Bowel and Brain Symptoms: What's the Connection?

Stephen Paget, MD


January 26, 2016

Patient Profile

A 50-year-old woman presented for evaluation and treatment of granulomatosis with polyangiitis (also known as "Wegener granulomatosis") of 5 months' duration.

Since 20 years of age, the patient has had ulcerative colitis presenting with diarrhea and involving the entire colon, though primarily the lower. She was treated with sulfasalazine in the past, as well as intermittent courses of prednisone for flare-ups that would occur approximately twice a year. She had been hospitalized for her worst flare-up 7 years before I saw her; at that time, she had presented with anal abscess with fistula formation, which eventually responded to intravenous steroids and surgery and also raised the possibility of Crohn disease. Her last colonoscopy, performed 5 months before I saw her, was negative. She was doing well on mesalamine, with no abdominal symptoms.

Five months ago, the patient developed a temperature of 101°F, fatigue, general pain and swelling, redness, and pain in both eyes due to uveitis. She was admitted to the hospital for 3 weeks for evaluation of severe headache. A spinal tap showed changes consistent with meningitis. MRI of the brain showed dural enhancement.

One week into the hospitalization, the patient developed decreased hearing in her right ear with tinnitus and, soon thereafter, severe vertigo. Her sedimentation rate was over 100 mm/h, and her C-reactive protein level was 177 mg/L. The cerebrospinal fluid protein level was elevated at 77 mg/dL, with 39 white cells, mostly lymphocytes. MRI of the brain showed mild mucosal thickening within the ethmoid, sphenoid, and frontal sinuses. During the last 3 days of the hospitalization, the patient was given methylprednisolone 1000 mg a day for 3 days, which led to a marked improvement in her headache but not her hearing loss or vertigo.

After discharge, a repeat MRI showed clearing of the dural enhancement, but there was a persistent minimal focus of either vascular origin or left meningeal enhancement in the posterior superior left parietal lobe. An ear, nose, and throat evaluation raised the possibility of a diagnosis of a vasculitis as the cause of the patient's hearing loss, tinnitus, and vertigo.

The patient had the following negative tests: antinuclear antibody, rheumatoid factor, angiotensin-converting enzyme, Lyme test, anti-Sjögren syndrome A and B, anticardiolipin antibodies, and celiac autoantibodies. She did have a strongly positive antibody to proteinase 3, which at the time was thought to confirm the diagnosis of granulomatosis with polyangiitis.

Two weeks after discharge, the patient developed left-eye uveitis, which was treated with topical steroids. The patient was sent to me to decide whether she should be started on rituximab or cyclophosphamide.


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