EFNS/ENS Guidelines for the treatment of ocular myasthenia

Abstract

Background and purpose: 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. Search strategy: 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. Conclusions: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).

Outcomes of Preoperative and Postoperative Corticosteroid Therapies in Myasthenia Gravis

“…The consistent production of anti-AChR abs has been found to be elevated in 50% of patients with OMG and 90% of patients with GMG [36] . Some studies have focused on thymectomy in patients with OMG [25,[35][36][37][38] . Thymectomy has been shown to promote remission in all groups of patients, including patients with OMG [21,22,25,26] .…”

Precision medicine in myasthenia graves: begin from the data precision

“…MG with thymoma is considered as a paraneoplastic syndrome, which has different clinical and immunological features and different mechanisms compared with nonthymoma patients (5,11). Current guidelines of MG treatment do not recommend thymectomy in ocular MG and AChR antibody negative patients (12)(13)(14). Thus, pathological data on thymus is not available in all MG patients.…”

Myasthenia gravis – diagnostikk og behandling

“…Ytterligere noen har antistoffer mot muskelspesifikk kinase (MuSK). Hos pasienter med myasthenia gravis og rent okulaere symptomer har bare omkring halvparten påvisbare antistoffer, og da alltid mot acetylkolinreseptor (10). Måling av antistoffer mot acetylkolinreseptor skal utføres hos alle der det er klinisk mistanke om sykdommen.…”

“…Dersom pasienten etter to år fortsatt kun har okulaere symptomer, er det mest sannsynlig at sykdommen forblir okulaer, selv om generalisering med alvorlige symptomer kan skje også etter lang tid (10). Antistoffer mot acetylkolinereseptor, MuSK og lipoproteinrelatert protein 4 (LRP4) finnes omtrent aldri samtidig.…”

“…Det er holdepunkter for at immunsuppressiv behandling ved okulaer myasthenia gravis reduserer risikoen for generalisering av symptomene (10). For denne subgruppen kan en liten dose prednisolon vaere tilstrekkelig.…”