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Idiopathic Bilateral Diaphragmatic Palsy FREE TO VIEW

Sravanthi Nandavaram, MBBS; Robert Lenox, MD; Sidharth Jogani, MD
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SUNY Upstate Medical University, Syracuse, NY

Chest. 2013;144(4_MeetingAbstracts):977A. doi:10.1378/chest.1705051
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SESSION TITLE: Miscellaneous Student/Resident Case Report Posters III

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Respiratory insufficiency and failure due to bilateral diaphragmatic paralysis is usually seen in systemic conditions affecting the neural axis. However, Varicella Zoster causing isolated bilateral diaphragmatic paralysis is rare and only a few cases have been reported to date.

CASE PRESENTATION: A 57 year old female with hypertension and hyperlipidemia presented with acute onset of shortness of breath for 4 days, which was worse on lying flat, in the absence of other associated symptoms. Physical examination revealed oxygen saturation of 96% on room air while sitting up and 83% in supine position with respiratory distress and paradoxical inward abdominal movement on inspiration. Complete blood count and comprehensive metabolic panel were normal. Chest X-ray showed bibasilar atelectasis and flattening of diaphragm. CTA of thorax ruled out pulmonary embolism. Echocardiogram showed normal cardiac function. PFTs showed FEV1/FVC of 58% and a dramatic drop in FEV1 from 1.49 L to 0.36 L (76% drop) and FVC from 1.9 L to 0.54 L (72% drop) from upright to supine position. Maximal Inspiratory Pressure (MIP) was -30 cm H2O. Surface EMG as an approximation of phrenic nerve function was normal. Imaging of the CNS showed no abnormalities. Serum inflammatory, rheumatologic markers, tests for herpes simplex, Lyme’s, Ehrlichiosis and myasthenia were negative, except for IgM varicella antibodies which was positive. Hence, though unusual, acute varicella zoster infection was presumed to be the cause of diaphragmatic paralysis and a trial of valacyclovir was initiated in adjunct to theophylline and supine respiratory distress was relieved with continuous positive airway pressure. Gradually symptoms improved and repeat PFTs after 12 weeks showed an increase in TLC to 4.32 L and MIP to -55 cm H2O.

DISCUSSION: Bilateral diaphragmatic paralysis is caused by diseases affecting the spinal cord, motor neurons and neuromuscular junction, myopathy or can be idiopathic. Extent of work up for diagnosis depends on the clinical situation. With classic symptoms and signs, spirometry and inspiratory pressure would suffice. If in question, use of transdiaphragmatic pressures and diaphragmatic EMG may be necessary. Affected patients would benefit from ventilatory support and the evidence of PCO2 higher than 48 or maximal inspiratory pressure less than -60 cms of H2O should necessitate the initiation of ventilatory support. Antiviral therapy with Valacyclovir in patients with positive serology has been shown to aid recovery. Diaphragmatic pacing is an option in patients with high quadriplegia and if ventilatory support fails.

CONCLUSIONS: Early diagnosis of diaphragmatic paralysis helps prevent overt respiratory failure, as is identifying the cause helps with the treatment modality.

Reference #1: Kumar N, Folger WN, Bolton CF. Mayo Clin Proc. 2004;79(12):1563.

Reference #2: Crausman RS, Summerhill EM, McCool FD Lung. 2009;187(3):153.

Reference #3: Celli BR, Rassulo J, Corral R Am Rev Respir Dis. 1987;136(5):1276

DISCLOSURE: The following authors have nothing to disclose: Sravanthi Nandavaram, Robert Lenox, Sidharth Jogani

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