11. tbl. 109. árg. 2023
A 50 year old man with progressive weakness in lower limbs
Vaxandi máttleysi í ganglimum hjá fimmtugum manni • Sjúkratilfelli •
Brynhildur Thors1
Bjarni Guðmundsson2
1Neurological department Karolinska University Hospital, Stockholm
2Neurology department Landspitali-University Hospital.
Correspondence: Brynhildur Thors, bthors@gmail.com
Key words: Lambert-Eaton Myasthenic Syndrome, proximal weakness, neuromuscular junction disorder, repetitive nerve stimulation, paraneoplastic phenomenon.
Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare neurological disorder caused by autoimmune antibodies attacking the presynaptic neuromuscular junction, in some cases caused by underlying cancer. The main clinical finding is fluctuating weakness of the extremities and a triad of symtoms can help physicians suspect the disease. A key to the diagnosis are the electrophysiological abnormalities seen in this group of diseases. Treatment with symtomatic and/or immunosuppressive therapy is important as well as a workup for possible malignancy. This article identifies the clinical features, diagnosis and treatment of this uncommon disease.