A Diagnostic and Management Dilemma: Combined Paraneoplastic Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome Presenting as Acute Respiratory Failure

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Abstract

Background:

Neuromuscular junction disorders are usually categorized as either presynaptic or postsynaptic. The most frequently encountered disorder of the postsynaptic neuromuscular junction is acquired myasthenia gravis. Lambert-Eaton myasthenic syndrome is a well-known prototype of the presynaptic autoimmune disorders of neuromuscular transmission. These major disorders of neuromuscular transmission are relatively common and distinctly recognized, but co-occurrence of these disorders (overlap myasthenic syndrome) is rare and has so far attracted little attention.

Review Summary:

This report describes a patient with acquired myasthenia gravis and immunologic coexistence of Lambert-Eaton myasthenic syndrome (overlap myasthenic syndrome) in association with abdominal/uterine leiomyosarcoma. The patient presented with acute respiratory failure, making identification and management of her illness challenging. A general overview of the complexities associated with overlap between myasthenia gravis and Lambert-Eaton myasthenic syndrome is provided and this patient's complicated clinical course and response to therapy are discussed.

Conclusion:

To our knowledge, this is the first report of overlap myasthenic syndrome in conjunction with abdominal leiomyosarcoma. The immunologic coexistence of acquired myasthenia gravis and Lambert-Eaton myasthenic syndrome in a patient with a malignant smooth-muscle tumor is intriguing and suggests that a common paraneoplastic process targeting 2 different onconeural antigens was the underlying pathogenic mechanism in this patient.

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