Abstract
One of the most common progressive neurodegenerative diseases is amyotrophic lateral sclerosis (ALS). It is diagnosed in the 5th to 7th decade and is common in males. ALS in women of childbearing age and coexisting pregnancy is uncommon. We would like to describe the first reported case in which non-invasive ventilation (NIV) was acutely initiated in a pregnant patient with ALS
Case report
A 25-year-old patient, G2P1, had complaints of speech, swallowing difficulty and tiredness that she credited to her pregnancy. She later developed weakness in her right arm and sought consultation. Physical exam revealed myopathic facies, dysarthria, tongue atrophy and fasciculation, weakness in neck flexor and right arm muscles. After two months ALS was diagnosed. Fetal development was uncomplicated. At her first visit to the respiratory department at 32 weeks of gestation, she had no pulmonary complaints. Examination revealed weak cough capacity, peripheral saturation 98% (room air) and decreased forced vital capacity (42% of predicted). Transcutaneous CO2 and O2 measurement was normal during sleep. Four days later she was admitted with progressive dyspnea, orthopnea and respiratory exhaustion. NIV with oronasal mask was acutely initiated and well tolerated despite poor bulbar function. Due to rapid deterioration a caesarian section was performed under NIV and spinal anesthesia. Intubation and total anesthesia with was averted. A healthy male baby was born. Postoperative NIV could be weaned slowly to only nighttime use and she was discharged with domiciliary NIV.
Conclusion
Acute NIV in pregnant ALS patient with respiratory distress could be safely initiated.
- © 2014 ERS
