A diagnostic and management dilemma: combined paraneoplastic myasthenia gravis and Lambert-Eaton myasthenic syndrome presenting as acute respiratory failure.

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.

Myokymia in obstetrically related brachial plexopathy.

Myokymic discharges are spontaneous bursts of semirhythmic potentials that are sometimes correlated with rippling movements of skin and muscle. They have been reported in limb muscles in patients with Guillain-Barré syndrome, spinal stenosis, nerve root and nerve compression, and envenomations. They commonly occur with radiation induced plexopathies (approximately 60% of patients), but have not been reported in obstetrically related brachial plexopathies. We report 2 instances of myokymia in children with obstetric brachial plexus palsies. Each child was studied twice, and it was only at the later study, when the child was 10 or 11 months of age, that these potentials were noted. This could represent ongoing recovery from lesions incurred at birth or developmental changes. The final common pathway of all causes of myokymia could be to generate axonal membrane hyperexcitability.