Year: 2013
Type: article
Abstract: To the Editor:
Respiratory dysfunction from multiple sclerosis (MS) is most commonly of insidious onset and related to progressive respiratory muscle weakness [1]. Disorders of respiratory muscle coordination are poorly recognised and challenging to diagnose. We present a case of a patient with MS and no clear muscle weakness whose dyspnoea was associated with a disorder of respiratory muscle control. The potential utility of oesophageal manometry for diagnosis of unexplained dyspnoea is highlighted.
A 54-year-old female with relapsing-remitting MS presented with 2 weeks of dyspnoea and orthopnoea. She was ambulatory with a low extended disability score of 1.5.
6 months earlier she started cyclophosphamide for disease progression recognised by magnetic resonance imaging (MRI). A week after her last infusion she developed severe dyspnoea and orthopnoea. She had no other symptoms. Her vital signs were normal. Her laboratory tests and arterial blood gas were unremarkable. Chest computed tomography showed no pulmonary parenchymal disease. Echocardiography was unremarkable. Pulmonary function testing (PFTs) demonstrated severe restriction, with a 14% lower vital capacity in the supine position.
Her dyspnoea and PFTs improved over the following 1–2 months, but she did not return to her baseline level of pulmonary function. Electromyography (EMG) with phrenic nerve conduction studies demonstrated no abnormalities. MRI was prohibited by her orthopnoea. Previously, MRI demonstrated extensive demyelination in the brain and brainstem that was asymptomatic.
Oesophageal manometry was performed to exclude the possibility of bilateral diaphragmatic dysfunction and to elucidate her unexplained dyspnoea. A balloon-catheter was inserted via the nose to position the balloon in the distal oesophagus to estimate pleural pressure. Repeated attempts to perform a forced vital capacity manoeuvre consistently demonstrated fluctuating exhalations, as evidenced by the saw tooth pattern of the flow volume curve (fig. …
Source: European Respiratory Journal
Language: English
Subfield: Pathology and Forensic Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W2139631492