Key result
Nasal intermittent pressure ventilation provided a good clinical response in a 49-year-old woman presenting with hypercapnia due to adult-onset nemaline myopathy.
Case Report (n=1)
Adult-onset nemaline myopathy can present as respiratory insufficiency and may respond well to nasal intermittent pressure ventilation.
May support NIPPV consideration in rare hypercapnic adult-onset nemaline myopathy; leaves open efficacy and generalizability pending prospective data.
A 49-year-old woman was admitted to the hospital for hypercapnia. Pulmonary function testing showed small lung volumes without parenchymal lung disease. Muscle enzyme levels were normal and the EMG was nonspecific. Finally, muscle biopsy revealed abundant nemaline bodies characteristic of nemaline myopathy. Nasal intermittent pressure ventilation was started with a preset pressure ventilator during sleeping hours with a good response.
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Falgà-Tirado et al. (2009) conducted a case report in Adult-onset nemaline myopathy (n=1). Nasal intermittent pressure ventilation was evaluated. Nasal intermittent pressure ventilation provided a good clinical response in a 49-year-old woman presenting with hypercapnia due to adult-onset nemaline myopathy.
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