A 40 year old woman presented with a short history of acute onset of breathlessness to the ER of our hospital and after initial evaluation for acute pulmonary embolism which was ruled out after carrying out the appropriate investigations, she was diagnosed to be afflicted with syringomyelia based on her neurological symptoms and clinical findings, which was confirmed by doing an MRI scan, which was her basic diagnosis that was complicated by acute hypercapnic respiratory failure. This case is being reported to highlight syringomyelia as an unusual cause of acute respiratory failure, which manifested clinically in this patient as its first presentation and the underlying neurological diagnosis has been found to be present in very few reported cases (less than 0.01% of case reports) in the available literature as the basic disease in the absence of its classical presenting features. Problems associated with acute respiratory failure in the setting of syringomyelia are discussed.
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