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Frequency of nocturnal sudden death in patients with multiple system atrophy

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Abstract

Sudden death has been reported in patients with multiple system atrophy (MSA), although the frequency of this event has not been well delineated. We investigated the frequency and potential causes of sudden death in patients with MSA. During the 5-year observation period, 10 of 45 patients with probable MSA died. The causes of death included sudden death of unknown etiology (seven patients), aspiration pneumonia (one patient), asphyxia after vomiting (one patient), and lung cancer (one patient). The mean survival time of patients with sudden death was 63.0 ± 24.7 months (range, 39–116 months). Among seven patients who experienced sudden death, six were found to have died during sleep. Among these patients, two had been treated with tracheostomy and three with continuous positive airway pressure (CPAP) or noninvasive positive pressure ventilation (NPPV) during sleep, suggesting that these treatments do not always prevent sudden death in patients with MSA. Nocturnal sudden death should be recognized as the most common mechanism of death in patients with MSA.

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References

  1. Gilman S, Low PA, Quinn N, et al. (1999) Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci 163:94–98

    Article  PubMed  CAS  Google Scholar 

  2. Iranzo A, Santamaria J, Tolosa E, et al. (2004) Long-term effect of CPAP in the treatment of nocturnal stridor in multiple system atrophy. Neurology 63:930–932

    PubMed  CAS  Google Scholar 

  3. Isozaki E, Naito A, Horiguchi S, et al. (1996) Early diagnosis and stage classification of vocal cord abductor paralysis in patients with multiple system atrophy. J Neurol Neurosurg Psychiatry 60:399–402

    Article  PubMed  CAS  Google Scholar 

  4. Jin K, Okabe S, Chida K, et al. (2007) Tracheostomy can fatally exacerbate sleep-disordered breathing in multiple system atrophy. Neurology 68:1618–1621

    Article  PubMed  CAS  Google Scholar 

  5. Konagaya M, Konagaya Y, Iida M (1994) Clinical and magnetic resonance imaging study of extrapyramidal symptoms in multiple system atrophy. J Neurol Neurosurg Psychiatry 57:1528–1531

    Article  PubMed  CAS  Google Scholar 

  6. Lapresle J, Annabi A (1979) Olivopontocerebellar atrophy with velopharyngolaryngeal paralysis: a contribution to the somatotopy of the nucleus ambiguus. J Neuropathol Exp Neurol 38:401–406

    Article  PubMed  CAS  Google Scholar 

  7. Munschauer FE, Loh L, Bannister R, et al. (1990) Abnormal respiration and sudden death during sleep in multiple system atrophy with autonomic failure. Neurology 40:677–679

    PubMed  CAS  Google Scholar 

  8. Papapetropoulos S, Tuchman A, Laufer D, et al. (2007) Causes of death in multiple system atrophy. J Neurol Neurosurg Psychiatry 78:327–329

    Article  PubMed  Google Scholar 

  9. Shimohata T, Nakayama H, Shinoda H, et al. (2006) Multiple system atrophy with progressive nocturnal hypoxemia: case report with polysomnography and continuous positive airway pressure treatment. Eur Neurol 56:258–260

    Article  PubMed  Google Scholar 

  10. Shimohata T, Shinoda H, Nakayama H, et al. (2007) Daytime hypoxemia, sleep-disordered breathing and laryngopharyngeal findings in multiple system atrophy. Arch Neurol 64:856–861

    Article  PubMed  Google Scholar 

  11. Silber MH, Levine S (2000) Stridor and death in multiple system atrophy. Move Disord 15:699–704

    Article  CAS  Google Scholar 

  12. Tsuda T, Onodera H, Okabe S, et al. (2002) Impaired chemosensitivity to hypoxia is a marker of multiple system atrophy. Ann Neurol 52:367–371

    Article  PubMed  CAS  Google Scholar 

  13. Yabe I, Soma H, Takei A, et al. (2006) MSA-C is the predominant clinical phenotype of MSA in Japan: analysis of 142 patients with probable MSA. J Neurol Sci 249:115–121

    Article  PubMed  Google Scholar 

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Correspondence to T. Shimohata M.D., Ph.D..

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Shimohata, T., Ozawa, T., Nakayama, H. et al. Frequency of nocturnal sudden death in patients with multiple system atrophy. J Neurol 255, 1483–1485 (2008). https://doi.org/10.1007/s00415-008-0941-4

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  • DOI: https://doi.org/10.1007/s00415-008-0941-4

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