Skip to main content
SpringerLink
Log in

Sleep Issues in Motor Neuron Diseases

  • Chapter
  • First Online:
Sleep Issues in Neuromuscular Disorders

  • 547 Accesses

Abstract

Motor neuron diseases (MND) are progressive neuromuscular disorders that affect lower motor neurons and sometimes, upper motor neurons. Some of the MNDs manifest early in life while some develop later. Sleep disordered breathing (SDB) is a very common association in these diseases and can present as sleep apnea or hypoventilation. Untreated, SDB contributes to morbidity and reduced quality of life. In this chapter, we discuss the different types of MNDs and then about the associated sleep issues in these disorders. Early recognition of these associated sleep disorders and appropriate therapeutic intervention is the key to improve quality of life and reduce morbidity and mortality in these patients.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 54.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 69.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 99.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Abbreviations

AHI:

Apnea hypopnea index

ALS:

Amyotrophic lateral sclerosis

LOSMoN:

Late onset spinal motor neuronopathy

MA:

Monomelic amyotrophy

NIV:

Noninvasive ventilation

NREM:

Non-rapid eye movement

OSA:

Obstructive sleep apnea

PLMS:

Periodic leg movements in sleep

PLS:

Primary lateral sclerosis

PMA:

Progressive muscular atrophy

PSG:

Polysomnogram

REM:

Rapid eye movement

RERA:

Respiratory effort-related arousals

RLS:

Restless leg syndrome

SDB:

Sleep disordered breathing

SMA:

Spinal muscular atrophy

SMA-LED:

SMA with lower extremity predominance

SMN:

Survival motor neuron

SNIP:

Sniff nasal inspiratory pressure

SPSMA:

Scapuloperoneal SMA

References

  1. Kolb SJ, Kissel JT. Spinal muscular atrophy. Neurol Clin. 2015;33(4):831–46.

    Article  Google Scholar 

  2. Jokela M, Penttila S, Huovinen S, Hackman P, Saukkonen AM, Toivanen J, et al. Late-onset lower motor neuronopathy: a new autosomal dominant disorder. Neurology. 2011;77(4):334–40.

    Article  CAS  Google Scholar 

  3. Harms MB, Allred P, Gardner R Jr, Fernandes Filho JA, Florence J, Pestronk A, et al. Dominant spinal muscular atrophy with lower extremity predominance: linkage to 14q32. Neurology. 2010;75(6):539–46.

    Article  CAS  Google Scholar 

  4. Rudnik-Schoneborn S, Deden F, Eggermann K, Eggermann T, Wieczorek D, Sellhaus B, et al. Autosomal dominant spinal muscular atrophy with lower extremity predominance: a recognizable phenotype of BICD2 mutations. Muscle Nerve. 2016;54(3):496–500.

    Article  Google Scholar 

  5. Tsurusaki Y, Saitoh S, Tomizawa K, Sudo A, Asahina N, Shiraishi H, et al. A DYNC1H1 mutation causes a dominant spinal muscular atrophy with lower extremity predominance. Neurogenetics. 2012;13(4):327–32.

    Article  CAS  Google Scholar 

  6. Niu Q, Wang X, Shi M, Jin Q. A novel DYNC1H1 mutation causing spinal muscular atrophy with lower extremity predominance. Neurol Genet. 2015;1(2):e20.

    Article  Google Scholar 

  7. La Spada AR, Wilson EM, Lubahn DB, Harding AE, Fischbeck KH. Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature. 1991;352(6330):77–9.

    Article  Google Scholar 

  8. Finsterer J. Bulbar and spinal muscular atrophy (Kennedy’s disease): a review. Eur J Neurol. 2009;16(5):556–61.

    Article  CAS  Google Scholar 

  9. Goldenberg JN, Bradley WG. Testosterone therapy and the pathogenesis of Kennedy’s disease (X-linked bulbospinal muscular atrophy). J Neurol Sci. 1996;135(2):158–61.

    Article  CAS  Google Scholar 

  10. Finsterer J, Soraru G. Onset manifestations of spinal and bulbar muscular atrophy (Kennedy’s disease). J Mol Neurosci. 2016;58(3):321–9.

    Article  CAS  Google Scholar 

  11. Statland JM, Barohn RJ, McVey AL, Katz JS, Dimachkie MM. Patterns of weakness, classification of motor neuron disease, and clinical diagnosis of sporadic amyotrophic lateral sclerosis. Neurol Clin. 2015;33(4):735–48.

    Article  Google Scholar 

  12. Ingre C, Roos PM, Piehl F, Kamel F, Fang F. Risk factors for amyotrophic lateral sclerosis. Clin Epidemiol. 2015;7:181–93.

    PubMed  PubMed Central  Google Scholar 

  13. Al-Chalabi A, Hardiman O, Kiernan MC, Chio A, Rix-Brooks B, van den Berg LH. Amyotrophic lateral sclerosis: moving towards a new classification system. Lancet Neurol. 2016;15(11):1182–94.

    Article  Google Scholar 

  14. Chio A, Calvo A, Moglia C, Mazzini L, Mora G, PARALS Study Group. Phenotypic heterogeneity of amyotrophic lateral sclerosis: a population based study. J Neurol Neurosurg Psychiatry. 2011;82(7):740–6.

    Article  Google Scholar 

  15. Phukan J, Elamin M, Bede P, Jordan N, Gallagher L, Byrne S, et al. The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study. J Neurol Neurosurg Psychiatry. 2012;83(1):102–8.

    Article  Google Scholar 

  16. Hamano T, Mutoh T, Hirayama M, Kuriyama M. [Benign monomelic amyotrophy]. Ryoikibetsu Shokogun Shirizu. 1999;(27 Pt 2):388–91.

    Google Scholar 

  17. Delerue O, Hurtevent JF, Destee A. [Benign, monomelic juvenile amyotrophy of a hand (Hirayama type): a new case report]. Acta Neurol Belg. 1990;90(2):82–6.

    Google Scholar 

  18. Drozdowski W, Baniukiewicz E, Lewonowska M. [Juvenile monomelic amyotrophy: Hirayama disease]. Neurol Neurochir Pol. 1998;32(4):943–50.

    Google Scholar 

  19. Nalini A, Gourie-Devi M, Thennarasu K, Ramalingaiah AH. Monomelic amyotrophy: clinical profile and natural history of 279 cases seen over 35 years (1976-2010). Amyotroph Lateral Scler Frontotemporal Degener. 2014;15(5-6):457–65.

    Article  Google Scholar 

  20. Liewluck T, Saperstein DS. Progressive muscular atrophy. Neurol Clin. 2015;33(4):761–73.

    Article  Google Scholar 

  21. Statland JM, Barohn RJ, Dimachkie MM, Floeter MK, Mitsumoto H. Primary lateral sclerosis. Neurol Clin. 2015;33(4):749–60.

    Article  Google Scholar 

  22. Dion PA, Daoud H, Rouleau GA. Genetics of motor neuron disorders: new insights into pathogenic mechanisms. Nat Rev Genet. 2009;10(11):769–82.

    Article  CAS  Google Scholar 

  23. Fermin AM, Afzal U, Culebras A. Sleep in neuromuscular diseases. Sleep Med Clin. 2016;11(1):53–64.

    Article  Google Scholar 

  24. Gozal D. Pulmonary manifestations of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy. Pediatr Pulmonol. 2000;29(2):141–50.

    Article  CAS  Google Scholar 

  25. Santos C, Braghiroli A, Mazzini L, Pratesi R, Oliveira LV, Mora G. Sleep-related breathing disorders in amyotrophic lateral sclerosis. Monaldi Arch Chest Dis. 2003;59(2):160–5.

    CAS  PubMed  Google Scholar 

  26. Horner RL. Pathophysiology of obstructive sleep apnea. J Cardiopulm Rehabil Prev. 2008;28(5):289–98.

    Article  Google Scholar 

  27. Dhand UK, Goyal MK, Sahota PK. Sleep related hypoventilation/hypoxemia due to neuromuscular and chest wall disorders. In: Kushida C, editor. Encyclopedia of sleep. 1st ed: Academic Press; 2012.

    Google Scholar 

  28. Gaig C, Iranzo A. Sleep-disordered breathing in neurodegenerative diseases. Curr Neurol Neurosci Rep. 2012;12(2):205–17.

    Article  Google Scholar 

  29. Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, et al. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009;73(15):1218–26.

    Article  CAS  Google Scholar 

  30. Berry RB, Budhiraja R, Gottlieb DJ, Gozal D, Iber C, Kapur VK, et al. Rules for scoring respiratory events in sleep: update of the 2007 AASM manual for the scoring of sleep and associated events. Deliberations of the Sleep Apnea Definitions Task Force of the American Academy of Sleep Medicine. J Clin Sleep Med. 2012;8(5):597–619.

    PubMed  PubMed Central  Google Scholar 

  31. Piper AJ. Nocturnal hypoventilation - identifying & treating syndromes. Indian J Med Res. 2010;131:350–65.

    PubMed  Google Scholar 

  32. Flenley DC. Sleep in chronic obstructive lung disease. Clin Chest Med. 1985;6(4):651–61.

    CAS  PubMed  Google Scholar 

  33. Labanowski M, Schmidt-Nowara W, Guilleminault C. Sleep and neuromuscular disease: frequency of sleep-disordered breathing in a neuromuscular disease clinic population. Neurology. 1996;47(5):1173–80.

    Article  CAS  Google Scholar 

  34. Nicolle MW. Sleep and neuromuscular disease. Semin Neurol. 2009;29(4):429–37.

    Article  CAS  Google Scholar 

  35. Romigi A, Liguori C, Placidi F, Albanese M, Izzi F, Uasone E, et al. Sleep disorders in spinal and bulbar muscular atrophy (Kennedy’s disease): a controlled polysomnographic and self-reported questionnaires study. J Neurol. 2014;261(5):889–93.

    Article  Google Scholar 

  36. Sleep-related breathing disorders in adults: recommendations for syndrome definition and measurement techniques in clinical research. The report of an American Academy of Sleep Medicine Task Force. Sleep. 1999;22(5):667–89.

    Google Scholar 

  37. Kimura K, Tachibana N, Kimura J, Shibasaki H. Sleep-disordered breathing at an early stage of amyotrophic lateral sclerosis. J Neurol Sci. 1999;164(1):37–43.

    Article  CAS  Google Scholar 

  38. David WS, Bundlie SR, Mahdavi Z. Polysomnographic studies in amyotrophic lateral sclerosis. J Neurol Sci. 1997;152(Suppl 1):S29–35.

    Article  Google Scholar 

  39. Arnulf I, Similowski T, Salachas F, Garma L, Mehiri S, Attali V, et al. Sleep disorders and diaphragmatic function in patients with amyotrophic lateral sclerosis. Am J Respir Crit Care Med. 2000;161(3 Pt 1):849–56.

    Article  CAS  Google Scholar 

  40. Lyall RA, Donaldson N, Polkey MI, Leigh PN, Moxham J. Respiratory muscle strength and ventilatory failure in amyotrophic lateral sclerosis. Brain. 2001;124(Pt 10):2000–13.

    Article  CAS  Google Scholar 

  41. Allen RP, Picchietti D, Hening WA, Trenkwalder C, Walters AS, Montplaisi J, et al. Restless legs syndrome: diagnostic criteria, special considerations, and epidemiology. A report from the restless legs syndrome diagnosis and epidemiology workshop at the National Institutes of Health. Sleep Med. 2003;4(2):101–19.

    Article  Google Scholar 

  42. Lo Coco D, Piccoli F, La Bella V. Restless legs syndrome in patients with amyotrophic lateral sclerosis. Mov Disord. 2010;25(15):2658–61.

    Article  Google Scholar 

  43. Lo Coco D, Puligheddu M, Mattaliano P, Congiu P, Borghero G, Fantini ML, et al. REM sleep behavior disorder and periodic leg movements during sleep in ALS. Acta Neurol Scand. 2017;135(2):219–24.

    Article  CAS  Google Scholar 

  44. Schutte-Rodin S, Broch L, Buysse D, Dorsey C, Sateia M. Clinical guideline for the evaluation and management of chronic insomnia in adults. J Clin Sleep Med. 2008;4(5):487–504.

    PubMed  PubMed Central  Google Scholar 

  45. Javad Mousavi SA, Zamani B, Shahabi Shahmiri S, Rohani M, Shahidi GA, Mostafapour E, et al. Pulmonary function tests in patients with amyotrophic lateral sclerosis and the association between these tests and survival. Iran J Neurol. 2014;13(3):131–7.

    PubMed  PubMed Central  Google Scholar 

  46. Bach JR, Bakshiyev R, Hon A. Noninvasive respiratory management for patients with spinal cord injury and neuromuscular disease. Tanaffos. 2012;11(1):7–11.

    PubMed  PubMed Central  Google Scholar 

  47. Kim SM, Park KS, Nam H, Ahn SW, Kim S, Sung JJ, et al. Capnography for assessing nocturnal hypoventilation and predicting compliance with subsequent noninvasive ventilation in patients with ALS. PLoS One. 2011;6(3):e17893.

    Article  CAS  Google Scholar 

  48. Stefanutti D, Benoist MR, Scheinmann P, Chaussain M, Fitting JW. Usefulness of sniff nasal pressure in patients with neuromuscular or skeletal disorders. Am J Respir Crit Care Med. 2000;162(4 Pt 1):1507–11.

    Article  CAS  Google Scholar 

  49. Miller JM, Moxham J, Green M. The maximal sniff in the assessment of diaphragm function in man. Clin Sci (Lond). 1985;69(1):91–6.

    Article  CAS  Google Scholar 

  50. Carratu P, Cassano A, Gadaleta F, Tedone M, Dongiovanni S, Fanfulla F, et al. Association between low sniff nasal-inspiratory pressure (SNIP) and sleep disordered breathing in amyotrophic lateral sclerosis: Preliminary results. Amyotroph Lateral Scler. 2011;12(6):458–63.

    Article  Google Scholar 

  51. Aurora RN, Kristo DA, Bista SR, Rowley JA, Zak RS, Casey KR, et al. The treatment of restless legs syndrome and periodic limb movement disorder in adults–an update for 2012: practice parameters with an evidence-based systematic review and meta-analyses: an American Academy of Sleep Medicine Clinical Practice Guideline. Sleep. 2012;35(8):1039–62.

    Article  Google Scholar 

  52. Clinical indications for noninvasive positive pressure ventilation in chronic respiratory failure due to restrictive lung disease, COPD, and nocturnal hypoventilation–a consensus conference report. Chest. 1999;116(2):521–34.

    Google Scholar 

  53. Boentert M, Brenscheidt I, Glatz C, Young P. Effects of non-invasive ventilation on objective sleep and nocturnal respiration in patients with amyotrophic lateral sclerosis. J Neurol. 2015;262(9):2073–82.

    Article  CAS  Google Scholar 

  54. Gonzalez-Bermejo J, Morelot-Panzini C, Salachas F, Redolfi S, Straus C, Becquemin MH, et al. Diaphragm pacing improves sleep in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2012;13(1):44–54.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, University of Rochester, Rochester, NY, USA

    Sushma Yerram M.D.

  2. Department of Neurology, University of Missouri, Columbia, MO, USA

    Pradeep C. Bollu M.D. & Pradeep Sahota M.D., F.A.A.N.

Authors
  1. Sushma Yerram M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Pradeep C. Bollu M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Pradeep Sahota M.D., F.A.A.N.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Pradeep C. Bollu M.D. .

Editor information

Editors and Affiliations

  1. Department of Neurology, University of Missouri, Columbia, Missouri, USA

    Raghav Govindarajan

  2. Department of Neurology, University of Missouri , Columbia, Missouri, USA

    Pradeep C. Bollu

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Yerram, S., Bollu, P.C., Sahota, P. (2018). Sleep Issues in Motor Neuron Diseases. In: Govindarajan, R., Bollu, P. (eds) Sleep Issues in Neuromuscular Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-73068-4_3

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-73068-4_3

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-73067-7

  • Online ISBN: 978-3-319-73068-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation