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Lambert-Eaton syndrome

_ERN-NMD DESEASES_

Versione in italiano

WHAT IS
Lambert-Eaton myasthenic syndrome (LEMS) is a rare disease that affects the junction between muscle and nerve (neuromuscular junction). It is an autoimmune disease, in which our own immune system produces damaging antibodies. In about 50-60% of cases, it is associated to lung cancer.

HOW DO WE RECOGNIZE IT
The onset is in adult age. The main symptom is weakness of thighs and shoulders, but the disease can eventually involve every muscle. Other common symptoms that may accompany the weakness are dry mouth, erectile dysfunction, and constipation. The most important assessments are electromyography (especially repetitive nerve stimulation test) and blood test for pathologic antibodies (anti-VGCC). Once the diagnosis of LEMS is made, TC and/or PET-TC are usually required to exclude a simultaneous cancer. If negative, these exams should be repeated periodically in the following months.

HOW GETS SICK
3,4 diaminopyridine is useful to reduce weakness. If a cancer is found, its treatment usually implies LEMS remission. Anyway, resistant forms might respond to immune suppressive treatments or intravenous immune globulines.

HOW DO WE TREAT IT
Through a detailed neurological and neurophysiological evaluation, we can get a precise diagnosis and establish the appropriate treatment. Furthermore, multidisciplinary evaluation will be performed to rule out solid tumors.

WHAT DO WE DO IN OSPEDALE SAN RAFFAELE
The diagnosis is performed by a team of physicians specialized in muscle disorders based on clinical evaluation, electromyography and muscle biopsy.


REFERRING DOCTORS for this desease

at Ospedale San Raffaele

Ospedale San Raffaele - Milano, via Olgettina 60, 20132 Milano, Italia - Tel. 02 26 431
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