[SMC] Electrodiagnostic Evaluation Of Neuromuscular Junction Disorder

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[SMC] Electrodiagnostic Evaluation Of Neuromuscular Junction Disorder

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2021 Dec 19. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–.

ABSTRACT

Neuromuscular junction disorders are a group of conditions that cause muscle weakness. Their etiology can be autoimmune, congenital, metabolic, or toxic mediated. The three most common neuromuscular junction disorders are Myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS), and botulism. The primary pathology is impaired neurotransmission at the interface (synapse) between the nerve ending and the skeletal muscle fiber. In Myasthenia gravis, pathology occurs at the postsynaptic membrane. In Lambert-Eaton myasthenic syndrome and botulism, the presynaptic membrane is affected.

Neuromuscular Junction disorders patients present with complaints of muscle fatigue and weakness that fluctuate with episodes of worsening after activity. The sensory system is not affected, as sensory nerves do not have a neuromuscular junction. Patients present with proximal greater than distal muscle weakness. It is common to receive complaints related to bulbar or extraocular muscle weakness as well. Myasthenia gravis patients frequently present with bulbar weakness and limb weakness. It is important to investigate if the patient has a history of thymoma.

LEMS patients less commonly present with bulbar weakness but have diffuse proximal limb weakness. LEMS has a strong correlation with small cell carcinoma. Botulism is a rare condition caused by a toxin produced by clostridium botulinum. In the United States majority of the cases are seen in infants.

PMID:32965973 | Bookshelf:NBK562302


Source: https://pubmed.ncbi.nlm.nih.gov/3296597 ... 0&v=2.17.5
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Bonne lecture...
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