A case of quadriparesis with type 2 respiratory failure: A diagnostic conundrum

Abstract

Background: Acute flaccid paralysis with respiratory failure is commonly attributed to Guillain-Barré Syndrome; however, other causes should be kept in mind.

Case Report: A 41-year-old woman presented with acute ascending weakness, areflexia, and respiratory failure requiring intubation. Initial clinical suspicion was Guillain–Barré Syndrome. However, Laboratory evaluation revealed severe hypokalemia (serum potassium 1.1 mEq/L) and normal anion gap metabolic acidosis (pH 6.9, HCO₃⁻ 14.6 mmol/L). Urine anion gap was positive, consistent with distal renal tubular acidosis. MRI showed pontine demyelination. In view of suspected combined central and peripheral demyelination syndrome, autoimmune workup was done, which revealed ANA, anti-SSA, anti-SSB, and anti-RNP positivity. The patient improved with intravenous potassium replacement, IVIG, Steroids and immunosuppressants. She was extubated on hospital day 4.

Why should an Emergency Physician be aware of this? Autoimmune polyradiculoneuropathy secondary to connective tissue disorder is a reversible cause of respiratory failure that can mimic Guillain–Barré Syndrome and hypokalemic periodic paralysis. Early recognition with prompt initiation of immunosuppression can be lifesaving.