Syndrome myasthénique de Lambert Eaton : Revue clinique
Posté : 16 août 2016 10:34
Lu sur http://onlinelibrary.wiley.com/wol1/doi ... 6/abstract
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Lambert–Eaton myasthenic syndrome: Clinical review
Masakatsu Motomura1,2,*, Ruka Nakata2,3 andHirokazu Shiraishi2
Version of Record online: 15 AUG 2016
DOI: 10.1111/cen3.12326
© 2016 Japanese Society for Neuroimmunology
Abstract
Lambert–Eaton myasthenic syndrome (LEMS) is an autoimmune disease of the neuromuscular junction and approximately 60% of LEMS patients have a tumor, mostly small cell lung cancer, as a paraneoplastic neurological syndrome.
LEMS patients develop a unique set of clinical characteristics, which include proximal muscle weakness, depressed tendon reflexes with post-tetanic potentiation and autonomic symptoms. Interestingly, slightly <10% of LEMS patients have cerebellar ataxia (LEMS with paraneoplastic cerebellar degeneration). Considering its pathomechanism, LEMS is a presynaptic disorder of neuromuscular transmission in which quantal release of acetylcholine is impaired by autoantibodies for P/Q-type voltage-gated calcium channels at active zones, although an animal model by immunizing purified P/Q-type voltage-gated calcium channels has not yet been successful.