Myasthenia Gravis, Myasthenic Syndrome, and Related Disorders

2013 ◽  
pp. 809-840 ◽  
Author(s):  
Jun Kimura
Keyword(s):  
Myasthenia Gravis ◽  
Myasthenic Syndrome

scholarly journals Presynaptic Paraneoplastic Disorders of the Neuromuscular Junction: An Update

2021 ◽  
Vol 11 (8) ◽  
pp. 1035
Author(s):  
Maria Pia Giannoccaro ◽  
Patrizia Avoni ◽  
Rocco Liguori
Keyword(s):  
Potassium Channels ◽  
Neuromuscular Junction ◽  
Calcium Channels ◽  
Myasthenia Gravis ◽  
Neuromuscular Transmission ◽  
Voltage Gated ◽  
Voltage Gated Calcium Channels ◽  
Immune Mediated ◽  
Recent Developments ◽  
Myasthenic Syndrome

The neuromuscular junction (NMJ) is the target of a variety of immune-mediated disorders, usually classified as presynaptic and postsynaptic, according to the site of the antigenic target and consequently of the neuromuscular transmission alteration. Although less common than the classical autoimmune postsynaptic myasthenia gravis, presynaptic disorders are important to recognize due to the frequent association with cancer. Lambert Eaton myasthenic syndrome is due to a presynaptic failure to release acetylcholine, caused by antibodies to the presynaptic voltage-gated calcium channels. Acquired neuromyotonia is a condition characterized by nerve hyperexcitability often due to the presence of antibodies against proteins associated with voltage-gated potassium channels. This review will focus on the recent developments in the autoimmune presynaptic disorders of the NMJ.

Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome

2014 ◽  
Vol 20 ◽  
pp. 1413-1425 ◽  
Author(s):  
Donald B. Sanders ◽  
Jeffrey T. Guptill
Keyword(s):  
Myasthenia Gravis ◽  
Myasthenic Syndrome

Electrodiagnostic Findings in Neuromuscular Disorders

2018 ◽  
Author(s):  
Bashar Katirji
Keyword(s):  
Neuromuscular Junction ◽  
Peripheral Nerve ◽  
Myasthenia Gravis ◽  
Nerve Conduction ◽  
Neuromuscular Disorders ◽  
Nerve Conduction Studies ◽  
Anterior Horn Cell ◽  
Peripheral Neuropathies ◽  
Peripheral Nerve Lesions ◽  
Myasthenic Syndrome

Neuromuscular disorders are often classified into four major categories: anterior horn cell disorders, peripheral neuropathies, neuromuscular junction disorders and myopathies. This chapter discusses the electrodiagnostic and clinical EMG findings in these various neuromuscular disorders. Peripheral neuropathies are subdivided into focal mononeuropathies, radiculopathies, plexopathies and generalized peripheral polyneuropathies. Focal peripheral nerve lesions and generalized peripheral polyneuropathies may be axonal or demyelinating, and manifest quite distinctly on nerve conduction studies. Neuromuscular junction disorders may be presynaptic, as seen with the Lambert-Eaton myasthenic syndrome, or postsynaptic, as seen with myasthenia gravis.

A 46-Year-Old Man with Double Vision and Proximal Leg Weakness

2016 ◽  
Author(s):  
Jeffrey A. Cohen ◽  
Justin J. Mowchun ◽  
Victoria H. Lawson ◽  
Nathaniel M. Robbins
Keyword(s):  
Neuromuscular Junction ◽  
Myasthenia Gravis ◽  
Clinical Picture ◽  
Double Vision ◽  
Junction Conditions ◽  
Antibody Testing ◽  
Tumor Surveillance ◽  
Difficult Condition ◽  
Leg Weakness ◽  
Myasthenic Syndrome

Lambert-Eaton myasthenic syndrome (LEMS) can be a difficult condition to diagnose. In this chapter, the clinical picture and characteristics are discussed. The pathophysiology is also reviewed. Features to distinguish LEMS from more common neuromuscular junction conditions such as myasthenia gravis are reviewed. We review features that distinguish between idiopathic and paraneoplastic forms, and we discuss the importance of tumor surveillance. Antibody testing and the correct electrodiagnostic strategy are presented. Treatment of LEMS is outlined.Lambert-Eaton syndrome (LES) is a difficult condition to diagnose. The unique clinical picture and its characteristics are discussed. This is especially true in the patient without a diagnois of cancer. Antibody testing and the correct electrodiagnostic strategy are presented. Treatment of LES is outlined.

Ophthalmoplegia

2015 ◽  
Author(s):  
Aziz Shaibani
Keyword(s):  
Family History ◽  
Muscular Dystrophy ◽  
Myasthenia Gravis ◽  
Eye Movement ◽  
Congenital Myasthenic Syndrome ◽  
Oculopharyngeal Muscular Dystrophy ◽  
Detailed Family History ◽  
Myasthenic Syndrome ◽  
The Brain

Ophthalmoplegia is usually chronic and therefore diplopia is not a feature. There is enough time for the brain to suppress one image. It is amazing how much impairment of eye movement has to occur before the patient becomes concerned or considers it as abnormal. Neglected myasthenia gravis may be confused with mitochondrial ophthalmoplegia or oculopharyngeal muscular dystrophy or even congenital myasthenic syndrome. Central cause of ophthalmoplegia should be ruled out first by performing doll’s eye movement. Detailed family history looking in particular for ptosis, ophthalmoplegia, and dysphagia is diagnostically very useful. Non neurological causes of ophthalmoplegia such as severe exophthalmus, and retroorbital pathology should be considered.

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