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PubMed This is a summary of 16 peer-reviewed journal articles Updated
Anesthesiology

Anesthesia Risks & Emergency Precautions

At a Glance

Anesthesia carries life-threatening risks for people with Myotonic Dystrophy Type 1. The muscle relaxant succinylcholine must be strictly avoided as it triggers severe muscle contractions. Always carry a medical alert card and ensure your surgical team uses safe alternatives like Sugammadex.

For people living with Myotonic Dystrophy Type 1 (DM1), surgery and anesthesia are not “routine.” Even minor procedures requiring sedation carry significant risks that must be managed with extreme care [1][2]. Because your muscles, heart, and lungs all function differently due to DM1, your medical team must follow specific safety protocols to prevent life-threatening complications [3][4].

The Danger of a “Myotonic Crisis”

In DM1, certain triggers can cause a myotonic crisis—a sudden, severe, and generalized muscle contraction that can make it impossible to breathe or for a doctor to manage your airway [5][6].

  • Medication Triggers: The muscle relaxant succinylcholine is strictly contraindicated because it can trigger these severe contractions and cause a dangerous spike in potassium (hyperkalemia), leading to cardiac arrest [5][7].
  • Physical Triggers: Being cold (hypothermia), shivering, pain, or emotional stress can all trigger myotonia during or after surgery [8][1].
  • Mechanical Triggers: Even the electrical stimulation used by anesthesiologists to monitor muscle relaxation (nerve stimulation) can sometimes trigger a contraction [8].

Reversing Anesthesia Safely

If your surgery requires muscle relaxants, your team should use “non-depolarizing” agents (like rocuronium) rather than succinylcholine [9][10]. When the surgery is over, these medications must be “reversed” so you can breathe on your own again.

  • Why Sugammadex? Traditional reversal agents like neostigmine can sometimes make muscle weakness worse or even trigger myotonia in DM1 patients [11][12].
  • The Preferred Choice: Sugammadex is widely preferred for DM1 patients because it works quickly and safely to reverse muscle relaxation without interfering with the body’s other chemical systems [13][14].

Essential Safety Checklist

Share this information with your surgeon and anesthesiologist well before any procedure:

  • No Succinylcholine: Confirm that no “depolarizing” muscle relaxants will be used [5].
  • Avoid Neostigmine: Request Sugammadex for reversal if muscle relaxants are necessary [14][13].
  • Temperature Control: The operating room must be kept warm, and warming blankets should be used to prevent shivering [8].
  • Cardiac Monitoring: Continuous ECG monitoring is vital due to the high risk of heart rhythm issues (arrhythmias) during surgery [15][1].
  • Aspiration Precautions: Because DM1 can affect swallowing, there is a higher risk of inhaling stomach contents into the lungs (aspiration) during intubation [16].

Carry a Medical Emergency Card

Because many emergency room doctors and paramedics are unfamiliar with DM1, you should carry a Medical Emergency Card at all times [2]. This card should clearly list:

  1. Your diagnosis: Myotonic Dystrophy Type 1 (Steinert Disease).
  2. The warning: “NO SUCCINYLCHOLINE” in bold letters [5].
  3. Your risk of respiratory failure and the need for careful oxygen monitoring [4].
  4. Your risk of cardiac conduction blocks or arrhythmias [3].
  5. Contact information for your primary neurologist or DM1 specialist.

Common questions in this guide

Why is anesthesia dangerous for people with Myotonic Dystrophy Type 1?
Anesthesia carries significant risks for DM1 patients because certain medications, temperature changes, and stress can trigger a myotonic crisis, arrhythmias, or breathing problems. Routine procedures require specialized safety protocols and close cardiac monitoring.
What is a myotonic crisis during surgery?
A myotonic crisis is a sudden, severe, and generalized muscle contraction. During surgery, this can make your body rigid, making it impossible for you to breathe on your own or for the anesthesiologist to safely manage your airway.
Which anesthesia medications should be avoided if I have Steinert disease?
Patients with Myotonic Dystrophy Type 1 should never receive the muscle relaxant succinylcholine, as it can cause a dangerous spike in potassium and lead to cardiac arrest. Traditional reversal agents like neostigmine should also be avoided because they can worsen muscle weakness.
What is the safest anesthesia reversal agent for DM1?
Sugammadex is the preferred medication for reversing muscle relaxation in DM1 patients. It works quickly and safely to reverse the effects of anesthesia without interfering with other chemical systems in the body.
Why do I need to stay warm during surgery?
Being cold or shivering can easily trigger severe muscle contractions during or after a surgical procedure. Your medical team must use warming blankets and keep the operating room warm to prevent this from happening.
What should I put on my medical emergency card for DM1?
Your card should clearly state your Myotonic Dystrophy Type 1 (Steinert Disease) diagnosis and feature a bold warning against using succinylcholine. It should also note your risks for respiratory failure and heart rhythm issues, along with your specialist's contact information.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Are you aware of the specific anesthesia precautions required for patients with Myotonic Dystrophy Type 1?
  2. 2.How will you monitor my heart and breathing during and immediately after the procedure?
  3. 3.Can we ensure that sugammadex is available as the reversal agent if I need a muscle relaxant?
  4. 4.What steps will you take to keep me warm in the operating room to prevent myotonia from shivering?
  5. 5.How will you assess my breathing and swallow function before I am cleared to go home?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

Related questions

What to Put on a DM1 Medical Alert Card

References

References (16)
  1. 1

    Laparoscopic cholecystectomy under total intravenous anaesthesia in a patient with myotonic dystrophy type 1 (Steinert's disease) - a case report.

    Karras G, Nikouli E, Kiamiloglou B

    Folia medica 2022; (64(2)):333-336 doi:10.3897/folmed.64.e59905.

    PMID: 35851789
  2. 2

    [Medical emergency card for Steinert's disease: an unmet need].

    Rosado-Bartolomé A, Domínguez-González C

    Revista de neurologia 2023; (76(1)):15-19 doi:10.33588/rn.7601.2022380.

    PMID: 36544372
  3. 3

    Echocardiographic Features of Cardiac Involvement in Myotonic Dystrophy 1: Prevalence and Prognostic Value.

    Russo V, Capolongo A, Bottino R, et al.

    Journal of clinical medicine 2023; (12(5)) doi:10.3390/jcm12051947.

    PMID: 36902735
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    Myotonic dystrophy type 1 and pulmonary embolism: successful thrombus resolution with dabigatran etexilate therapy.

    Gallinoro E, Papa AA, Rago A, et al.

    Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology 2018; (37(3)):227-231.

    PMID: 30838353
  5. 5

    Complications After Dental Sedation: A Myotonic Mystery Case Report.

    Karamlou M, Asaria I, Barron J, et al.

    Anesthesia progress 2022; (69(4)):26-31 doi:10.2344/anpr-69-02-09.

    PMID: 36534775
  6. 6

    Pearls & Oy-sters: Severe Myotonic Crisis Resembling Malignant Hyperthermia.

    Wadhwani AR, Aggarwal A, Loscalzao S, et al.

    Neurology 2025; (104(8)):e213497 doi:10.1212/WNL.0000000000213497.

    PMID: 40127393
  7. 7

    Suxamethonium-Induced Hyperkalemia: A Short Review of Causes and Recommendations for Clinical Applications.

    Hovgaard HL, Juhl-Olsen P

    Critical care research and practice 2021; (2021()):6613118 doi:10.1155/2021/6613118.

    PMID: 33708444
  8. 8

    [Opioid-free general anesthesia and induced recovery from anesthesia in a patient with myotonic dystrophy type-1: a case report].

    Gurbuz H, Saracoglu KT

    Brazilian journal of anesthesiology (Elsevier) 2020; (70(6)):682-685 doi:10.1016/j.bjan.2020.07.004.

    PMID: 33190906
  9. 9

    Prevalence and associated factors of postoperative suxamethonium-induced myalgia in surgical patients at Debre Tabor Comprehensive Specialized Hospital Ethiopia: a cross-sectional study.

    Chekol B, Zurbachew N, Mekete G, et al.

    Scientific reports 2024; (14(1)):16552 doi:10.1038/s41598-024-65779-7.

    PMID: 39019942
  10. 10

    Comparison between succinylcholine and rocuronium as neuromuscular blocking agents for electroconvulsive therapy in a patient with pseudocholinesterase deficiency.

    Takazawa T, Suto T, Aihara M, et al.

    JA clinical reports 2015; (1(1)):7 doi:10.1186/s40981-015-0009-2.

    PMID: 29497639
  11. 11

    Myasthenia gravis and sugammadex: A case report and review of the literature.

    Dontukurthy S, Wisler C, Raman V, Tobias JD

    Saudi journal of anaesthesia 2020; (14(2)):244-248 doi:10.4103/sja.SJA_721_19.

    PMID: 32317886
  12. 12

    Sugammadex for Reversal of Neuromuscular Blockade: Uses and Limitations.

    Hawkins J, Khanna S, Argalious M

    Current pharmaceutical design 2019; (25(19)):2140-2148 doi:10.2174/1381612825666190704101145.

    PMID: 31272347
  13. 13

    Preparing for the unexpected: special considerations and complications after sugammadex administration.

    Iwasaki H, Renew JR, Kunisawa T, Brull SJ

    BMC anesthesiology 2017; (17(1)):140 doi:10.1186/s12871-017-0429-9.

    PMID: 29041919
  14. 14

    Optimizing Reversal of Neuromuscular Block in Older Adults: Sugammadex or Neostigmine.

    Togioka BM, Schenning KJ

    Drugs & aging 2022; (39(10)):749-761 doi:10.1007/s40266-022-00969-4.

    PMID: 35934764
  15. 15

    [Myotonic dystrophy type 1 - a rare cause of bradycardia in the young].

    Kataja Knight A, Tidehag L, Mattsson G, Magnusson P

    Lakartidningen 2020; (117()).

    PMID: 31961442
  16. 16

    Silent dysphagia in two patients with Steinert disease and recurrent respiratory exacerbations.

    Annunziata A, Valente T, Cauteruccio R, Fiorentino G

    Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology 2020; (39(3)):141-143 doi:10.36185/2532-1900-019.

    PMID: 33305171

This page provides general anesthesia safety guidelines for Myotonic Dystrophy Type 1 for educational purposes. Always consult with your anesthesiologist and surgical team to develop a personalized, safe surgical plan.

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