EFNS/ENS Guidelines for the treatment of ocular myasthenia
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).
Available from: Norihiro Suzuki
- "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 . However, the EFNS/ENS guidelines recommend that the treatment of ocular myasthenia should initially be started with pyridostigmine . 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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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.
BMC Neurology 07/2014; 14(1):142. DOI:10.1186/1471-2377-14-142 · 2.04 Impact Factor
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ABSTRACT: In this issue, we review studies of the genetic risk of developing polyneuropathy from the chemotherapeutic agent paclitaxel. Increased susceptibility was related to PRX and ARHGEF10 mutations and to genes associated with axonogenesis. Several articles regarding the use of subcutaneous administration of intravenous immunoglobulin for treatment of neuropathies were also reviewed. We also comment on 2 recent studies that evaluated how intravenous immunoglobulin modulates the immune attack in chronic inflammatory demyelinating polyneuropathy. On a somewhat related topic, a recent study from Taiwan also addressed whether childhood allergic disorders increase the risk of developing another autoimmune disease, namely myasthenia gravis (MG). An article regarding a potential new MG autoantigen, cortactin, was assessed. A study of outcome measures for MG and another regarding a new mutation—in SNAP25B—in congenital myasthenic syndrome were covered. Regarding myopathy, articles involving neuromuscular respiratory dysfunction and hereditary myopathy with early respiratory failure and interesting histopathologic features were analyzed. Results of a large study on congenital myopathy from the Dubowitz Neuromuscular Centre were summarized, and a study of adult-onset centronuclear myopathy was covered. Results of a bimagrumab trial in inclusion body myositis are reported. Regarding amyotrophic lateral sclerosis (ALS), we reviewed studies regarding the category of ALS-Plus and associated features and in medical conditions that may be associated with risk of developing ALS.
Journal of Clinical Neuromuscular Disease 09/2014; 16(1):32-41. DOI:10.1097/CND.0000000000000045
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ABSTRACT: Dear Sir,Giant cell arteritis (GCA) is infrequently reported as a cause of acute isolated monolateral third nerve palsy . 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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