Acute-Onset Oculobulbar and Proximal Weakness in a Patient With Crohn’s Disease

Abstract

BRIEF HISTORY AND PHYSICAL EXAMINATIONThe patient is a 54-yr-old woman who was admitted to a community hospital with recurrent small bowel obstruction and 3 days of right eye ptosis, mild dysarthria, and mild dyspnea.A medical history included Crohn's disease on chronic immunosuppression, diverting colostomy, proctocolectomy, short bowel syndrome, chronic pain, and osteoporosis. Functionally, she was modified independent with ambulation, using single-point cane intermittently and independent with activities of daily living. She had a caregiver for assistance with instrumental activities of daily living because of chronic pain and fatigue. She had no family history of neurologic disease.At the community hospital, she was started on intravenous methylprednisolone for small bowel obstruction. Over the next several days, she developed progressive, proximal greater than distal weakness in the bilateral upper limbs and neck, bilateral ptosis with diplopia, facial weakness, hypophonia, worsening dysarthria, and dysphagia. Intravenous immunoglobulin was initiated for presumed myasthenia gravis crisis without significant improvement. She also received pyridostigmine on hospital day 4 without improvement in neurologic symptoms. She ultimately required intubation and tracheostomy for acute hypoxemic respiratory failure on hospital day 8 and was transferred to our tertiary care hospital on hospital day 12.Physical examination on hospital day 13 revealed the following: Cognition: oriented, able to write responses to questions Speech: aphonic secondary to tracheostomy and mechanical ventilation Cranial nerves: visual fields full. Extraocular eye movements intact. Severe bilateral ptosis, worsened with sustained upward gaze. Facial weakness with puffing cheeks.