Related Experiment Video
Updated: Jun 10, 2026
10:30
Multi-exon Skipping Using Cocktail Antisense Oligonucleotides in the Canine X-linked Muscular Dystrophy
Published on: May 24, 2016
Myotonic dystrophy: an anaesthetic dilemma
N Gupta1, K N Saxena, Asish Kumar Panda
1Assistant Prof.,Department of Anaesthesia & Intensive Care, PGIMER and associated RML hospital.
Indian Journal of Anaesthesia
|July 20, 2010
Summary
Myotonic dystrophy (DM) presents significant challenges for anesthesiologists, particularly in patients with cardiac complications. Early diagnosis and meticulous perioperative care are crucial for managing these complex cases.
Area of Science:
- Anesthesiology
- Genetics
- Cardiology
Background:
- Myotonic dystrophy (DM) is a progressive, inherited multisystemic disorder with variable clinical presentation.
- The case involves a 32-year-old female with a history of dilated cardiomyopathy (DCMP) undergoing exploratory laparotomy.
- Preoperative assessment revealed significant cardiac dysfunction, including severe left ventricular dysfunction and pulmonary artery hypertension.
Purpose of the Study:
- To highlight the anesthetic management challenges in a patient with undiagnosed myotonic dystrophy and significant cardiac comorbidities.
- To emphasize the importance of considering rare genetic disorders in patients with unexplained postoperative complications.
- To underscore the necessity of multidisciplinary collaboration for optimal patient outcomes.
Main Methods:
- Case report of a 32-year-old female with adenexal mass and DCMP.
- General anesthesia combined with epidural anesthesia was administered.
- Postoperative respiratory distress prompted neurological consultation, leading to diagnosis via muscle biopsy and genetic analysis.
- Prolonged mechanical ventilation was required due to complications.
Main Results:
- The patient developed severe respiratory distress and hypotension on postoperative day 2, indicative of Type 1 respiratory failure.
- Despite intensive care, the patient required prolonged mechanical ventilation for 390 days.
- Myotonic dystrophy was confirmed postoperatively through muscle biopsy and genetic testing.
Conclusions:
- Patients with myotonic dystrophy pose significant anesthetic challenges due to potential multisystemic involvement, especially cardiac and respiratory complications.
- Optimized preoperative medical treatment and careful perioperative planning are essential for managing patients with DM.
- Prompt neurological referral and diagnostic workup are critical when unexplained postoperative respiratory failure occurs in patients with underlying cardiac conditions.
Related Concept Videos
01:31Skeletal Muscle Relaxants: Therapeutic Uses
Skeletal muscle relaxants are used to relax muscle tone and alleviate painful muscle contractions. However, the choice of skeletal muscle relaxants depends on the duration of the surgical procedure in order to minimize potential side effects. Skeletal muscle relaxants like neuromuscular blocking agents [NMBAs] are commonly employed as adjuvants alongside general anesthetics in clinical settings. NMBAs are also used to maintain controlled ventilation during surgery of the larynx or pharynx as...
01:21Skeletal Muscle Relaxants: Adverse Effects
Skeletal muscle relaxants are widely used for muscle paralysis and relieving pain following any muscle injury or stiffness. However, depending on the drug type, they can have adverse effects that range from mild to severe. Usually, nondepolarizing neuromuscular blockers have minimal side effects. For example, drugs like d-tubocurarine, cisatracurium, and rocuronium cause hypotension, whereas drugs like baclofen, when stopped abruptly, can lead to the recurrence of spastic conditions.
Unlike...
Unlike...
01:27Nondepolarizing (Competitive) Neuromuscular Blockers: Pharmacological Actions
Nondepolarizing neuromuscular blockers prevent the membrane depolarization of muscle cells and inhibit muscle contraction. These are usually administered with anesthetics to achieve complete muscle relaxation. Upon administration, these drugs first block the small, rapidly contracting muscles of the face and hands, followed by the larger muscles of the trunk and the intercostal muscles. The diaphragm is the last muscle to be affected.
Although all competitive neuromuscular blockers are designed...
Although all competitive neuromuscular blockers are designed...
01:26Directly 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...
The binding of dantrolene to the RYR1...
01:11Alterations 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...
01:19Depolarizing Blockers: Pharmocokinetics
Depolarizing blockers are administered through intravenous injection. Succinylcholine is the most common choice of depolarizing blockers in emergency clinical practices. Although they have a rapid onset, they readily diffuse away from the motor end plate into the extracellular fluid. They are metabolized by enzymes such as liver butyrylcholinesterase and plasma pseudocholinesterases. This produces a short duration of action, typically 5-10 minutes long, unlike nondepolarizing blockers, which...