Myotonia and paramyotonia - clinical differentiation

Myotonia and paramyotonia - clinical differentiation:
The myotonic disorders are a heterogeneous group of genetically determined diseases that are unified by the presence of myotonia, which is defined as failure of muscle relaxation after activation. The presentation of these disorders can range from asymptomatic electrical myotonia, as seen in some forms of myotonia congenita (MC), to severe disability with muscle weakness, cardiac conduction defects, and other systemic features as in myotonic dystrophy type I (DM1).
The myotonic disorders are a group of rare, genetically heterogeneous syndromes presenting with clinical and/or electrical myotonia. Clinical myotonia is characterized by the failure of muscle relaxation after activation.
Clinical myotonia manifests with painless muscle stiffness, although some forms can be associated with pain. The typical location of stiffness varies depending on the underlying disorder but commonly seen in the eyelids, mouth, hands, and proximal legs.
Common triggers include cold, stress and exercise, and symptoms can worsen during pregnancy and menstruation. Most demonstrate a “warm-up” phenomenon, where myotonia improves with repeated action.
In contrast, paradoxical myotonia or paramyotonia worsens with repeated use. Some forms of myotonia are also associated with diffuse muscle hypertrophy.
Myotonia can be brought out by asking the patient to repeatedly grip and relax their hand or open and close their eyes. Alternatively, direct percussion of a muscle can achieve the same effect; including tapping the thenar eminence, forearm extensors, or even tongue.
Ref:
Hahn C, Salajegheh MK. Myotonic disorders: A review article. Iran J Neurol. 2016;15(1):46-53.

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[@dr.anooparyan007]

Very Helpful video thank you sir

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Thanks

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Welcome

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What are the treatments

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The clinical experience of treating myotonia has involved a variety of medications, including phenytoin, mexiletine, procainamide, propafenone, flecainide, and carbamazepine, all of which are sodium channel blockers and share the potential to increase weakness while reducing myotonia. (Uptodate ,2024) please contact your doctor for further details