ARTICLE
Guidelines for treatment of autoimmune neuromuscular
transmission disorders
G. O. Skeiea, S. Apostolskib, A. Evolic, N. E. Gilhusd, I. Illae, L. Harmsf, D. Hilton-Jonesg,
A. Melmsh, J. Verschuureni and H. W. Horgej
aDepartment of Neurology, University of Bergen, Norway; bInstitute of Neurology, School of Medicine, University of Belgrade, Serbia and
Montenegro; cNeuroscience Department, Catholic University, Rome, Italy; dDepartment of Neurology, University of Bergen, Norway; eServei
Neurologia, Hospital Sta. Creu i Sant Pau, Barcelona, Ciberned, Spain; fUniversita¨tsmedizin Berlin Charite´, Neurologische Klinik Berlin,
Germany; gRadcliffe Infirmary, Oxford, UK; hNeurologische Klinik, Universita¨t Tu¨bingen, Germany; iDepartment of Neurology, LUMC,
Leiden, The Netherlands; and jThe Norwegian Musculary Disorders Association, Norway
Keywords:
Lambert–Eaton
myasthenic syndrome,
myasthenia gravis,
neuromuscular
transmission disorders,
neuromyotonia
Received 8 January 2010
Accepted 19 February 2010
Background: Important progress has been made in our understanding of the autoimmune
neuromuscular transmission (NMT) disorders; myasthenia gravis (MG),
Lambert–Eaton myasthenic syndrome (LEMS) and neuromyotonia (Isaacs syndrome).
Methods: To prepare consensus guidelines for the treatment of the autoimmune
NMT disorders, references retrieved from MEDLINE, EMBASE and the Cochrane
Library were considered and statements prepared and agreed on by disease experts.
Conclusions: Anticholinesterase drugs should be given first in the management of
MG, but with some caution in patients with MuSK antibodies (good practice point).
Plasma exchange is recommended in severe cases to induce remission and in preparation
for surgery (recommendation level B). IvIg and plasma exchange are effective
for the treatment of MG exacerbations (recommendation level A). For patients with
non-thymomatous MG, thymectomy is recommended as an option to increase the
probability of remission or improvement (recommendation level B). Once thymoma is
diagnosed, thymectomy is indicated irrespective of MG severity (recommendation
level A). Oral corticosteroids are first choice drugs when immunosuppressive drugs are
necessary (good practice point). When long-term immunosuppression is necessary,
azathioprine is recommended to allow tapering the steroids to the lowest possible dose
whilst maintaining azathioprine (recommendation level A). 3,4-Diaminopyridine is
recommended as symptomatic treatment and IvIG has a positive short-term effect in
LEMS (good practice point). Neuromyotonia patients should be treated with an antiepileptic
drug that reduces peripheral nerve hyperexcitability (good practice point).
For paraneoplastic LEMS and neuromyotonia optimal treatment of the underlying
tumour is essential (good practice point). Immunosuppressive treatment of LEMS and
neuromyotonia should be similar to MG (good practice point).
Background and objectives
Autoimmune neuromuscular transmission (NMT) disorders
are relatively rare, but often debilitating diseases.
Myasthenia gravis (MG) is caused by autoantibodies
against components of the post-synaptic neuromuscular
junction. The autoimmune attack at the muscle endplate
leads to NMT failure and muscle weakness.
Lambert–Eaton myasthenic syndrome (LEMS) is
caused by antibodies against the voltage-gated calcium
channels (VGCC) at the pre-synaptic side of the muscle
endplate. The antibodies inhibit acetylcholine release
and cause NMT failure and muscle weakness. Neuromyotonia
(peripheral nerve hyperexcitability; Isaacs
syndrome) is caused by antibodies to nerve voltagegated
potassium channels (VGKC) that produce nerve
hyperexcitability and spontaneous and continuous
Correspondence: Geir Olve Skeie (chair), Department
of Neurology, Haukeland University Hospital, 5021 Bergen, Norway
(tel.: +47 55 97 5000; fax: +55 97 51 65;
e-mail: Tato e-mailová adresa je chráněna před spamboty. Pro její zobrazení musíte mít povolen Javascript.).
This is continuing Medical Education article, and can be found with
corresponding questions on the internet at http://www.efns.org/EFNS
Continuing-Medical-Education-online.301.0.html. Certificates for
correctly answering the questions will be issued by the EFNS.
 2010 The Author(s)
Journal compilation  2010 EFNS 893