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Lambert-Eaton Myasthenic Syndrome (Lambert-Eaton Syndrome)

An autoimmune disease characterized by weakness and fatigability of proximal muscles, particularly of the pelvic girdle, lower extremities, trunk, and shoulder girdle. There is relative sparing of extraocular and bulbar muscles. CARCINOMA, SMALL CELL of the lung is a frequently associated condition, although other malignancies and autoimmune diseases may be associated. Muscular weakness results from impaired impulse transmission at the NEUROMUSCULAR JUNCTION. Presynaptic calcium channel dysfunction leads to a reduced amount of acetylcholine being released in response to stimulation of the nerve. (From Adams et al., Principles of Neurology, 6th ed, pp 1471)
Also Known As:
Lambert-Eaton Syndrome; Eaton Lambert Syndrome; Syndrome, Lambert-Eaton; Syndrome, Lambert-Eaton Myasthenic; Eaton-Lambert Myasthenic Syndrome; Myasthenic-Myopathic Syndrome of Eaton-Lambert; Myasthenic-Myopathic Syndrome of Lambert-Eaton; Myopathic-Myasthenic Syndrome of Eaton-Lambert; Myopathic-Myasthenic Syndrome of Lambert-Eaton; Eaton Lambert Myasthenic Syndrome; Eaton-Lambert Myasthenic-Myopathic Syndrome; Eaton-Lambert Myopathic-Myasthenic Syndrome; Eaton-Lambert Myopathic-Myasthenic Syndromes; Lambert Eaton Myasthenic Syndrome; Lambert Eaton Syndrome; Lambert-Eaton Myasthenic-Myopathic Syndrome; Lambert-Eaton Myasthenic-Myopathic Syndromes; Lambert-Eaton Myopathic-Myasthenic Syndrome; Lambert-Eaton Myopathic-Myasthenic Syndromes; Myasthenic Myopathic Syndrome of Eaton Lambert; Myasthenic Myopathic Syndrome of Lambert Eaton; Myasthenic Syndrome, Eaton-Lambert; Myasthenic Syndrome, Lambert Eaton; Myopathic Myasthenic Syndrome of Eaton Lambert; Syndrome, Eaton-Lambert; Syndrome, Eaton-Lambert Myasthenic; Eaton-Lambert Syndrome; Myasthenic Syndrome, Lambert-Eaton
Networked: 457 relevant articles (28 outcomes, 20 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Paraneoplastic Cerebellar Degeneration
2. Neoplasms (Cancer)
3. Stiff-Person Syndrome (Stiff Man Syndrome)
4. Myasthenia Gravis
5. Dermatomyositis (Dermatopolymyositis)

Experts

1. Vincent, Angela: 10 articles (02/2015 - 03/2003)
2. Lang, Bethan: 9 articles (02/2015 - 05/2002)
3. Titulaer, Maarten J: 7 articles (07/2013 - 07/2006)
4. Verschuuren, Jan J G M: 6 articles (12/2012 - 07/2005)
5. Wirtz, Paul W: 6 articles (11/2010 - 07/2003)
6. Verschuuren, Jan J: 5 articles (07/2013 - 07/2003)
7. Takamori, Masaharu: 5 articles (12/2012 - 10/2004)
8. Honnorat, J: 5 articles (10/2011 - 01/2000)
9. Motomura, M: 5 articles (07/2009 - 08/2000)
10. Lang, B: 5 articles (03/2004 - 09/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Lambert-Eaton Myasthenic Syndrome:
1. Intravenous Immunoglobulins (IVIG)FDA Link
2. 3,4-diaminopyridineIBA
3. Immunoglobulins (Immunoglobulin)IBA
4. AntibodiesIBA
5. Guanidine (Guanidine Nitrate)FDA Link
6. Pyridostigmine Bromide (Pyridostigmine)FDA LinkGeneric
7. Calcium Channels (Calcium Channel)IBA
8. AutoantibodiesIBA
9. Immunoglobulin M (IgM)IBA
05/01/1997 - "In other controlled or open-label trials and case reports, IVIg produced improvement in several patients with the Lambert-Eaton myasthenic syndrome and myasthenia gravis but had a variable, mild, or unsubstantiated benefit in some patients with inclusion-body myositis, paraproteinemic IgM demyelinating polyneuropathy, certain intractable childhood epilepsies, polymyositis, multiple sclerosis, optic neuritis, and the stiff-man syndrome. "
05/01/1995 - "The neurological diseases with definite or putative immune pathogenesis include myasthenia gravis; Lambert-Eaton myasthenic syndrome; IgM monoclonal anti-myelin-associated glycoprotein-associated demyelinating polyneuropathy; Guillain-Barré syndrome; chronic inflammatory demyelinating polyneuropathy; multifocal motor neuropathy with or without GM1 antibodies; multiple sclerosis; inflammatory myopathies; stiff-man syndrome; autoimmune neuromyotonia; paraneoplastic neuronopathies and cerebellar degeneration; and neurological diseases associated with systemic autoimmune conditions, vasculitis, or viral infections. "
04/01/2007 - "Recommendations for use of IVIG were made for 14 conditions, including acute disseminated encephalomyelitis, chronic inflammatory demyelinating polyneuropathy, dermatomyositis, diabetic neuropathy, Guillain-Barré syndrome, Lambert-Eaton myasthenic syndrome, multifocal motor neuropathy, multiple sclerosis, myasthenia gravis, opsoclonus-myoclonus, pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections, polymyositis, Rasmussen's encephalitis, and stiff person syndrome; IVIG was not recommended for 8 conditions including adrenoleukodystrophy, amyotropic lateral sclerosis, autism, critical illness polyneuropathy, inclusion body, myositis, intractable childhood epilepsy, paraproteinemic neuropathy (IgM variant), and POEMS syndrome. "
11/01/1994 - "These are; ple21 in limbic encephalitis; PCD17, CDR62, CDR34, and CZF in paraneoplastic cerebellar degeneration; one of the anion transporters band 3 in chorea-acanthocytosis; visinin-like substance in cancer-associated retinopathy (CAR syndrome); myelin basic protein (MBP) and proteolipid protein (PLP) in acute disseminated encephalomyelitis; MBP, PLP and myelin-oligodendrocyte glycoprotein (MOG) in multiple sclerosis; glutamic acid decarboxylase in stiff-man syndrome; GM1 ganglioside in amyotrophic lateral sclerosis; peripheral nerve K+ channel in Isaacs syndrome; synaptotagmin in Lambert-Eaton syndrome; acetylcholine receptor in myasthenia gravis; GM1 ganglioside in Guillain-Barré syndrome; GQ1b ganglioside in Fisher syndrome; myelin-associated glycoprotein in IgM paraproteinemic neuropathy; HuD in paraneoplastic sensory neuropathy; and tRNA and HSP65 in polymyositis."
10. Immunoglobulin G (IgG)IBA

Therapies and Procedures

1. Drug Therapy (Chemotherapy)
2. Plasma Exchange
3. Immunomodulation
4. Radiotherapy
5. Aftercare (After-Treatment)