is characterised by proximal muscle weakness with picture is characterised by proximal muscle augmentation of strength after exercise, mild weakness with augmentation of strength after oculomotor signs, depressed deep tendon reflexes exercise, mild oculomotor signs, depressed deep and autonomic dysfunction (dry mouth, tendon reflexes and autonomic dysfunction (dry constipation, erectile failure). mouth, constipation, erectile failure). Diagnosis should be confirmed by the presence of antibodies to Voltage gated calcium channels, electrophysiology consistent with LEMS, or both. Justification In the Biotext (2004) review, one systematic review In the Biotext (2004) review, one systematic Section has been revised including for Evidence (containing one RCT with 9 patients) and one case review (containing one RCT with 9 patients) and evidence for Ig treatment of Category series of 7 patients with a crossover design were one case series of 7 patients with a crossover paraneoplastic LEMS. included. IVIg appeared to provide some benefit to design were included. IVIg appeared to provide patients with LEMS. However, both studies only some benefit to patients with LEMS. However, included a small number of patients. both studies only included a small number of patients. Expert consensus states that IVIg produces temporary improvement in patients with LEMS. It Expert consensus states that IVIg produces therefore has a role as second line therapy temporary improvement in patients with LEMS. It IVIg Advisory Board 2004). therefore has a role as second line therapy IVIg Advisory Board 2004). Treatment of One submission to the National Blood Authority LEMS with 3,4DAP and pyridostigmine has been reported on a randomised controlled trial that shown to be efficacious in an RCT (Wirtz Clinical showed significant improvement in strength pharmacology & Therapeutics; VOLUME 86 associated with a decline in the level of pathogenic NUMBER 1, JULY 2009) and should be regarded antibodies (NSW IVIg User Group). as combined firstline therapy. 3,4DAP is not a licensed medication in Australia but can be
is characterised by proximal muscle weakness with augmentation of strength after exercise, mild oculomotor signs, depressed deep tendon reflexes and autonomic dysfunction (dry mouth, constipation, erectile failure). picture is characterised by proximal muscle weakness with augmentation of strength after exercise, mild oculomotor signs, depressed deep tendon reflexes and autonomic dysfunction (dry mouth, constipation, erectile failure). Diagnosis should be confirmed by the presence of antibodies to Voltage gated calcium channels, electrophysiology consistent with LEMS, or both. Justification for Evidence Category In the Biotext (2004) review, one systematic review (containing one RCT with 9 patients) and one case series of 7 patients with a crossover design were included. IVIg appeared to provide some benefit to patients with LEMS. However, both studies only included a small number of patients. Expert consensus states that IVIg produces temporary improvement in patients with LEMS. It therefore has a role as second line therapy IVIg Advisory Board 2004). One submission to the National Blood Authority reported on a randomised controlled trial that showed significant improvement in strength associated with a decline in the level of pathogenic antibodies (NSW IVIg User Group). In the Biotext (2004) review, one systematic review (containing one RCT with 9 patients) and one case series of 7 patients with a crossover design were included. IVIg appeared to provide some benefit to patients with LEMS. However, both studies only included a small number of patients. Expert consensus states that IVIg produces temporary improvement in patients with LEMS. It therefore has a role as second line therapy IVIg Advisory Board 2004). Treatment of LEMS with 3,4DAP and pyridostigmine has been shown to be efficacious in an RCT (Wirtz Clinical pharmacology & Therapeutics; VOLUME 86 NUMBER 1, JULY 2009) and should be regarded as combined firstline therapy. 3,4DAP is not a licensed medication in Australia but can be Section has been revised including evidence for Ig treatment of paraneoplastic LEMS.