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Sleep disorders in spinal and bulbar muscular atrophy (Kennedy’s disease): a controlled polysomnographic and self-reported questionnaires study

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Abstract

No data are available regarding the occurrence of sleep disorders in spinal and bulbar muscular atrophy (SBMA). We investigated the sleep-wake cycle in SBMA patients compared with healthy subjects. Nine SBMA outpatients and nine age-matched and sex-matched healthy controls were evaluated. Subjective quality of sleep was assessed by means of the Pittsburgh Sleep Quality Index (PSQI). The Epworth Sleepiness Scale was used in order to evaluate excessive daytime sleepiness. All participants underwent a 48-h polysomnography followed by the multiple sleep latency test. Time in bed, total sleep time and sleep efficiency were significantly lower in SBMA than controls. Furthermore, the apnea–hypopnea index (AHI) was significantly higher in SBMA than controls. Obstructive sleep apnea (OSA: AHI >5/h) was evident in 6/9 patients (66.6 %). REM sleep without atonia was evident in three patients also affected by OSA and higher AHI in REM; 2/9 (22.2 %) SBMA patients showed periodic limb movements in sleep. The global PSQI score was higher in SBMA versus controls. Sleep quality in SBMA is poorer than in controls. OSA is the most common sleep disorder in SBMA. The sleep impairment could be induced both by OSA or/and the neurodegenerative processes involving crucial areas regulating the sleep-wake cycle.

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References

  1. La Spada AR, Wilson EM, Lubahn DB, Harding AE, Fischbeck KH (1991) Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature 352:77–79

    Article  PubMed  Google Scholar 

  2. Katsuno M, Banno H, Suzuki K et al (2010) Clinical features and molecular mechanisms of spinal and bulbar muscular atrophy (SBMA). Adv Exp Med Biol 685:64–74

    Article  CAS  PubMed  Google Scholar 

  3. Adachi H, Katsuno M, Minamiyama M et al (2005) Widespread nuclear and cytoplasmic accumulation of mutant androgen receptor in SBMA patients. Brain 128:659–670

    Article  PubMed  Google Scholar 

  4. Hobson JA, Pace-Schott EF (2002) The cognitive neuroscience of sleep: neuronal systems, consciousness and learning. Nat Rev Neurosci 3:679–693

    Article  CAS  PubMed  Google Scholar 

  5. Romigi A, Izzi F, Pisani V et al (2011) Sleep disorders in adult-onset myotonic dystrophy type 1: a controlled polysomnographic study. Eur J Neurol 18:1139–1145

    Article  CAS  PubMed  Google Scholar 

  6. The Report of an American Academy of Sleep Medicine Task Force (1999) Sleep-related breathing disorders in adults: recommendations for syndrome definition and measurement techniques in clinical research. Sleep 22:667–689

    Google Scholar 

  7. ASDA (1993) The atlas task force. Recording and scoring leg movements. Sleep 16:748–759

    Google Scholar 

  8. American Academy of Sleep Medicine (2005) ICSD-2-international classification of sleep disorders, 2nd ed.: diagnostic and coding manual. American Academy of Sleep Medicine, Westchester

    Google Scholar 

  9. Consens FB, Chervin RD, Koeppe RA et al (2005) Validation of a polysomnographic score for REM sleep behavior disorder. Sleep 28:993–997

    PubMed  Google Scholar 

  10. Romigi A, Albanese M, Liguori C et al (2013) Sleep-wake cycle and daytime sleepiness in the myotonic dystrophies. J Neurodegener Dis 2013:13, Article ID 692026. doi:10.1155/2013/692026

  11. Bhat S, Gupta D, Chokroverty S (2012) Sleep disorders in neuromuscular diseases. Neurol Clin 30:1359–1387

    Article  PubMed  Google Scholar 

  12. Rye DB (1997) Contributions of the pedunculopontine region to normal and altered REM sleep. Sleep 20:757–788

    CAS  PubMed  Google Scholar 

  13. Huang J, Zhang J, Lam SP et al (2011) Amelioration of obstructive sleep apnoea in REM sleep behaviour disorder: implications for the neuromuscular control of OSA. Sleep 34:909–915

    PubMed Central  PubMed  Google Scholar 

  14. Romigi A, Albanese M, Placidi F et al (2013) Sleep disorders in myotonic dystrophy type 2: a controlled polysomnographic study and self-reported questionnaires. Eur J Neurol. doi:10.1111/ene.12226

    PubMed  Google Scholar 

  15. Chervin RD, Aldrich MS (1999) The Epworth Sleepiness Scale may not reflect objective measures of sleepiness or sleep apnea. Neurology 52:125–131

    Article  CAS  PubMed  Google Scholar 

Download references

Conflicts of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

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Authors and Affiliations

  1. Neurophysiopathology Unit, Department of Systems Medicine, Sleep Medicine Centre, Tor Vergata University and Hospital, Rome, Italy

    Andrea Romigi, Claudio Liguori, Fabio Placidi, Maria Albanese, Francesca Izzi, Elisabetta Uasone, Nicola Biagio Mercuri & Raffaella Ludovisi

  2. Neurology Unit, Department of Systems Medicine, Neuromuscular Centre, Tor Vergata University and Hospital, Rome, Italy

    Chiara Terracciano, Maria Grazia Marciani & Roberto Massa

  3. IRCCS Santa Lucia Foundation, Rome, Italy

    Nicola Biagio Mercuri & Roberto Massa

Authors
  1. Andrea Romigi
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  2. Claudio Liguori
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  3. Fabio Placidi
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  4. Maria Albanese
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  5. Francesca Izzi
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  6. Elisabetta Uasone
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  7. Chiara Terracciano
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  8. Maria Grazia Marciani
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  9. Nicola Biagio Mercuri
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  10. Raffaella Ludovisi
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  11. Roberto Massa
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Corresponding author

Correspondence to Andrea Romigi.

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Romigi, A., Liguori, C., Placidi, F. et al. Sleep disorders in spinal and bulbar muscular atrophy (Kennedy’s disease): a controlled polysomnographic and self-reported questionnaires study. J Neurol 261, 889–893 (2014). https://doi.org/10.1007/s00415-014-7293-z

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  • DOI: https://doi.org/10.1007/s00415-014-7293-z

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