Lambert-Eatons myastena syndrom (LEMS) (563)

Tests in package

Acetylcholine receptor antibodies, Amphiphysin antibodies, GAD antibodies, Hu antibodies, Ri (Nova 1/ ANNA-2) antibodies, SOX1 antibodies, VGCC (voltage-gated calcium channel) antibodies

Clinic

Lambert-Eaton myasthenic syndrome (LEMS) was first described in 1953 by HJ Anderson and his associates as a muscular fatigue (myasthenia), in combination with lung cancer. The American doctors EH Lambert, LM Eaton and ED Rooke noted in 1956 that the symptoms were due to an impaired impulse transmission from nerve to muscle (neuromuscular transmission disorder). For practical reasons, the disease is divided into two groups:

  • Paraneoplastic LEMS, which means that the disease is present along with malignancy, usually small cell lung cancer. More than 50% of the patients have this form of the disease.
  • Idiopathic LEMS, which means that you cannot find the triggering cause of the illness.

The international medical literature indicates a prevalence of one person per 100000 inhabitants.

The paraneoplastic form is slightly more common in men than in women. Just as many men as women have the idiopathic form, which in rare cases can occur in children before the age of 10. It is likely that about 3 percent of all small cell lung cancers also have Lambert-Eaton myasthenic syndrome.

Both forms of Lambert-Eaton syndrome are autoimmune. The Lambert-Eaton myasthenic syndrome is associated with autoantibodies against proteins that form channels for the transport of calcium into the nerve terminal. The autoantibodies inhibit the influx of calcium into nerve ends, which in turn leads to a decreased release of the neurotransmitter acetylcholine, which typically transmit nerve impulses to muscles. The cause of antibody formation is unknown.

It is possible to analyze blood samples for antibodies against voltage-dependent calcium channels (VGCC). This test is positive in almost all of the paraneoplastic forms, and slightly less with the idiopathic. The Lambert-Eaton myasthenic syndrome can also be associated with other autoantibodies and the tests that are included in Wieslabs analysis package may in addition be used for differential diagnosis.

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References

1.  Farrugia ME, Vincent A. Autoimmune mediated neuromuscular junction defects. Curr Opin Neurol 2010; 23: 489-495.

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