EFNS/ENS Guidelines for the treatment of ocular myasthenia

European Journal of Neurology (Impact Factor: 4.06). 01/2014; 21(5). DOI: 10.1111/ene.12359
Source: PubMed


The symptoms of acquired autoimmune ocular myasthenia are restricted to the extrinsic eye muscles, causing double vision and drooping eyelids. These guidelines are designed to provide advice about best clinical practice based on the current state of clinical and scientific knowledge and the consensus of an expert panel.
Evidence for these guidelines was collected by searches in the MEDLINE and Cochrane databases. The task force working group reviewed evidence from original articles and systematic reviews. The evidence was classified (I, II, III, IV) and consensus recommendation graded (A, B or C) according to the EFNS guidance. Where there was a lack of evidence but clear consensus, good practice points are provided.
The treatment of ocular myasthenia should initially be started with pyridostigmine (good practice point). If this is not successful in relieving symptoms, oral corticosteroids should be used on an alternate-day regimen (recommendation level C). If steroid treatment does not result in good control of the symptoms or if it is necessary to use high steroid doses, steroid-sparing treatment with azathioprine should be started (recommendation level C). If ocular myasthenia gravis is associated with thymoma, thymectomy is indicated. Otherwise, the role of thymectomy in ocular myasthenia is controversial. Steroids and thymectomy may modify the course of ocular myasthenia and prevent myasthenia gravis generalization (good practice point).

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    • "An evidence-based review made no conclusion regarding whether it was appropriate to initiate therapy with AChE inhibitors or with corticosteroids for patients with ocular myasthenia [12]. However, the EFNS/ENS guidelines recommend that the treatment of ocular myasthenia should initially be started with pyridostigmine [13]. We agree with the principal concept of the EFNS/ENS guidelines’ recommendation and initially treated by AChE inhibitors in most patients with ocular myasthenia. "
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    ABSTRACT: Background 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. Methods 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. Results 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. Conclusion 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.
    BMC Neurology 07/2014; 14(1):142. DOI:10.1186/1471-2377-14-142 · 2.04 Impact Factor
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    ABSTRACT: Dear Sir,Giant cell arteritis (GCA) is infrequently reported as a cause of acute isolated monolateral third nerve palsy [1]. We report the case of a 77-year-old man who complained of 4-5 days of left palpebral ptosis and 1 day of low-grade fever. No orbital pain was reported. He had a medical history of ankylosing spondylitis, hypertension and one episode of angina pectoris.Neurological examination revealed fatigable left eye ptosis and diplopia after 5 s of ocular fixation, as well as partial left third nerve palsy with moderate adduction and depression deficits. The fourth and sixth cranial nerves were not involved. Pupils were equal and reactive to light; limb or bulbar weakness/fatigability and dyspnoea were absent. Routine haematological investigations showed raised erythrocyte sedimentation rate (ESR) 71 mm/h (nv
    Neurological Sciences 01/2015; 36(4). DOI:10.1007/s10072-015-2074-8 · 1.45 Impact Factor
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    ABSTRACT: Unlabelled: Current available therapies control Myasthenia gravis (MG) reasonably well, but Health Related Quality of life (HRQOL) remains lower than expected. The aim was provide insights in how HRQOL in MG stands across borders and time, compare the scores to general population controls and other chronic disorders and assess the impact of potential predictors for quality of life such as a) clinical characteristics b) antibodies c) thymoma and d) treatment in a population-based cohort. Methods: We designed a population-based cross-sectional study including 858 patients, 373 from Norway and 485 from the Netherlands. The Short Form Health Survey 36 (SF-36) and a cross-cultural validated questionnaire were used. Data were in addition compared to the general population, other chronic diseases and previous studies. Results: Mean physical composite score was 59.4 and mental composite score 69.0 with no differences between the countries. The mean HRQOL score was lower in patients with bulbar and generalized symptoms (p < 0.001) compared to sex and age adjusted healthy controls, but not in patients with ocular symptoms or patients in remission. Multivariate analysis revealed that female gender, generalized symptoms and use of secondary immunosuppressive drugs at the time of testing were risk factors for reduced HRQOL. Conclusions: Remission and absence of generalized symptoms were favorable factors for HRQOL in MG patients. Historically, the HRQOL levels have not changed since 2001 and no new clinical predictors could be detected in this exhaustive population-based study. Further studies should explore the impact of non clinical factors like ethnic variations, socio-economic and hormonal factors on HRQOL.
    Health and Quality of Life Outcomes 08/2015; 13(1):115. DOI:10.1186/s12955-015-0298-1 · 2.12 Impact Factor