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Alterations in Muscle Tone lll

Alterations in Muscle Tone lll

Rigidity and myotonia are distinct abnormalities of muscle tone that affect resistance and relaxation during movement. Although both involve altered muscle contraction, they arise from different neurological and muscular mechanisms.CharacteristicsRigidity is characterized by uniform resistance to passive movement across the entire range, independent of speed, affecting flexors and extensors equally. It may appear as lead-pipe rigidity (smooth, constant resistance) or cogwheel rigidity...

Alterations in Muscle Tone ll

Alterations in Muscle Tone ll

Alterations in muscle tone are common manifestations of neurological disorders and reflect dysfunction within different nervous system regions. Spasticity, paratonia, and dystonia represent distinct forms of hypertonia, each with unique mechanisms, clinical features, and diagnostic importance.CharacteristicsSpasticity happens from upper motor neuron lesions and is characterized by velocity-dependent resistance to passive movement. Clinical features include:Exaggerated deep tendon reflexesClonus...

Disorders of the Skeletal Muscle

Disorders of the Skeletal Muscle

The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...

Satellite Stem Cells and Muscular Dystrophy

Satellite Stem Cells and Muscular Dystrophy

Satellite stem cells or myosatellite cells are quiescent stem cells that Alexander Mauro first identified in 1961. These cells are located between the sarcolemma, the plasma membrane of muscle fibers, and the basal lamina, the connective tissue sheath covering it. These mononucleated cells are activated in response to muscle injury, can transform into myoblasts, and may form or repair muscle fibers. Myosatellite cells can provide additional myonuclei for muscle regeneration or return to a...

Directly Acting Muscle Relaxants: Dantrolene and Botulinum Toxin

Directly Acting Muscle Relaxants: Dantrolene and Botulinum Toxin

Directly acting muscle relaxants like dantrolene and botulinum toxin (BoNT) have distinct mechanisms and applications. Dantrolene, a hydantoin derivative, acts on the ryanodine receptor (RYR1) in skeletal muscle cells. RYR1 are calcium channels present at the sarcoplasmic reticulum membrane. In response to excitation, they release calcium ions from the sarcoplasmic reticulum to the cytosol. Calcium promotes actin-myosin-mediated contraction of muscles.
The binding of dantrolene to the RYR1...

Myasthenia Gravis: Diagnostic Tests

Myasthenia Gravis: Diagnostic Tests

Myasthenia gravis is an autoimmune condition affecting neuromuscular transmission, causing generalized weakness in skeletal muscles. Initial diagnoses rely on patients' signs, symptoms, and medical history. The challenge lies in distinguishing myasthenia from other muscular dystrophies. An important diagnostic feature is the significant improvement of symptoms after administering anticholinesterase inhibitors.
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...

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Updated: Jun 19, 2026

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells

Published on: July 29, 2016

Myotonic dystrophy and pregnancy.

Zainab Ashraf Khan¹, Shahid Aziz Anwer Khan

¹West Cumberland Hospital, Whitehaven, United Kingdom.

JPMA. the Journal of the Pakistan Medical Association

|October 10, 2009

Summary

Myotonic dystrophy (DM) presents significant obstetric challenges. These cases highlight the critical need for prenatal diagnosis and genetic counseling in managing pregnant individuals with DM.

Area of Science:

Neurology
Obstetrics & Gynecology
Medical Genetics

Background:

Myotonic dystrophy (DM) is the most prevalent adult neuromuscular disorder.
DM affects multiple organ systems, posing complex management challenges during pregnancy.

Observation:

Case 1: A multiparous woman experienced polyhydramnios, abdominal pain, and postpartum hemorrhage attributed to DM.
Case 2: A pregnant woman with dichorionic, diamniotic twins underwent chorionic villus sampling, revealing one fetus with DM.

Findings:

Selective termination of the affected twin in Case 2 led to severe oligohydramnios and intrauterine fetal demise of the remaining twin.
Both cases underscore the significant obstetric risks associated with myotonic dystrophy.

Implications:

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Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells

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Isometric and Eccentric Force Generation Assessment of Skeletal Muscles Isolated from Murine Models of Muscular Dystrophies

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Implantation of Osmotic Pumps and Induction of Stress to Establish a Symptomatic, Pharmacological Mouse Model for DYT/PARK-ATP1A3 Dystonia

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Prenatal diagnosis and genetic counseling are crucial for managing pregnancies in women with myotonic dystrophy.
A multidisciplinary team approach is essential for optimizing outcomes in these high-risk pregnancies.