Abstract
Background Idiopathic bilateral phrenic neuropathy leading to bilateral diaphragmatic paralysis is a rare cause of respiratory failure. Patients with this condition often present with orthopnoea, dyspnoea on exertion, and sleep disturbance. Prognosis is usually poor.
Case Description A 69-year-old Caucasian female presented with acute onset of chest pain and dyspnoea. Initial investigations ruled out acute myocardial infarction or pulmonary embolus. Three days later, patient was found obtunded with severe type 2 respiratory failure, which required urgent intubation. She was extubated on the same day and transitioned to non-invasive ventilation (NIV). Day 3 post extubation, her sniff test revealed no movement of the bilateral hemidiaphragms. She was urgently re-intubated. There was no history of pain, prodromal illness, trauma, COVID infection or vaccinations. Her past medical history is significant for mild COPD and hypertension. Her neurological examination was unremarkable. Her CSF studies were unremarkable, and so was her MRI Brain and cervical spine result. Nerve conduction studies had shown absent phrenic motor response and normal upper and lower limb motor and sensory study. Patient received 5 days of plasma exchange. She was successfully extubated and transitioned to NIV afterward. Nine days later, she also had right-sided diaphragmatic plication. Patient had excellent clinical response and she was weaned off NIV gradually.
Conclusion There is no established guideline for treatment of idiopathic bilateral phrenic neuropathy. Supportive therapy with NIV is the mainstay of treatment. This case highlights plasma exchange in conjunction to unilateral diaphragmatic plication as an effective treatment for idiopathic bilateral phrenic neuropathy.
