Myasthenic Syndrome

undefined: Myasthenia literally means muscle weakness. Myasthenic syndrome is the name for a set of symptoms characteristic of muscle activity disorders caused by damage to the neuromuscular disc. This includes autoimmune diseases - Myasthenia gravis, Lambert-Eaton syndrome (including paraneoplastic syndromes, e.g. in small cell carcinoma), and also botulinum toxin, which affects transmission on the neuromuscular disc.

Clinical picture
A typical manifestation is muscle fatigue and weakness, which is particularly pronounced after exertion. It can also affect respiratory muscles and phonation. Sensory impairment and pain are not manifested. During the examination, the tone and reflexes are normal, the muscles are not atrophic.

Damage to the respiratory muscles can lead to a life-threatening myasthenic crisis accompanied by respiratory failure.

Symptoms are at their peak in the evening. Transient paresis may occur after repeated movements, which disappear after a certain period of rest.

Diagnostics

 * EMG;
 * antibodies to ACh;
 * antiMuSk antibodies;
 * muscle neck test, where the patient does not keep the chin at the sternum;
 * a test of muscle strength, when the patient opens the HK and we count the time it takes for the limbs to drop.

Symptomatic

 * Cholinesterase inhibitors: pyridostigmine, neostigmine
 * regimen measures: do not exert yourself, ventilation support
 * do not use ATBs that reduce MG, or benzodiazepines

Immunomodulatory

 * corticosteroids: prednisone, prednisolone, methylprednisolone (necessary to think about NU - steroid diabetes, ulcer disease, osteoporosis, depression, hypertension, Na retention, Cushing's syndrome)
 * immunosuppressants: azatiprine (most used), cyclosporine A, mycophenolate mofetil, tacrolimus, cyclophosphamide, methotrexate, rituximab,

Intravenous immunoglobulins
Drug of choice in myasthenic crisis. Improvement within a week.

Therapeutic plasmapheresis
The effect lasts 4-10 weeks. The disadvantage is venous access and non-selective removal of blood proteins. A variant of Plasmapheresis can be immunoadsorption, when Ig is removed, or only some class of Ig.

Thymectomy
Increases the likelihood of remission or improvement. It also reduces overall mortality.

Related articles

 * Myopathic syndrome
 * Myasthenia gravis
 * Neuromuscular diseases
 * Neuromuscular diseases (pediatrics)

Reference

 * 1) VOKURKA, Martin a Jan HUGO, et al. Velký lékařský slovník. 6. vydání. Praha : Maxdorf, 2006.  ISBN 8073451050.
 * 2) ↑ Skočit nahoru k:a b JECH, Robert. Základy obecné neurologie [přednáška k předmětu Neurologie, obor Všeobecné lékařství, 1. LF UK v Praze]. Praha. 14.05.2012. Dostupné také z < https://www.neuro.lf1.cuni.cz/vyuka/soubory/5r/Obecna_neurol_CZ.pdf >.