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Myotonic Dystrophy pp 171–187Cite as

Dysphagia in Myotonic Dystrophy

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Abstract

In myotonic dystrophy type 1, all stages in the five swallowing model are disturbed.

Deglutition-related muscle weakness, as opposed to myotonia, was the most significant contributor to impairment.

In anticipatory stage, some patients show poor awareness of dysphagia. Aberrations of feeding behaviors and silent aspiration have been reported.

In oral preparatory and propulsive stage, force of masticatory and labia oris is degraded, but patients sometimes swallow foods without adequate chewing. Myotonic phenomena are seen in the circumferential musculature of the buccal capsule.

In pharyngeal stage, decreased pharyngeal peristalsis, delayed deglutition reflex, and epiglottis dysraphic are observed. The threshold of the cough reflex is high, and this is related to silent aspiration.

In esophageal stage, degradation of esophagus peristalsis and gastroesophageal reflux are present.

Myotonic phenomena reduce with hyperthermia treatment on the masseter muscle, and that induces easier mouth opening. A warm-up phenomenon is seen with repeated opening and closing of the mouth.

Dilatation of upper esophageal sphincter using balloon catheter is effective.

When drop in SpO2 is revealed during meals due to respiratory disturbance, ventilation is desirable while eating.

During percutaneous endoscopic gastrostomy, we need to keep watching for aggravation of respiratory insufficiency.

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References

  1. Takara K, Fukunaga H, Kawai M. Clinical survey of muscular dystrophy in hospitals of National Hospital Organization. Iryo. 2006;60:112–8.

    Google Scholar 

  2. Baptista H, Lopes Cardoso I. Steinert syndrome and repercussions in dental medicine. Arch Oral Biol. 2017;75:37–47.

    Article  Google Scholar 

  3. Hiiemae KM, Palmer JB. Food transport and bolus formation during complete feeding sequences on foods of different initial consistency. Dysphagia. 1999;14(1):31–42.

    Article  CAS  Google Scholar 

  4. Leopold NA, Kagel MC. Swallowing, ingestion and dysphagia: a reappraisal. Arch Phys Med Rehabil. 1983;64(8):371–3.

    CAS  PubMed  Google Scholar 

  5. Mann G. The Mann assessment of swallowing ability. New York: Delmar Cengage Learning; 2001.

    Google Scholar 

  6. Cook IJ, Dodds WJ, Dantas RO, Kern MK, Massey BT, Shaker R, et al. Timing of videofluoroscopic, anometric events, and bolus transit during the oral and pharyngeal phases of swallowing. Dysphagia. 1989;4(1):8–15.

    Article  CAS  Google Scholar 

  7. Langmore SE. Endoscopic evaluation and treatment of swallowing disorders. New York: Thieme Med Pub; 2001.

    Google Scholar 

  8. Wakasugi Y, Tohara H, Hattori F, Motohashi Y, Nakane A, Goto S, et al. Screening test for silent aspiration at the bedside. Dysphagia. 2008;23(4):364–70.

    Article  Google Scholar 

  9. Takahashi K, Groher ME, Michi K. Symmetry and reproducibility of swallowing sounds. Dysphagia. 1994 Summer;9(3):168–73.

    Article  CAS  Google Scholar 

  10. Yoshikawa M, Yoshida M, Tsuga K, Akagawa Y, Groher ME. Comparison of three types of tongue pressure measurement devices. Dysphagia. 2011;26(3):232–7.

    Article  Google Scholar 

  11. Takasaki K, Umeki H, Enatsu K, Tanaka F, Sakihama N, Kumagami H, et al. Investigation of pharyngeal swallowing function using high-resolution manometry. Laryngoscope. 2008;118(10):1729–32.

    Article  Google Scholar 

  12. Marcon M, Briani C, Ermani M, Menegazzo E, Iurilli V, Feltrin GP, et al. Positive correlation of CTG expansion and pharyngoesophageal alterations in myotonic dystrophy patients. Ital J Neurol Sci. 1998;19(2):75–80.

    Article  CAS  Google Scholar 

  13. Bellini M, Biagi S, Stasi C, Costa F, Mumolo MG, Ricchiuti A, et al. Gastrointestinal manifestations in myotonic muscular dystrophy. World J Gastroenterol. 2006;12(12):1821–8.

    Article  Google Scholar 

  14. Ertekin C, Yüceyar N, Aydogdu I, Karasoy H. Electrophysiological evaluation of oropharyngeal swallowing in myotonic dystrophy. J Neurol Neurosurg Psychiatry. 2001;70(3):363–71.

    Article  CAS  Google Scholar 

  15. Ercolin B, Sassi FC, Mangilli LD, Mendonça LI, Limongi SC, de Andrade CR. Oral motor movements and swallowing in patients with myotonic dystrophy type 1. Dysphagia. 2013;28(3):446–54.

    Article  Google Scholar 

  16. Dogan C, De Antonio M, Hamroun D, Varet H, Fabbro M, Rougier F, et al. Gender as a modifying factor influencing myotonic dystrophy type 1 phenotype severity and mortality: a nationwide multiple databases cross-sectional observational study. PLoS One. 2016;11(2):e0148264.

    Article  Google Scholar 

  17. Leonard RJ, Kendall KA, Johnson R, McKenzie S. Swallowing in myotonic muscular dystrophy: a videofluoroscopic study. Arch Phys Med Rehabil. 2001;82(7):979–85.

    Article  CAS  Google Scholar 

  18. Willaert A, Jorissen M, Goeleven A. Swallowing dysfunction in myotonic dystrophy: a retrospective study of symptomatology and radiographic findings. B-ENT. 2015;11(4):249–56.

    CAS  PubMed  Google Scholar 

  19. LaDonna KA, Koopman WJ, Ray SL, Venance SL. Hard to swallow: a phenomenological exploration of the experience of caring for individuals with myotonic dystrophy and dysphagia. J Neurosci Nurs. 2016;48(1):42–51.

    Article  Google Scholar 

  20. Kiliaridis S, Katsaros C. The effect of myotonic dystrophy and Duchenne muscular dystrophy on the orofacial muscles and dentofacial morphology. Acta Odontol Scand. 1998;56:369–74.

    Article  CAS  Google Scholar 

  21. Sasaki T. Oral and maxillofacial studies in patients with n/1uscular dystrophies. Iryo. 2007;61(12):786–90.

    Google Scholar 

  22. Sasaki T. Clinical studies in patients with muscular dystrophies: oral and maxillofacial morphology. Iryo. 2007;61(10):645–51.

    Google Scholar 

  23. Umemoto G, Tsukiyama Y, Nakamura H, Koyano K. Characterization of masticatory function in patients with Myotonic dystrophy. Part 2: comparison between patients with myotonic dystrophy and healthy individuals. Prosthodont Res Pract. 2006;5(2):68–71.

    Article  Google Scholar 

  24. Umemoto G, Nakamura H, Tsukiyama Y, Koyano K, Kikuta T. The relationship between masticatory function and dysphagia in patients with myotonic dystrophy. Prosthodont Res Pract. 2007;6(2):109–13.

    Article  Google Scholar 

  25. Umemoto G, Tsukiyama Y, Nakamura H, Koyano K. Characterization of masticatory function in patients with myotonic dystrophy. Part1: correlations among the factors of masticatory function. Prosthodont Res Pract. 2006;5(1):31–6.

    Article  Google Scholar 

  26. Umemoto G, Nakamura H, Oya Y, Kikuta T. Masticatory dysfunction in patients with myotonic dystrophy (type 1): a 5-year follow-up. Spec Care Dentist. 2009;29(5):210–4.

    Article  Google Scholar 

  27. Jungheim M, Kühn D, Ptok M. High resolution manometry study of pharyngeal function in patients with myotonic dystrophy. Nervenarzt. 2015;86(8):997–1006.

    Article  CAS  Google Scholar 

  28. Pruzanski W. Respiratory tract infections and silent aspiration in myotonic dystrophy. Dis Chest. 1962;42(6):608–10.

    Article  Google Scholar 

  29. Pilz W, Baijens LW, Passos VL, Verdonschot R, Wesseling F, Roodenburg N, et al. Swallowing assessment in myotonic dystrophy type 1 using fiber optic endoscopic evaluation of swallowing (FEES). Neuromuscul Disord. 2014;24(12):1054–62.

    Article  Google Scholar 

  30. Takahashi N, Hanayama K, Toikawa H, Ishihara T, Chino N. Cough reflex in myotonic dystrophy subjects. J Rehabil Med. 2002;39(3):141–4.

    Article  Google Scholar 

  31. Modolell I, Mearin F, Baudet JS, Gámez J, Cervera C, Malagelada JR. Pharyngo-esophageal motility disturbances in patients with myotonic dystrophy. Scand J Gastroenterol. 1999;34(9):878–82.

    Article  CAS  Google Scholar 

  32. Mano Y, Honda H, Takayanagi T. Electrophysiological analysis of warming up phenomenon in myotonia. Jpn J Med. 1985;24(2):131–4.

    Article  CAS  Google Scholar 

  33. Smith CA, Gutmann L. Myotonic dystrophy type 1 management and therapeutics. Curr Treat Options Neurol. 2016;18(12):52.

    Article  Google Scholar 

  34. Michel P, Sternberg D, Jeannet PY, Dunand M, Thonney F, Kress W, et al. Comparative efficacy of repetitive nerve stimulation, exercise, and cold in differentiating myotonic disorders. Muscle Nerve. 2007;36(5):643–50.

    Article  CAS  Google Scholar 

  35. Nozaki S, Kawaii M, Kimura T, Adachi K, Futamura N, Matsumura T, et al. Oral phase exercise with hot pack in myotonic dystrophy type 1. Iryo. 2011;65(11):555–61.

    Google Scholar 

  36. Nozaki S, Kawai M, Shimoyama R, Futamura N, Matsumura T, Adachi K, et al. Range of motion exercise of temporo-mandibular joint with hot pack increases occlusal force in patients with Duchenne muscular dystrophy. Acta Myol. 2010;29(3):392–7.

    CAS  PubMed  PubMed Central  Google Scholar 

  37. Sjögreen L, Tulinius M, Kiliaridis S, Lohmander A. The effect of lip strengthening exercises in children and adolescents with myotonic dystrophy type 1. Int J Pediatr Otorhinolaryngol. 2010;74(10):1126–34.

    Article  Google Scholar 

  38. Nozaki S, Umaki Y, Tatara K, Tanoue E, Shinno S. Upper esophageal dilation with balloon catheter for patients with muscular dystrophy. Iryo. 2005;59(10):556–60.

    Google Scholar 

  39. Grazia M, Bini A, Stella F, Petrella F, Bazzocchi R. Heller esophageal myotomy and Dor fundoplication for esophageal achalasia in Steinert’s disease. Cardiovasc Surg (Torino). 2003;44(6):785–6.

    CAS  Google Scholar 

  40. Bach JR, Gonzalez M, Sharma A, Swan K, Patel A. Open gastrostomy for noninvasive ventilation users with neuromuscular disease. Am J Phys Med Rehabil. 2010;89(1):1–6.

    Article  Google Scholar 

  41. Fitzgerald BP, Conn KM, Smith J, Walker A, Parkhill AL, Hilbert JE, et al. Medication adherence in patients with myotonic dystrophy and facioscapulohumeral muscular dystrophy. J Neurol. 2016;263(12):2528–37.

    Article  Google Scholar 

  42. Day JW, Ricker K, Jacobsen JF, Rasmussen LJ, Dick KA, Kress W, et al. Myotonic dystrophy type 2: molecular, diagnostic and clinical spectrum. Neurology. 2003;60(4):657–64.

    Article  CAS  Google Scholar 

  43. Tieleman AA, van Vliet J, Jansen JB, van der Kooi AJ, Borm GF, van Engelen BG. Gastrointestinal involvement is frequent in myotonic dystrophy type 2. Neuromuscul Disord. 2008;18(8):646–9.

    Article  Google Scholar 

  44. 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(3):196–8.

    Article  CAS  Google Scholar 

  45. Kimura K, Saito T. Myotonic dystrophy type 2. Brain Nerve. 2011;63(11):1151–60.

    PubMed  Google Scholar 

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Acknowledgment

We wish to thank the Ashiya Municipal Hospital and Toneyama National Hospital for providing valuable cases.

Author information

Authors and Affiliations

  1. Department of Neurology and Rehabilitation, Kansai Rosai Hospital, Amagasaki, Hyogo, Japan

    Sonoko Nozaki

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  1. Sonoko Nozaki
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Correspondence to Sonoko Nozaki .

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Editors and Affiliations

  1. Department of Functional Diagnostic Science, Osaka University Graduate School of Medicine, Osaka, Japan

    Masanori P. Takahashi

  2. Department of Neurology, National Hospital Organization Toneyama National Hospital, Osaka, Japan

    Tsuyoshi Matsumura

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Nozaki, S. (2018). Dysphagia in Myotonic Dystrophy. In: Takahashi, M., Matsumura, T. (eds) Myotonic Dystrophy. Springer, Singapore. https://doi.org/10.1007/978-981-13-0508-5_11

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  • DOI: https://doi.org/10.1007/978-981-13-0508-5_11

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  • Publisher Name: Springer, Singapore

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  • Online ISBN: 978-981-13-0508-5

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