The Italian Journal of Neurological Sciences

, Volume 14, Issue 7, pp 565–569 | Cite as

Sleep related breathing patterns in patients with spinal muscular atrophy

  • Manni R. 
  • Cerveri I. 
  • Ottolini A. 
  • Fanfulla F. 
  • Zoia M. C. 
  • Lanzi G. 
  • Tartara A. 
Original Articles

Abstract

A clinical and polygraphic study of nocturnal sleep was performed in 8 (4 males, 4 females; age range 10–37 years) patients with spinal muscular atrophy, whose baseline respiratory function assessment during wakefulness showed restrictive ventilatory syndrome but blood-gas tension within normal limits. No patient reported any significant sleep complaint suggestive of sleepdisordered breathing. However, in 4 patients HbSaO2 desaturations below 90% (HbSaO2 nadir 68%) were detected during nocturnal polysomnography. The HbSaO2 desaturations occurred during brief central apneas or hypopneas, mainly during REM sleep, the apnoea hypopnea index being within normal limits in all cases. The data suggest that nocturnal polysomnography can detect otherwise clinically silent hypoxemia in SMA patients without any predisposing factor to sleep-disordered breathing other than their illness and still showing normal blood-gas tensions during wakefulness. Further studies are needed to determine the long-term evolution and the prognostic significance of nocturnal hypoxemia in these patients.

Key Words

Sleep breathing spinal muscular atrophy 

Sommario

Sono illustrati i risultati di una indagine clinica e polisonnografica sugli aspetti del sonno notturno e della respirazione durante sonno in 8 pazienti affetti da Amiotrofia Spinale (4 maschi, 4 femmine, di età compresa tra 10 e 37 anni) e che mostravano in veglia una sindrome restrittiva toracica ma valori emogasanalitici ancora nei limiti della norma.

Nessun paziente riportava disturbi soggettivi del sonno tra quelli suggestivi di un sottostante disordine respiratorio sonno-relato. In 4 pazienti sono state documentate ricorrenti desaturazioni emoglobiniche di O2 sotto il 90% (valore minimo osservato 68%) nel corso della polisonnografia notturna, in relazione ad apnee o ipopnee non ostruttive, principalmente durante sonno REM. L'Indice di Apnea/Ipopnea è risultato essere nei limiti in tutti i pazienti.

I dati suggeriscono che una iniziale tendenza alla desaturazione di O2 sonno relata può essere presente in pazienti con Amiotrofia Spinale senza apparenti disturbi clinici del sonno e che mostrano ancora un quadro di compenso emogasanalitico in veglia.

L'evoluzione, la rilevanza clinica e dunque il significato prognostico di tali patterns di ipossiemia notturna andranno verificati in studi clinici e polisonnografici più estesi e con sviluppo longitudinale.

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Copyright information

© Masson Italia Periodici S.r.l. 1993

Authors and Affiliations

  • Manni R. 
    • 1
  • Cerveri I. 
    • 2
  • Ottolini A. 
    • 3
  • Fanfulla F. 
    • 2
  • Zoia M. C. 
    • 2
  • Lanzi G. 
    • 3
  • Tartara A. 
    • 1
  1. 1.Clinica Neurologica dell'Università di PaviaPaviaItalia
  2. 2.Istituto delle Malattie Respiratorie dell' Università di PaviaPaviaItalia
  3. 3.Divisione di Neurologia InfantileIstituto C. MondinoPaviaItalia

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