To report a rare case of AIDP secondary to Waldenström’s Macroglobulinemia

Acute inflammatory demyelinating polyneuroradiculopathy (AIDP) is a rare complication of Waldenström’s Macroglobulinemia (WM). Typical findings are a slowly progressive neuropathy, thought to be mediated through autoantibodies. Anti-myelin-associated glycoprotein (MAG) antibodies are found in 50% of patients with WM and neuropathy. A rapidly progressing course can result in fatal respiratory depression and cardiac arrhythmias.

This is a 77 year-old male with chronic WM presenting with rapidly progressive appendicular weakness over several days. Neurologic exam was notable for diffuse areflexia, appendicular weakness (MRC Grade 1-2 of 5), and globally reduced sensation. Initially, no bulbar weakness was noted but as symptoms progressed patient required intubation on hospital day #2. MRI brain was unremarkable, however, MRI lumbosacral spine showed enhancement of nerve. Plasma levels of Anti-MAG IgM antibody (1:102400) were substantially elevated. CSF analysis showed elevated protein (55), glucose (40), and 76 nucleated cells (70% lymphocytes). Electromyography and nerve conduction studies revealed temporal dispersion, reduced motor unit recruitment and rapid firing in all muscles. Patient was treated with intravenous immunoglobulins (IVIG) 2g/kg over 5 days followed by plasma exchange when IVIG had no effect. The patient’s hospital course was complicated by severe autonomic instability with periods of bradycardia necessitating atropine and percutaneous pacing. The patient also suffered pneumonia and eventually required tracheostomy and feeding tube placement. After a prolonged ICU course, the family eventually decided to change code status to do not resuscitate and patient expired on Hospital Day #19 following an episode of bradycardia refractory to atropine.