Hospital Practice
ISSN: 2154-8331 (Print) 2377-1003 (Online) Journal homepage: http://www.tandfonline.com/loi/ihop20
The Lambert-Eaton Syndrome R. Glenn Smith & Stanley H. Appel To cite this article: R. Glenn Smith & Stanley H. Appel (1992) The Lambert-Eaton Syndrome, Hospital Practice, 27:12, 101-116, DOI: 10.1080/21548331.1992.11705539 To link to this article: http://dx.doi.org/10.1080/21548331.1992.11705539
Published online: 17 May 2016.
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Date: 16 June 2016, At: 23:41
IPHV§~OlOGV ~N MIED~C~NIE
The Lambert-Eaton Syndrome and STANLEY H. Baylor College of Medicine
Downloaded by [RMIT University Library] at 23:41 16 June 2016
R. GLENN SMITH
APPEL
This rare myasthenic disorder is the first neurologic disease found to be caused by antibodies against an ion channel complex. It presents an unusual opportunity to clarify the pathobiology of so-called receptor diseases, as well as to gain better understanding of the normal biology of acetylcholine release and its effect on neuromuscular junctions.
The Lambert-Eaton myasthenic syndrome is a disorder of the neuromuscular system often associated with small cell carcinoma of the lung and characterized by progressive weakness of the arms and legs, as well as by autonomic dysfunction. Experimental and clinical studies have suggested that a decrease in the release of acetylcholine at the neuromuscular junction is responsible for impaired neuromuscular transmission leading to clinical weakness. The pathologic process is thought to be triggered by autoimmune mechanisms in which antibodies against the motor nerve terminal, and more specifically against voltage-gated calcium channels or associated proteins, alter the membrane morphology and the number of calcium channels. Lambert-Eaton syndrome is the first example of a specific neurologic syndrome due to antibodies against an ion channel complex, and thus may provide new insights into other neurologic disorders of unknown etiology.
Dr. Smith is Assistant Professor and Dr. Appel is Professor and Chairman, Department of Neurology, Baylor College of Medicine, Houston. This article is the sixth in a series on neuromuscular transmission disorders.
Clinical Features The entity was first described as a separate syndrome by Edward Lambert and Lee Eaton at the Mayo Clinic in 195 7. In large part because of their work, we now know that those affected are most typically older men, and that the median onset of symptoms is at age 60. Up to 50% of patients have associated small cell lung carcinoma. An additional 10% to 15% have other malignancies (primarily renal cell carcinoma or hematologic tumors). The paraneoplastic variant of the syndrome may appear years before detection of malignancy. It usually progresses rapidly and may be accompanied by other paraneoplastic syndromes, including subacute cerebellar degeneration and sensory or sensorimotor polyneuropathy. Patients without associated malignancy are typically much younger at presentation and have a more protracted and variable course. The nonparaneoplastic form of the syndrome is often associated with a number of known or suspected autoimmune diseases, including Graves' disease, Hashimoto's thyroiditis, Sjogren syndrome, Beh
ISSN: 2154-8331 (Print) 2377-1003 (Online) Journal homepage: http://www.tandfonline.com/loi/ihop20
The Lambert-Eaton Syndrome R. Glenn Smith & Stanley H. Appel To cite this article: R. Glenn Smith & Stanley H. Appel (1992) The Lambert-Eaton Syndrome, Hospital Practice, 27:12, 101-116, DOI: 10.1080/21548331.1992.11705539 To link to this article: http://dx.doi.org/10.1080/21548331.1992.11705539
Published online: 17 May 2016.
Submit your article to this journal
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ihop20 Download by: [RMIT University Library]
Date: 16 June 2016, At: 23:41
IPHV§~OlOGV ~N MIED~C~NIE
The Lambert-Eaton Syndrome and STANLEY H. Baylor College of Medicine
Downloaded by [RMIT University Library] at 23:41 16 June 2016
R. GLENN SMITH
APPEL
This rare myasthenic disorder is the first neurologic disease found to be caused by antibodies against an ion channel complex. It presents an unusual opportunity to clarify the pathobiology of so-called receptor diseases, as well as to gain better understanding of the normal biology of acetylcholine release and its effect on neuromuscular junctions.
The Lambert-Eaton myasthenic syndrome is a disorder of the neuromuscular system often associated with small cell carcinoma of the lung and characterized by progressive weakness of the arms and legs, as well as by autonomic dysfunction. Experimental and clinical studies have suggested that a decrease in the release of acetylcholine at the neuromuscular junction is responsible for impaired neuromuscular transmission leading to clinical weakness. The pathologic process is thought to be triggered by autoimmune mechanisms in which antibodies against the motor nerve terminal, and more specifically against voltage-gated calcium channels or associated proteins, alter the membrane morphology and the number of calcium channels. Lambert-Eaton syndrome is the first example of a specific neurologic syndrome due to antibodies against an ion channel complex, and thus may provide new insights into other neurologic disorders of unknown etiology.
Dr. Smith is Assistant Professor and Dr. Appel is Professor and Chairman, Department of Neurology, Baylor College of Medicine, Houston. This article is the sixth in a series on neuromuscular transmission disorders.
Clinical Features The entity was first described as a separate syndrome by Edward Lambert and Lee Eaton at the Mayo Clinic in 195 7. In large part because of their work, we now know that those affected are most typically older men, and that the median onset of symptoms is at age 60. Up to 50% of patients have associated small cell lung carcinoma. An additional 10% to 15% have other malignancies (primarily renal cell carcinoma or hematologic tumors). The paraneoplastic variant of the syndrome may appear years before detection of malignancy. It usually progresses rapidly and may be accompanied by other paraneoplastic syndromes, including subacute cerebellar degeneration and sensory or sensorimotor polyneuropathy. Patients without associated malignancy are typically much younger at presentation and have a more protracted and variable course. The nonparaneoplastic form of the syndrome is often associated with a number of known or suspected autoimmune diseases, including Graves' disease, Hashimoto's thyroiditis, Sjogren syndrome, Beh
