Corticosteroid use in the treatment of neuromuscular disorders: Empirical and evidence‐based data

Abstract

Corticosteroids have been used to treat neuromuscular disorders for many years. With few randomized, controlled trials, efficacy has been established primarily from empirical data. This has led to a range of treatment regimens varying in terms of initial dosing, dosing schedules, and taper rates. The goals of this review were to examine the literature for data concerning corticosteroid pharmacokinetics and for evidence-based treatment regimens in several prototypic neuromuscular disorders. The results provide a number of sound principles for corticosteroid use, but also indicate that corticosteroid regimens and patient management are largely based on empirical clinical experiences.

Myasthenic crisis: Guidelines for prevention and treatment

“…The principles of use in patients with myasthenia gravis are similar to their application in other immune-mediated systemic and neuromuscular diseases such as systemic lupus erythematosus, chronic inflammatory demyelinating polyneuropathy (CIDP) and acquired inflammatory muscle diseases among others [40]. Historically patients with MG were among the first to be treated with corticosteroid therapy [41][42][43].…”

“…Historically patients with MG were among the first to be treated with corticosteroid therapy [41][42][43]. However there is still a lack of controlled prospective and randomized trials [39,40].…”

Immunomodulatory Therapies in Neurologic Critical Care

“…Corticosteroids likely hasten motor recovery in acute MG [56] but, in about 50% of patients, their use is associated with a transient worsening of weakness (beginning at an average of 5 days after initiation, range 1-21 days) [57]. This is thought to be related in part to lysis of lymphocytes with release of immunoglobulin and in part due to direct effects on the neuromuscular junction [57].…”

“…This is thought to be related in part to lysis of lymphocytes with release of immunoglobulin and in part due to direct effects on the neuromuscular junction [57]. Multiple treatment regimens exist.…”

“…A common regimen is oral prednisone at 10-100 mg/d (often 60 or 80 mg/d) or 100 mg QOD followed by a slow taper over weeks to months. An alternative is high dose pulse therapy (2 g IV methylprednisolone repeated every 5 days for a maximum of three doses) [57]. To mitigate transient worsening, many centers concurrently treat with either IVIG or plasmapheresis [58].…”