Eyes and Bowels Bottled Up - Video

The procedure was a chest CT that revealed a thymic mass. Substernal surgery extirpated a stage B3 thymoma. Myasthenia, usually with thymoma, can rarely affect the autonomic nervous system, causing intestinal pseudo-obstruction. In a review of 12 patients with myasthenia and dysautonomia, 10 had gastrointestinal dysmotility, 6 had cardiovascular symptoms and 2 had pupils fixed to light.(1) Eight had thymoma, 3 with positive anti-ganglionic antibodies. Two additional cases of GI dysmotility, thymoma, and myasthenia have since been reported, (2,3) one with negative skeletal acetylcholine receptor antibodies, the other with moderately positive skeletal antibodies and borderline elevated anti-ganglionic antibodies, which most resembles our case. We speculate that the intestinal pseudo-obstruction in our case was related either to occult anti-ganglionic antibodies (this test is only 50% sensitive in myasthenic dysautonomia) or a high titer of anti-skeletal muscle antibodies cross-reacting with enteric plexus receptors, given a 60% sequence homology shared by the extracellular domains of the two distinct receptor types.(4) Prior to thymectomy, our patient had received prednisone, intravenous immunoglobulin, plasmapheresis, and pyridostigmine for 6 weeks, but eye movements and ptosis improved only minimally. Two weeks after thymectomy, these eye signs disappeared. The tumor showed extra-thymic involvement, so he underwent radiotherapy of 50.4 Gy. On maintenance mycophenolate mofetil 1gm/day, he has remained asymptomatic for 18 months.