Treatment of sleep central apnea with non-invasive mechanical ventilation with 2 levels of positive pressure (bilevel) in a patient with myotonic dystrophy type 1.
We are reporting a case of a 29 year-old female with diagnosis of myotonic dystrophy type 1 (Steinert's disease) with excessive daytime sleepiness, muscle fatigue, snoring, frequent arousals, non-restorative sleep, and witnessed apneas. Pulmonary function tests revealed a mild decrease of forced vital capacity. Nocturnal polysomnography showed an increase of apnea/hypopnea index (85.9 events/h), mainly of central type (236), minimal oxygen saturation of 72%, and end-tidal carbon dioxide values that varied from 45 to 53 mmHg. Bi-level positive airway pressure titration was initiated at an inspiratory pressure (IPAP) of 8 and an expiratory pressure (EPAP) of 4 cm H2O. IPAP was then gradually increased to eliminate respiratory events and improve oxygen saturation. An IPAP of 12cm H20 and an EPAP of 4cm H2O eliminated all respiratory events, and the oxygen saturation remained above 90%. Bi-level positive airway pressure treatment at spontaneous/timed mode showed an improvement in snoring, apneas, and Epworth sleepiness scale decreased from 20 to 10. This case illustrates the beneficial effects of Bi-level positive airway pressure support in central sleep apnea syndrome of a patient with myotonic dystrophy type 1.
Setor de Tratamento de Doenças Neuromusculares - Departamento de Psicobiologia, Universidade Federal de Sao Paulo UNIFESP, Brazil.
Ricardo Tera
RT
Akamine
Setor de Tratamento de Doenças Neuromusculares - Departamento de Psicobiologia, Universidade Federal de Sao Paulo UNIFESP, Brazil.
Luís Fernando
LF
Grossklauss
Setor de Tratamento de Doenças Neuromusculares - Departamento de Psicobiologia, Universidade Federal de Sao Paulo UNIFESP, Brazil.
Gustavo Antonio
GA
Moreira
Setor de Tratamento de Doenças Neuromusculares - Departamento de Psicobiologia, Universidade Federal de Sao Paulo UNIFESP, Brazil.
Marcia
M
Pradella-Hallinan
Setor de Tratamento de Doenças Neuromusculares - Departamento de Psicobiologia, Universidade Federal de Sao Paulo UNIFESP, Brazil.
Marco Antônio
MA
Chiéia
Setor de Tratamento de Doenças Neuromusculares - Departamento de Psicobiologia, Universidade Federal de Sao Paulo UNIFESP, Brazil.
Denis
D
Mesquita
Setor de Tratamento de Doenças Neuromusculares - Departamento de Psicobiologia, Universidade Federal de Sao Paulo UNIFESP, Brazil.
Acary Souza
AS
Bulle Oliveira
Setor de Tratamento de Doenças Neuromusculares - Departamento de Psicobiologia, Universidade Federal de Sao Paulo UNIFESP, Brazil.
Sergio
S
Tufik
Bi-level positive airway pressure; Central sleep apnea syndrome; Myotonic dystrophy type 1