Marked hyperinsulinemia in a patient with myotonic dystrophy.

[Marked hyperinsulinemia in a patient with myotonic dystrophy].

Laparoscopic Cholecystectomy for Cholelithiasis in a Patient With Myotonic Dystrophy

Myotonic dystrophy (DM) is a rare autosomal dominant inherited neuromuscular disease involving several systems. The anesthetic method of choice remains uncertain. The risk of perioperative complications, particularly pulmonary and cardiac complications, in these patients is of major concern. We report on a 16-year-old female patient with DM type 1 undergoing laparoscopic cholecystectomy for sym...

Use of Sugammadex in a Patient With Myotonic Dystrophy

One of the challenges during the perioperative care of patients with myotonic dystrophy is the reversal of neuromuscular blocking agents. Agents that inhibit acetylcholinesterase, such as neostigmine, may precipitate myotonia, and are therefore relatively contraindicated. Sugammadex is a novel pharmacologic agent, which encapsulates rocuronium or vecuronium, thereby reversing their effect. We r...

Myotonic Dystrophy Type 1 with Syringomyelia in a Young Patient

Myotonic dystrophy type 1 (DM1) is the most common disease causing muscle weakness and atrophy in adults. The prevalence of DM1 in China is not clear. DM1 is an autosomal dominant genetic disorder associated with the cytosine‑thymine‑guanine (CTG) repeat expansion in 3'untranslated region in dystrophia myotonica‑protein kinase (DMPK) gene on chromosome 19q13.3. In DM1, CTG pathological repeat n...

Caesarean section for twins in a patient with myotonic dystrophy.

The management of a patient with myotonic dystrophy undergoing Caesarean section for delivery of twins in breech position is reported. Anaesthetic management must reflect the multi-system nature of the disease in addition to the implications of pregnancy. Known triggers of myotonic crisis (succinylcholine, shivering) must be avoided. Attention to respiratory reserve is necessary in view of the ...