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Myasthenia gravis (MG) is characterized by muscle weakness caused by autoantibodies that bind to the postsynaptic membrane at the neuromuscular junction and impair acetylcholine receptor function. Weakness of respiratory muscles represents the most severe MG manifestation, and 10–15% of all patients experience an MG crisis with the need of mechanical ventilatory support at least once in their life. MG patients with respiratory muscle weakness need active immunosuppressive drug treatment long term, and they need regular specialist follow-up. Comorbidities affecting respiratory function need attention and optimal treatment. Respiratory tract infections can lead to MG exacerbations and precipitate an MG crisis. Intravenous immunoglobulin and plasma exchange are the core treatments for severe MG exacerbations. High-dose corticosteroids, complement inhibitors, and FcRn blockers represent fast-acting treatments that are effective in most MG patients. Neonatal myasthenia is a transient condition with muscle weakness in the newborn caused by mother's muscle antibodies. In rare cases, treatment of respiratory muscle weakness in the baby is required. [ABSTRACT FROM AUTHOR]

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) can be treated with corticosteroids or intravenous immunoglobulins. Various corticosteroid regimens are currently used in CIDP, but it is unknown whether they are equally efficacious. In this retrospective study, we compared efficacy and safety of three corticosteroid regimens in CIDP patients.Methods: We included treatment naïve patients that fulfilled the EFNS/PNS criteria for CIDP. Patients were treated with corticosteroids according to the local protocol of three CIDP expertise centres. Corticosteroid regimens consisted of daily oral prednisolone, pulsed oral dexamethasone, or pulsed intravenous methylprednisolone. Outcomes were number of responders to treatment, remission rate of treatment responders, overall probability of 5-year remission, and the occurrence of adverse events.Results: A total of 125 patients were included. Sixty-seven (54%) patients received daily prednisone or prednisolone, 37 (30%) pulsed dexamethasone, and 21 (17%) pulsed intravenous methylprednisolone. Overall, 60% (95% CI 51-69%) responded to corticosteroids, with no significant difference between the three treatment regimens (p = 0.56). From the 75 responders, 61% (95% CI 50-73%) remained in remission, during a median follow-up of 55 months (range 1-197 months). The probability of responders reaching 5-year remission was 55% (95% Cl 44-70%), with no difference between the three groups. Adverse events leading to a change in treatment occurred in ten patients (8%). Two patients had a serious adverse event.Conclusion: Corticosteroids lead to improvement in 60% of patients and to remission in 61% of treatment responders. There were no differences between treatment modalities in terms of efficacy and safety. [ABSTRACT FROM AUTHOR]

Patients suffering from multiple sclerosis (MS) are treated with high-dose intravenous steroids during acute relapses. In case of steroid refractory relapses, patients are treated with plasmapheresis or immunoadsorption. Until now data concerning the efficacy of both procedures are scarce. Visual evoked potentials (VEP), visual acuity and degree of remission of deficits caused by a relapse that had led to admission in MS patients ( n = 48) treated with PLEX, IA or both in a single university centre setting were evaluated retrospectively. In a grouped analysis of patients treated with combined PLEX/IA, PLEX or IA alone, patients in all groups profited as assessed by VEP. Visual acuity also showed a trend towards a better performance, but lacked significance. In a subgroup analysis only concerning patients with initially pathological VEP there was a significant beneficial effect in the groups treated with PLEX/IA as well as in the group summarizing all patients. The combination of PLEX and IA provides a valid treatment option in steroid-refractory MS-relapses, and IA should be considered in acute relapses especially in patients with side effect of PLEX. [ABSTRACT FROM AUTHOR]

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