It is known that in the early stages of desminopathy there is no spontaneous muscle activity. In the late stages of desminopathy, spontaneous muscle activity appears. What are the causes of spontaneous muscle activity in the late stages of desminopathy?
pathological changes in the muscle and motor units as
1)Axonal Damage and Motor Unit Instability
2)Inflammatory or Fibrotic Changes
3)Abnormal Ion Channel Activity which may develop abnormal membrane properties
4)Progressive Muscle Fiber Degeneration by mutations in the desmin gene
Leading to degenerative like changes
5)Compensatory Reinnervation Efforts
creating large motor units that are electrically unstable and prone to spontaneous activity.
Dear Hager Zayed Aljafarawi, thank you very much for your answer!
Still, is spontaneous activity in muscles in the late stages of desminopathy "good" or "bad"?
Many thanks to the respected Pavel Franzevich Zabrodsky and Sodiq Adetunji Adekola for recommending the question!
There are multiple explanations for spontaneous muscle activity, including a defect in the gene encoding the protein desmin and the production of multiple forms of it, which will affect the muscle fiber and cause an increase in excitability and electrical potential. It also causes the collapse of sarcomeres and an imbalance in the number and function of ion channels, and this can lead to spontaneous depolarization. Likewise, an imbalance in desmin can cause a disturbance in the functioning of mitochondria and the calcium functioning system.
In the late stages of desminopathy, spontaneous muscle activity appears due to progressive muscle and nerve involvement. This activity is a result of how the body responds to ongoing muscle fiber damage.
Key Causes:
in the late stages of desminopathy typically on EMG reflects ongoing muscle fiber degeneration and denervation
So generally considered bad sign indicates disease progression plus muscle instability and a worsening of neuromuscular integrity
Thanks Dr Viacheslav Pauls
Dear Rashied Rashied, Kazi Akib Islam and Hager Zayed Aljafarawi, thank you very much for your answers!
Thank you very much to dear Hager Zayed Aljafarawi for recommending the question and answer!
La actividad muscular espontánea en las últimas etapas de la desminopatía puede ser atribuida a varias causas, incluyendo:
Estos factores contribuyen a la presentación clínica de la desminopatía, que se caracteriza por debilidad muscular y otros síntomas neuromusculares.
Dear Cecilia Guiliana Solano Garcia, thank you very much for your detailed answer!
And can toxins also be the cause of spontaneous muscle activity in the late stages of desminopathy?
In the late stages of desminopathy, spontaneous muscle activity (like cramps or twitching) happens because the damaged desmin protein disrupts muscle fiber structure. This leads to unstable muscle membranes, abnormal electrical signals, and involuntary contractions. Nerve dysfunction and muscle degeneration worsen these symptoms over time.
- Badges
- Science topic
- Similar topics
- Biological Science
- Botany
- Pharmaceutical Botany
- Plant Extracts