Skip to main content
Top
Published in: Canadian Journal of Anesthesia/Journal canadien d'anesthésie 3/2010

01-03-2010 | Case Reports/Case Series

Anesthesia and myotonic dystrophy type 2: a case series

Authors: Toby N. Weingarten, MD, Ryan E. Hofer, MMS, Margherita Milone, MD, PhD, Juraj Sprung, MD, PhD

Published in: Canadian Journal of Anesthesia/Journal canadien d'anesthésie | Issue 3/2010

Login to get access

Abstract

Background

Myotonic dystrophy type 2 (DM2) is a genetically distinct disorder that shares some phenotypical features of myotonic dystrophy type 1 (DM1). However, anesthetic management of patients with DM2 has not been described. The purpose of this study is to report the anesthetic management of a series of patients with DM2 and to describe their response to anesthesia.

Methods

We performed a computerized search of the Mayo Clinic medical records database looking for patients with DM2 who underwent general anesthesia. The medical records were reviewed for anesthetic technique, medications used, and postoperative complications.

Results

We identified 19 patients with DM2 who underwent 39 general anesthetics, 17 monitored anesthetic care cases, and two regional anesthetics. The patients exhibited normal responses to succinylcholine, nondepolarizing neuromuscular blockers, neostigmine, induction agents, and volatile anesthetics. Serious postoperative complications related to DM2 did not occur.

Conclusion

In our series, patients with DM2 tolerated commonly used anesthetics without obvious complications, and they exhibited normal responses to muscle relaxants. These observations suggest that these medications may be used safely in patients with DM2.
Literature
2.
go back to reference Day JW, Ricker K, Jacobsen JF, et al. Myotonic dystrophy type 2: molecular, diagnostic and clinical spectrum. Neurology 2003; 60: 657–64.CrossRefPubMed Day JW, Ricker K, Jacobsen JF, et al. Myotonic dystrophy type 2: molecular, diagnostic and clinical spectrum. Neurology 2003; 60: 657–64.CrossRefPubMed
3.
go back to reference Buzello W, Krieg N, Schlickewei A. Hazards of neostigmine in patients with neuromuscular disorders. Report of two cases. Br J Anaesth 1982; 54: 529–34.CrossRefPubMed Buzello W, Krieg N, Schlickewei A. Hazards of neostigmine in patients with neuromuscular disorders. Report of two cases. Br J Anaesth 1982; 54: 529–34.CrossRefPubMed
4.
go back to reference Ranum LP, Rasmussen PF, Benzow KA, Koob MD, Day JW. Genetic mapping of a second myotonic dystrophy locus. Nat Genet 1998; 19: 196–8.CrossRefPubMed Ranum LP, Rasmussen PF, Benzow KA, Koob MD, Day JW. Genetic mapping of a second myotonic dystrophy locus. Nat Genet 1998; 19: 196–8.CrossRefPubMed
5.
6.
go back to reference Udd B, Krahe R, Wallgren-Pettersson C, Falck B, Kalimo H. Proximal myotonic dystrophy–a family with autosomal dominant muscular dystrophy, cataracts, hearing loss and hypogonadism: heterogeneity of proximal myotonic syndromes? Neuromuscul Disord 1997; 7: 217–28.CrossRefPubMed Udd B, Krahe R, Wallgren-Pettersson C, Falck B, Kalimo H. Proximal myotonic dystrophy–a family with autosomal dominant muscular dystrophy, cataracts, hearing loss and hypogonadism: heterogeneity of proximal myotonic syndromes? Neuromuscul Disord 1997; 7: 217–28.CrossRefPubMed
8.
go back to reference Orndahl G. Myotonic human musculature: stimulation with depolarizing agents. I. Mechanical registration of the effects of acetylcholine and choline. Acta Med Scand 1962; 172: 739–51.PubMed Orndahl G. Myotonic human musculature: stimulation with depolarizing agents. I. Mechanical registration of the effects of acetylcholine and choline. Acta Med Scand 1962; 172: 739–51.PubMed
9.
go back to reference Orndahl G. Myotonic human musculature: stimulation with depolarizing agents. II. A clinico-pharmacological study. Acta Med Scand 1962; 172: 753–65.PubMed Orndahl G. Myotonic human musculature: stimulation with depolarizing agents. II. A clinico-pharmacological study. Acta Med Scand 1962; 172: 753–65.PubMed
10.
go back to reference Orndahl G, Stenberg K. Myotonic human musculature: stimulation with depolarizing agents. Mechanical registration of the effects of succinyldicholine, succinylmonocholine and decamethonium. Acta Med Scand Suppl 1962; 389: 3–29.PubMed Orndahl G, Stenberg K. Myotonic human musculature: stimulation with depolarizing agents. Mechanical registration of the effects of succinyldicholine, succinylmonocholine and decamethonium. Acta Med Scand Suppl 1962; 389: 3–29.PubMed
11.
go back to reference Dundee JW. Thiopentone in dystrophia myotonia. Curr Res Anesth Analg 1952; 31: 257–62.PubMed Dundee JW. Thiopentone in dystrophia myotonia. Curr Res Anesth Analg 1952; 31: 257–62.PubMed
12.
go back to reference Speedy H. Exaggerated physiological responses to propofol in myotonic dystrophy. Br J Anaesth 1990; 64: 110–2.CrossRefPubMed Speedy H. Exaggerated physiological responses to propofol in myotonic dystrophy. Br J Anaesth 1990; 64: 110–2.CrossRefPubMed
13.
go back to reference White DA, Smyth DG. Exaggerated physiological responses to propofol in myotonic dystrophy. Br J Anaesth 1990; 64: 758–9.CrossRefPubMed White DA, Smyth DG. Exaggerated physiological responses to propofol in myotonic dystrophy. Br J Anaesth 1990; 64: 758–9.CrossRefPubMed
14.
go back to reference Aldridge LM. Anaesthetic problems in myotonic dystrophy. A case report and review of the Aberdeen experience comprising 48 general anaesthetics in a further 16 patients. Br J Anaesth 1985; 57: 1119–30.CrossRefPubMed Aldridge LM. Anaesthetic problems in myotonic dystrophy. A case report and review of the Aberdeen experience comprising 48 general anaesthetics in a further 16 patients. Br J Anaesth 1985; 57: 1119–30.CrossRefPubMed
15.
go back to reference Liquori CL, Ricker K, Moseley ML, et al. Myotonic dystrophy type 2 caused by a CCTG expansion in intron 1 of ZNF9. Science 2001; 293: 864–7.CrossRefPubMed Liquori CL, Ricker K, Moseley ML, et al. Myotonic dystrophy type 2 caused by a CCTG expansion in intron 1 of ZNF9. Science 2001; 293: 864–7.CrossRefPubMed
16.
go back to reference Savkur RS, Philips AV, Cooper TA, et al. Insulin receptor splicing alteration in myotonic dystrophy type 2. Am J Hum Genet 2004; 74: 1309–13.CrossRefPubMed Savkur RS, Philips AV, Cooper TA, et al. Insulin receptor splicing alteration in myotonic dystrophy type 2. Am J Hum Genet 2004; 74: 1309–13.CrossRefPubMed
17.
go back to reference Wahbi K, Meune C, Bassez G, et al. Left ventricular non-compaction in a patient with myotonic dystrophy type 2. Neuromuscul Disord 2008; 18: 331–3.CrossRefPubMed Wahbi K, Meune C, Bassez G, et al. Left ventricular non-compaction in a patient with myotonic dystrophy type 2. Neuromuscul Disord 2008; 18: 331–3.CrossRefPubMed
18.
go back to reference Groh WJ, Groh MR, Saha C, et al. Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1. N Engl J Med 2008; 358: 2688–97.CrossRefPubMed Groh WJ, Groh MR, Saha C, et al. Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1. N Engl J Med 2008; 358: 2688–97.CrossRefPubMed
19.
go back to reference Schoser BG, Ricker K, Schneider-Gold C, et al. Sudden cardiac death in myotonic dystrophy type 2. Neurology 2004; 63: 2402–4.PubMed Schoser BG, Ricker K, Schneider-Gold C, et al. Sudden cardiac death in myotonic dystrophy type 2. Neurology 2004; 63: 2402–4.PubMed
20.
go back to reference Schneider-Gold C, Beer M, Kostler H, et al. Cardiac and skeletal muscle involvement in myotonic dystrophy type 2 (DM2): A quantitative 31P-MRS and MRI study. Muscle Nerve 2004; 30: 636–44.CrossRefPubMed Schneider-Gold C, Beer M, Kostler H, et al. Cardiac and skeletal muscle involvement in myotonic dystrophy type 2 (DM2): A quantitative 31P-MRS and MRI study. Muscle Nerve 2004; 30: 636–44.CrossRefPubMed
21.
go back to reference Flachenecker P, Schneider C, Cursiefen S, Ricker K, Toyka KV, Reiners K. Assessment of cardiovascular autonomic function in myotonic dystrophy type 2 (DM2/PROMM). Neuromuscul Disord 2003; 13: 289–93.CrossRefPubMed Flachenecker P, Schneider C, Cursiefen S, Ricker K, Toyka KV, Reiners K. Assessment of cardiovascular autonomic function in myotonic dystrophy type 2 (DM2/PROMM). Neuromuscul Disord 2003; 13: 289–93.CrossRefPubMed
22.
go back to reference Tieleman AA, van Vliet J, Jansen JB, van der Kooi AJ, Borm GF, van Engelen BGM. Gastrointestinal involvement is frequent in myotonic dystrophy type 2. Neuromuscul Disord 2008; 18: 646–9.CrossRefPubMed Tieleman AA, van Vliet J, Jansen JB, van der Kooi AJ, Borm GF, van Engelen BGM. Gastrointestinal involvement is frequent in myotonic dystrophy type 2. Neuromuscul Disord 2008; 18: 646–9.CrossRefPubMed
23.
go back to reference Auvinen S, Suominen T, Hannonen P, Bachinski LL, Krahe R, Udd B. Myotonic dystrophy type 2 found in two of sixty-three persons diagnosed as having fibromyalgia. Arthritis Rheum 2008; 58: 3627–31.CrossRefPubMed Auvinen S, Suominen T, Hannonen P, Bachinski LL, Krahe R, Udd B. Myotonic dystrophy type 2 found in two of sixty-three persons diagnosed as having fibromyalgia. Arthritis Rheum 2008; 58: 3627–31.CrossRefPubMed
24.
go back to reference Rudnik-Schoneborn S, Schneider-Gold C, Raabe U, Kress W, Zerres K, Schoser BG. Outcome and effect of pregnancy in myotonic dystrophy type 2. Neurology 2006; 66: 579–80.CrossRefPubMed Rudnik-Schoneborn S, Schneider-Gold C, Raabe U, Kress W, Zerres K, Schoser BG. Outcome and effect of pregnancy in myotonic dystrophy type 2. Neurology 2006; 66: 579–80.CrossRefPubMed
25.
go back to reference Modoni A, Silvestri G, Pomponi MG, Mangiola F, Tonali PA, Marra C. Characterization of the pattern of cognitive impairment in myotonic dystrophy type 1. Arch Neurol 2004; 61: 1943–7.CrossRefPubMed Modoni A, Silvestri G, Pomponi MG, Mangiola F, Tonali PA, Marra C. Characterization of the pattern of cognitive impairment in myotonic dystrophy type 1. Arch Neurol 2004; 61: 1943–7.CrossRefPubMed
26.
go back to reference Gaul C, Schmidt T, Windisch G, et al. Subtle cognitive dysfunction in adult onset myotonic dystrophy type 1 (DM1) and type 2 (DM2). Neurology 2006; 67: 350–2.CrossRefPubMed Gaul C, Schmidt T, Windisch G, et al. Subtle cognitive dysfunction in adult onset myotonic dystrophy type 1 (DM1) and type 2 (DM2). Neurology 2006; 67: 350–2.CrossRefPubMed
27.
go back to reference Sansone V, Gandossini S, Cotelli M, Calabria M, Zanetti O, Meola G. Cognitive impairment in adult myotonic dystrophies: a longitudinal study. Neurol Sci 2007; 28: 9–15.CrossRefPubMed Sansone V, Gandossini S, Cotelli M, Calabria M, Zanetti O, Meola G. Cognitive impairment in adult myotonic dystrophies: a longitudinal study. Neurol Sci 2007; 28: 9–15.CrossRefPubMed
28.
go back to reference Moxley RT 3rd, Meola G, Udd B, Ricker K. Report of the 84th ENMC workshop: PROMM (proximal myotonic myopathy) and other myotonic dystrophy-like syndromes: 2nd workshop. 13-15th October, 2000, Loosdrecht, The Netherlands. Neuromuscul Disord 2002; 12: 306–17. Moxley RT 3rd, Meola G, Udd B, Ricker K. Report of the 84th ENMC workshop: PROMM (proximal myotonic myopathy) and other myotonic dystrophy-like syndromes: 2nd workshop. 13-15th October, 2000, Loosdrecht, The Netherlands. Neuromuscul Disord 2002; 12: 306–17.
29.
go back to reference Mathieu J, Allard P, Potvin L, Prevost C, Begin P. A 10-year study of mortality in a cohort of patients with myotonic dystrophy. Neurology 1999; 52: 1658–62.PubMed Mathieu J, Allard P, Potvin L, Prevost C, Begin P. A 10-year study of mortality in a cohort of patients with myotonic dystrophy. Neurology 1999; 52: 1658–62.PubMed
30.
go back to reference Meola G, Sansone V, Marinou K, et al. Proximal myotonic myopathy: a syndrome with a favourable prognosis? J Neurol Sci 2002; 193: 89–96.CrossRefPubMed Meola G, Sansone V, Marinou K, et al. Proximal myotonic myopathy: a syndrome with a favourable prognosis? J Neurol Sci 2002; 193: 89–96.CrossRefPubMed
31.
go back to reference von zur Muhlen F, Klass C, Kreuzer H, Mall G, Giese A, Reimers CD. Cardiac involvement in proximal myotonic myopathy. Heart 1998; 79: 619–21. von zur Muhlen F, Klass C, Kreuzer H, Mall G, Giese A, Reimers CD. Cardiac involvement in proximal myotonic myopathy. Heart 1998; 79: 619–21.
32.
go back to reference Tieleman AA, Knuijt S, van Vliet J, de Swart BJ, Ensink R, van Engelen BG. Dysphagia is present but mild in myotonic dystrophy type 2. Neuromuscul Disord 2009; 19: 196–8.CrossRefPubMed Tieleman AA, Knuijt S, van Vliet J, de Swart BJ, Ensink R, van Engelen BG. Dysphagia is present but mild in myotonic dystrophy type 2. Neuromuscul Disord 2009; 19: 196–8.CrossRefPubMed
33.
go back to reference Milone M, Batish SD, Daube JR. Myotonic dystrophy type 2 with focal asymmetric muscle weakness and no electrical myotonia. Muscle Nerve 2009; 39: 383–5.CrossRefPubMed Milone M, Batish SD, Daube JR. Myotonic dystrophy type 2 with focal asymmetric muscle weakness and no electrical myotonia. Muscle Nerve 2009; 39: 383–5.CrossRefPubMed
34.
go back to reference Young N, Milone M, Daube JR. Absent or unrecognized electromyographic myotonia in myotonic dystrophy type 2. Neurology 2009; 72 Suppl 3: A823. Young N, Milone M, Daube JR. Absent or unrecognized electromyographic myotonia in myotonic dystrophy type 2. Neurology 2009; 72 Suppl 3: A823.
Metadata
Title
Anesthesia and myotonic dystrophy type 2: a case series
Authors
Toby N. Weingarten, MD
Ryan E. Hofer, MMS
Margherita Milone, MD, PhD
Juraj Sprung, MD, PhD
Publication date
01-03-2010
Publisher
Springer-Verlag
Published in
Canadian Journal of Anesthesia/Journal canadien d'anesthésie / Issue 3/2010
Print ISSN: 0832-610X
Electronic ISSN: 1496-8975
DOI
https://doi.org/10.1007/s12630-009-9244-1

Other articles of this Issue 3/2010

Canadian Journal of Anesthesia/Journal canadien d'anesthésie 3/2010 Go to the issue

Review Article/Brief Review

Office-based anesthesia