Benign intracranial hypertension during corticosteroid therapy for idiopathic ulcerative colitis: another indication for cyclosporine?

To the Editor: The benign intracranial hypertension (BIH), or pseudotumor cerebri, is a rare clinical condition with unknown etiology, described as the increase of intracranial pressure with a normal composition of cerebrospinal fluid (CSF) in the absence of a space occupying intracranial lesion. It presents itself with headaches, blurred vision and, in progressive cases, loss of visual acuity. BIH is more prevalent among fertile women and most commonly associated with obesity and the use of some medicines, such as corticosteroids. The latter situation can occur even if on reduction or withdrawal of corticosteroid therapy. A 34-year-old Brazilian woman have been suffering from ulcerative pancolitis for 7 years, while using azathioprine (100 mg/PO/day), sulphasalazine (3.5 g/PO/day), and 5-ASA enema (3 g/night). In spite of the treatment, the patient was having relapses frequently. In July of 2003, she experienced abdominal cramping pain, significant rectal bleeding, loss of weight and anorexia. She was given prednisolone (60 mg/PO/day) that led to fast settlement of disease. Ten days after the symptoms control, a reduction of prednisolone was started at 5 mg per week. Thirty days after the beginning of corticosteroid therapy, when prednisolone daily (20 mg/PO) was used, she developed blurred vision and dairy orbitary headache of moderate intensity, becoming more intense in the evening. The headache was mildly relieved with analgesics. She sought medical attention, and underwent neurologic examination, which was normal. An ophthalmologic evaluation identified bilateral papilloedema. The patient was hospitalized. Cranial CT and MRI detected an empty sella and excluded both the presence of a mass in the brain and venous thrombosis. Lumbar puncture revealed a cerebrospinal fluid (CSF) opening pressure of 280 mmH2O. Glucose, protein concentration, red blood cells, and polymorphonuclear leukocyte counts were normal. Bacterial, fungal, and tuberculosis cultures were negative. A diagnosis of benign intracranial hypertension was made. In this occasion, enough CSF was removed to decrease the closing pressure to about 150 mmH2O. Additionally, acetazolamide was introduced and prednisolone was discontinued and stopped 2 weeks later. However, the patient experienced a flare of ulcerative colitis with severe rectal bleeding, despite the use of sulphasalazine, 5-ASA enema and azathioprine. Intravenous cyclosporine was used (2 mg/kg/day) for 7 days, and colitis symptoms disappeared. Cyclosporine, 6 mg/PO/day, use was continued for 2 months then stopped. In August of 2003 she was asymptomatic without papilloedema, and intracranial pressure normal. The patient is now on azathioprine (150 mg / day) sulphasalazine (3,5 g / PO / day) and 5-ASA enema (3 times a week). BIH is an uncommon condition characterized, basically, by headache and blurred vision. It occurs, generally, due to medicines such as corticosteroids, tetracycline, human growth hormone. It is essential that the physician be alert to the BIH symptoms when prescribing steroids to treat ulcerative colitis mainly in young women, because, although it has excellent results in most part of patients, the occurrence of BIH can lead to permanent visual loss. BIH diagnosis is based on a careful interview, emphasizing the previous disease and the medication used, a normal neurologic examination and the finding of bilateral papilloedema. Radiologic tests, like CT and MRI, are important to exclude a space occupying intracranial lesion, while lumbar puncture and CSF analysis reveal increase in intracranial pressure with a normal composition of CSF. BIH treatment is similar to that found in other situations consisting of lumbar puncture and CSF drainage to reduce the intracranial pressure. Acetazolamide and other diuretics may also be tried. Although the use of aminosalycilates is the major cause of headache in patients suffering from ulcerative colitis, in those patients with persistent headache, mainly during systemic corticosteroid therapy, an evaluation should be done to exclude BIH. The treatment of severe relapse of colitis in a patient who has had one episode of steroid related BIH remains a dilemma, because the reintroduction of the corticosteroid can develop another one of BIH, and there is no reference in the literature about how to proceed in these circunstances. We suggest that in the presence of BIH related to corticosteroids, in those patients with severe colitis, rescue therapy with cyclosporine can be an option that will allow rapid discontinuation of the steroid thereby contributing to regression of BIH symptoms and avoiding the complications indicated in our case study.