Data di Pubblicazione:
2009
Citazione:
Restrictive respiratory sindrome in DM2 patients / A. Zanolini, V. Sansone, B. Fossati, M.C. Panzeri, C. Baldessari, S.M. Donghi, A. Poltronieri, V. Valneti, G. Meola. ((Intervento presentato al 19. convegno Meeting of the European Neurological Society- ENS tenutosi a Milan nel 2009.
Abstract:
Objectives: Respiratory involvement in myotonic dystrophy type 1
(DM1) is frequent. Respiratory insufficiency is, together with cardiac
failure, the main cause of death in these patients. Reports on respiratory
involvement in DM2 are limited. The aims of our study were to determine
the frequency and severity of respiratory involvement and sleep
apnoea in patients with DM2 compared to DM1.
Methods: 20 patients with genetically determined DM2 (mean age
64.3 ± 8) and 40 patients with moderately severe age-matched DM1
(mean age 38.5 ± 11) were subjected to muscle strength assessment
and: (1) subjective assessment of excessive daytime sleepiness (Epworth
Sleepiness Scale-ESS); (2) spirometry and nocturnal
polygraphy (PSG).
Results: (1) 4 of 20 (20%) patients with DM2 and 12 of 40 (30%)
patients with DM1 complained of daytime sleepiness; (2) 5 of 20
(25%) patients with DM2 and 14 patients with DM1 (35%) showed a
restrictive respiratory impairment on respiratory function tests. Indication
to nocturnal assisted ventilation (Bi-level) either due to
nocturnal desaturation or sleep apnoeas was identified in 30% patients
with DM1 subjected to PSG compared to 10% of patients with DM2
subjected to PSG.
Conclusions: Although preliminary, our results indicate that
respiratory involvement in DM2 is present and needs to be considered
in patients with DM2 just like in DM1, because of the potentially
treatable nature of the associated symptoms. Whether severity of
respiratory involvement correlates with muscle impairment (MMRC)
and disease duration needs to be further explored on a larger number
of patients.
Tipologia IRIS:
14 - Intervento a convegno non pubblicato
Keywords:
respiratory sindrome ; DM2 ; myotonic dystrophy type 2
Elenco autori:
A. Zanolini, V. Sansone, B. Fossati, M.C. Panzeri, C. Baldessari, S.M. Donghi, A. Poltronieri, V. Valneti, G. Meola
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