatment ocular-QMG score was associated with unfavorable outcomes, but not associated with the patient’s QOL. A treatment strategy designed in accord with a patient's ocular presentation must be considered in order to improve ocular symptoms and the patient's QOL.
Tài liệu tham khảo
citation_journal_title=Lancet Neurol; citation_title=Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity; citation_author=MN Meriggioli, DB Sanders; citation_volume=8; citation_publication_date=2009; citation_pages=475-490; citation_doi=10.1016/S1474-4422(09)70063-8; citation_id=CR1
citation_journal_title=Neuromuscul Disord; citation_title=Therapeutic options in ocular myasthenia gravis; citation_author=A Evoli, AP Batocchi, C Minisci, C Di Schino, P Tonali; citation_volume=11; citation_publication_date=2001; citation_pages=208-216; citation_doi=10.1016/S0960-8966(00)00173-5; citation_id=CR2
citation_journal_title=Exp Neurol; citation_title=Complement regulators in extraocular muscle and experimental autoimmune myasthenia gravis; citation_author=HJ Kaminski, Z Li, C Richmonds, F Lin, ME Medof; citation_volume=189; citation_publication_date=2004; citation_pages=333-342; citation_doi=10.1016/j.expneurol.2004.06.005; citation_id=CR3
citation_journal_title=Muscle Nerve; citation_title=Lifetime course of myasthenia gravis; citation_author=D Grob, N Brunner, T Namba, M Pagala; citation_volume=37; citation_publication_date=2008; citation_pages=141-149; citation_doi=10.1002/mus.20950; citation_id=CR4
citation_journal_title=Ann Neurol; citation_title=Prognosis of ocular myasthenia; citation_author=CT Bever, AV Aquino, AS Penn, RE Lovelace, LP Rowland; citation_volume=14; citation_publication_date=1983; citation_pages=516-519; citation_doi=10.1002/ana.410140504; citation_id=CR5
citation_journal_title=J Neurol Neurosurg Psychiatry; citation_title=Ocular myasthenia gravis: response to long-term immunosuppressive treatment; citation_author=N Sommer, B Sigg, A Melms, M Weller, K Schepelmann, V Herzau, J Dichgans; citation_volume=62; citation_publication_date=1997; citation_pages=156-162; citation_doi=10.1136/jnnp.62.2.156; citation_id=CR6
citation_journal_title=Arch Neurol; citation_title=Development of generalized disease at 2 years in patients with ocular myasthenia gravis; citation_author=MJ Kupersmith, R Latkany, P Homel; citation_volume=60; citation_publication_date=2003; citation_pages=243-248; citation_doi=10.1001/archneur.60.2.243; citation_id=CR7
citation_journal_title=J Neuroophthalmol; citation_title=Immunotherapy of ocular myasthenia gravis reduces conversion to generalized myasthenia gravis; citation_author=J Mee, M Paine, E Byrne, J King, K Reardon, J O'Day; citation_volume=23; citation_publication_date=2003; citation_pages=251-255; citation_doi=10.1097/00041327-200312000-00002; citation_id=CR8
citation_journal_title=J Neurol Sci; citation_title=The effect of prednisone on the progression from ocular to generalized myasthenia gravis; citation_author=NT Monsul, HS Patwa, AM Knorr, RL Lesser, JM Goldstein; citation_volume=217; citation_publication_date=2004; citation_pages=131-133; citation_doi=10.1016/j.jns.2003.08.017; citation_id=CR9
citation_journal_title=Muscle Nerve; citation_title=Ocular myasthenia gravis in a senior population: diagnosis, therapy, and prognosis; citation_author=JA Allen, S Scala, HR Jones; citation_volume=41; citation_publication_date=2010; citation_pages=379-384; citation_doi=10.1002/mus.21555; citation_id=CR10
citation_journal_title=Eur J Neurol; citation_title=The effect of early prednisolone treatment on the generalization rate in ocular myasthenia gravis; citation_author=H Zach, H Cetin, E Hilger, A Paul, B Wuschitz, R Jung, E Auff, F Zimprich; citation_volume=20; citation_publication_date=2013; citation_pages=708-713; citation_doi=10.1111/ene.12057; citation_id=CR11
citation_journal_title=Cochrane Database Syst Rev; citation_title=Medical and surgical treatment for ocular myasthenia; citation_author=M Benatar, H Kaminski; citation_volume=2; citation_publication_date=2006; citation_pages=CD005081; citation_id=CR12
Kerty E, Elsais A, Argov Z, Evoli A, Gilhus NE: EFNS/ENS Guidelines for the treatment of ocular myasthenia. Eur J Neurol. doi: 10.1111/ene.12359
citation_journal_title=Neurology; citation_title=Myasthenia gravis: recommendations for clinical research standards. Task Force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America; citation_author=A Jaretzki, RJ Barohn, RM Ernstoff, HJ Kaminski, JC Keesey, AS Penn, DB Sanders; citation_volume=55; citation_publication_date=2000; citation_pages=16-23; citation_doi=10.1212/WNL.55.1.16; citation_id=CR14
citation_journal_title=Neurology; citation_title=Treatment of ocular symptoms in myasthenia gravis; citation_author=MJ Bhanushali, J Wuu, M Benatar; citation_volume=71; citation_publication_date=2008; citation_pages=1335-1341; citation_doi=10.1212/01.wnl.0000327669.75695.38; citation_id=CR15
citation_journal_title=J Neuroimmunol; citation_title=Clinical and immunological differences between early and late-onset myasthenia gravis in Japan; citation_author=S Suzuki, K Utsugisawa, Y Nagane, T Satoh, M Kuwana, N Suzuki; citation_volume=230; citation_publication_date=2011; citation_pages=148-152; citation_doi=10.1016/j.jneuroim.2010.10.023; citation_id=CR16
citation_journal_title=Muscle Nerve; citation_title=The MG-QOL15 Japanese version: validation and associations with clinical factors; citation_author=M Masuda, K Utsugisawa, S Suzuki, Y Nagane, C Kabasawa, Y Suzuki, Y Shimizu, H Utsumi, K Fujihara, S Uchiyama, N Suzuki; citation_volume=46; citation_publication_date=2012; citation_pages=166-173; citation_doi=10.1002/mus.23398; citation_id=CR17
citation_journal_title=Arch Neurol; citation_title=Treatment of ocular myasthenia: steroids only when compelled; citation_author=HJ Kaminski, RB Daroff; citation_volume=57; citation_publication_date=2000; citation_pages=752-753; citation_doi=10.1001/archneur.57.5.752; citation_id=CR18
citation_journal_title=J Neurol Neurosurg Psychiatry; citation_title=Extraocular muscle responses to high dose intravenous methylprednisolone in myasthenia gravis; citation_author=A Komiyama, H Arai, M Kijima, K Hirayama; citation_volume=68; citation_publication_date=2000; citation_pages=214-217; citation_doi=10.1136/jnnp.68.2.214; citation_id=CR19
citation_journal_title=Muscle Nerve; citation_title=Topical naphazoline in the treatment of myasthenic blepharoptosis; citation_author=Y Nagane, K Utsugisawa, S Suzuki, M Masuda, Y Shimizu, H Utsumi, S Uchiyama, N Suzuki; citation_volume=44; citation_publication_date=2011; citation_pages=41-44; citation_doi=10.1002/mus.22002; citation_id=CR20
citation_journal_title=Eur Neurol; citation_title=Is Surgical Intervention Safe and Effective in the Treatment of Myasthenic Blepharoptosis? A Multicenter Survey in Japan; citation_author=Y Shimizu, S Suzuki, K Utsugisawa, T Imai, H Murai, Y Nagane, E Tsuda, T Nagasao, H Ogata, M Yazawa, K Suzuki, K Kishi; citation_volume=71; citation_publication_date=2014; citation_pages=259-261; citation_doi=10.1159/000356793; citation_id=CR21