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Drug Induced Intracranial Hypertension Associated With Sulphasalazine Treatment

Authors:
Eser Sevgi
Eser Sevgi
Eser Sevgi
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Gul Yalcin Cakmakli at Hacettepe University
Gul Yalcin Cakmakli
Tulay Kansu at Hacettepe University
Tulay Kansu
Kubilay Varli
Kubilay Varli
Kubilay Varli
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Abstract and Figures

A 25-year-old female patient developed headache and papilledema under sulphasalazine treatment for ulcerative colitis. The patient met the International Headache Society's criteria for idiopathic intracranial hypertension. Sulphasalazine was discontinued and the patient was given azathioprine for ulcerative colitis and acetazolamide for intracranial hypertension. Three weeks later, her examination was normal and lumbar puncture revealed an opening pressure of 180-mm H(2)O. Sulphasalazine is a product of 5 aminosalicylate (5 ASA) and there seems to be a relationship between the administration of sulphasalazine and the onset of intracranial hypertension symptoms. Early diagnosis of intracranial hypertension is important in patients with ulcerative colitis receiving 5 ASA treatment to prevent visual complications.
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cause of headache in patients of this age group, as the
condition usually presents at a younger age. The mean age
for presentation with headache in one series was 23.5 ⫾9.3
years.8She was managed conservatively with analgesics.
CSF examination would have confirmed the diagnosis, but
this was not done as her symptoms settled with conservative
management.
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Drug Induced Intracranial Hypertension Associated With
Sulphasalazine Treatment
Eser Sevgi,MD;Gul Yalcin,MD;Tulay Kansu,MD;Kubilay Varli,MD
A 25-year-old female patient developed headache and papilledema under sulphasalazine treatment for ulcerative colitis.
The patient met the International Headache Society’s criteria for idiopathic intracranial hypertension. Sulphasalazine was
discontinued and the patient was given azathioprine for ulcerative colitis and acetazolamide for intracranial hypertension.
Three weeks later, her examination was normal and lumbar puncture revealed an opening pressure of 180-mm H2O. Sulphasala-
zine is a product of 5 aminosalicylate (5 ASA) and there seems to be a relationship between the administration of sulphasalazine
and the onset of intracranial hypertension symptoms. Early diagnosis of intracranial hypertension is important in patients with
ulcerative colitis receiving 5 ASA treatment to prevent visual complications.
Key words: idiopathic intracranial hypertension,sulphasalazine,ulcerative colitis
INTRODUCTION
Idiopathic intracranial hypertension (IIH) is a syn-
drome of raised intracranial pressure in the absence of an
intracranial mass lesion or cerebrospinal outflow obstruc-
tion. IIH is a secondary headache disorder characterized by
headaches and visual symptoms.1It most frequently occurs
in obese women of childbearing age; however, many sec-
ondary causes exist and it may affect children, men,and slim
individuals. Prompt recognition, evaluation, and treatment
are needed to prevent permanent visual loss. With the
exception of papilledema and sixth nerve palsy, the neuro-
logical examination is normal. Other cranial nerve palsies
may occur occasionally.2Although the cause is largely
unknown, several medications have been associated with
IIH.3Two cases of IIH reported in the literature have been
associated with mesalasine treatment, which is a product of
5 aminosalicylate (5 ASA).4,5 Sulfapyridine bound to 5ASA
is named as sulphasalazine. Here, we present a woman who
From the Hacettepe University Faculty of Medicine, Depart-
ment of Neurology, Ankara, Turkey.
Address all correspondence to Dr. Eser Basak Sevgi, Hac-
ettepe University Hospitals, Department of Neurology, 06100
Sıhhıye, Ankara, Turkey.
Accepted for publication August 28, 2007. Conflict of Interest: None
296 February 2008
developed IIH under sulphasalazine treatment for ulcer-
ative colitis.
CASE REPORT
A 25-year-old woman patient was admitted to the hos-
pital with the complaint of headache and blurred vision. She
had been put on sulphasalazine treatment (2000 mg per day)
for ulcerative colitis 3 weeks previously. Neurological
examination revealed 20/200 visual acuity on both sides with
the near card, peripheral constriction in visual fields, bilat-
eral papilledema (Fig. A), abduction deficit, and peripheral
facial palsy on the left.The motor and sensory examinations
were normal with no meningeal signs.A clinical diagnosis of
raised intracranial pressure was made. The patient was not
obese with a body mass index of 23.4, and she did not
report any recent weight gain either.The hemogram,eryth-
rocyte sedimentation rate, renal, hepatic and thyroid func-
tion tests, blood Venereal Disease Research Laboratory
titer, serum vitamin A level, antinuclear antibody titer, uri-
nalysis, and chest x-ray were all within normal limits.
Cranial magnetic resonance imaging and magnetic reso-
nance venography were found to be normal. Lumbar punc-
ture (LP) revealed an opening pressure of 300-mm H2O.
The cerebrospinal fluid (CSF) was found to be acellular
with a protein level of 19 mg/dL. Bacterial and viral
markers in CSF were negative. She was not under any con-
current medications or hormone therapy other than sul-
phasalazine treatment. Diagnosis of ulcerative colitis was
confirmed by colonoscopy and intestinal biopsy. The
patient met the International Headache Society’s criteria
for IIH. The disease was associated with sulphasalazine
treatment and sulphasalazine was accordingly discontin-
ued in this patient. The patient continued with azathio-
prine for ulcerative colitis and acetazolamide 750 mg/day
therapy for IIH.
Three weeks later, LP revealed an opening pressure of
180-mm H2O.All of her symptoms together with sixth and
seventh nerve palsies and papilledema (Fig. B) had
improved. At her 1-year follow-up, the neurological exami-
nation was completely normal and her ulcerative colitis was
also under control with azathioprine treatment so rechal-
lenge with sulphasalazine was not found appropriate as for
the ethical issues.
DISCUSSION
Idiopathic intracranial hypertension associated with
mesalasine treatment has been reported in 2 cases in the
literature.4,5 Rosa et al reported a patient with ulcerative
colitis and IIH due to mesalasine treatment. IIH was not
considered initially and the diagnosis was made 3 years
later following serious visual loss. After discontinuing
mesalasine, the patient improved but showed recurrence
A
B
Figure.—(A) Fully developed papilledema at time of diagnosis. (B) Resolution of papilledema 3 weeks later.
Headache 297
when the medication was restarted.4In the second patient,
an 11-year-old girl with Crohn’s disease, a causal relation-
ship between mesalasine treatment and development of
IIH was observed.5
Headache is a known side effect of sulphasalazine.
Improvement of headaches has been reported with lower
doses of sulphasalazine in ulcerative colitis patients.6The
headaches may be related to IIH but IIH due to sulphasala-
zine treatment has not been reported previously. The
mechanism of drug induced intracranial hypertension is
unknown. Our patient had sixth and seventh cranial nerve
palsies on the left side. Although sixth nerve palsy is
common, oculomotor, trochlear, trigeminal, and facial nerve
palsies have been reported but are very rare in IIH.2The
pathophysiology of cranial nerve palsies accompanying IIH
is poorly understood, but in most cases it probably repre-
sents a nonspecific pressure-related phenomenon.2
In conclusion, there is a likely relationship between the
administration of sulphasalazine and the onset of IIH symp-
toms. This case provides further evidence for the risk of
developing IIH during 5 ASA treatment. IIH may lead to
permanent visual loss.Early diagnosis of IIH is important in
patients with ulcerative colitis receiving 5 ASA treatment to
prevent visual complications.
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1. Headache Classification Subcommittee of the International
Headache Society. The international classification of head-
ache disorders: 2nd edition. Cephalalgia. 2004;24(Suppl.
1):9-160.
2. Capobianco DJ, Brazis PW, Cheshire WP, Idiopathic intrac-
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benign intracranial hypertension in a patient with ulcerative
colitis. Am J Ophthalmol. 2003;136:212-213.
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298 February 2008
... Rosa et al demonstrated that re-challenging mesalamine due to severe ulcerative colitis caused headache and papilledema again [3]. Sevgi et al did not re-challenge their patient with sulphasalazine due to stability on azathioprine [5]. Rottembourg et al's case demonstrated a temporal relation of symptoms of IIH related to starting and discontinuation of mesalamine [4]. ...
... Rottembourg et al's case demonstrated a temporal relation of symptoms of IIH related to starting and discontinuation of mesalamine [4]. Please refer to Table 1 for a description of the course of the syndrome for each case [3][4][5]. ...
... Age, sex Presence of papilledema Associated neurologic signs and symptoms Clinical outcome Rosa et al [3] 23, female Yes Headache, blurry vision Visual acuity did not return to baseline despite discontinuation Rottembourg et al [4] 11, female Yes Headache Returned to baseline after discontinuation Sevgi et al [5] 25, female Yes Headache, blurry vision, bilateral abducens nerve palsies, lower facial nerve palsy on the left Returned to baseline after discontinuation ...
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... In the aetiology, drugs such as corticosteroids, oral contraceptives, minocycline, tetracycline, sulfasalazine, vasculitis such as Behcet's Disease and systemic lupus erythematosis, arteriovenous malformations, sleep disturbations, extracranial venous hypertension secondary to cardiac septal defect, uremia, iron deficiency anemia, menstrual irregularities, hypo and hyperthyroidism. [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] The association of PTC with haematological malignencies is not well known. In this article, we report a case of PTC secondary to acute leukemia. ...
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Introduction: Idiopathic intracranial hypertension (IIH) is a rare clinical condition in which an increase in intracranial pressure is seen without a lesion in the head. The association of IIH with haematological malignencies is not well known. Case: We present 19-year-old male with frequent episodes of headache that lasted up to 24 hours, localized to the bilateral temporal region accompanied with nausea and vomiting for two months. On the neurological exam, the lateral gaze was slightly restricted. Ophthalmological exam revealed bilateral papilledema, which was more pronounced on the right. Bilateral concentric constriction, more pronounced on the right , was observed on the computerized visual field exam. Brain Magnetic Resonance Imaging (MRI) showed swelling in the optic nerve sheats, rather than on the right. In the analysis of cerebrospinal fluid (CSF), the opening pressure was 370 mmH2 Cytological examination of the CSF showed atypical lymphoid cells. The patient was diagnosed as precursor lymphoblastic leukemia/lymphoma. Conclusion: Acute leukemia–induced clinical IIH has not been reported in the literature up to now, and the present case study will contribute to the literature in this regard. This phenomenon will be noteworthy for clinicians who encounter high CSF opening pressure, abnormal CSF biochemistry, and substantial cytology in cases presenting with clinical IIH.
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... In some cases, ICH can be associated with inflammatory bowel disease (IBD), [1,2] or triggered by medications used in the management of such disorders, for example, sulfasalazine, mesalazine, or withdrawal of corticosteroid therapy. [3][4][5][6][7][8] Hereinafter, we report the unusual case of a male patient with ulcerative colitis (UC) in whom ICH resolved with mesalazine therapy. ...
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Rationale: The association between intracranial hypertension (ICH) and ulcerative colitis (UC) is rare. We report the unusual case of a male patient with UC and ICH in whom both conditions resolved with mesalazine therapy. Patient concerns: A 48-year-old Caucasian man presented to our department in June 2016 for decreased vision, transient visual obscuration, pulsatile tinnitus and headaches of 7 months duration. Bilateral optic disc swelling was found at fundus examination. Brain MRI excluded any brain tumor and lumbar puncture showed cerebrospinal fluid (CSF) opening pressure of 26 cm of water with normal CSF contents. Diagnoses: Idiopathic ICH was suspected. Interventions: The patient was managed with oral acetazolamide. Headaches initially improved but the dosage could not be decreased under 750 mg a day without recurrence of the symptoms. Extensive review of systems showed that the patient had active UC. He was given oral mesalazine, 2000 mg a day. Outcomes: The symptoms of UC and ICH quickly resolved. Acetazolamide was progressively tapered over the course of the 9 subsequent months and the patient did not show any worsening of his symptoms or papilledema. Lessons: UC should be added to the list of disorders associated with ICH. In case of atypical ICH with drug dependency, investigations should seek for UC. Treating efficiently UC with mesalazine may improve ICH, suggesting an underlying inflammatory process.
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... [94] Cerebral venous thrombosis has been found in 11.4% of patients who were presumed to have IIH. [95] IIH has been associated with many etiologies such as exposition to a number of drugs (vitamin A, growth hormone, steroids, minocycline and tetracycline, sulphasalazine, etc…), [96][97][98][99][100][101][102] Behçet's Disease, [103] arteriovenous malformations, [104] sleep disturbances including obstructive sleep apnea syndrome (OSAS), [105,106] extracranial venous hypertension secondary to cardiac septal defect, [107] systemic lupus erythematosus, [108] uremia, [109] iron deficiency anemia [110,111] as well as some endocrine changes such as menstrual irregularities, use of oral contraceptives, hyperthyroidism and hypothyroidism. [112,113] It has also been argued that an underlying thrombophilic defect in patients with IIH might play a role in the pathogenesis and some small studies showed abnormalities in prothrombotic factors. ...
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Idiopathic intracranial hypertension (IIH) is a syndrome characterized by increased intracranial pressure of unknown cause, leading to severe headache, papilledema and visual disturbances. Its former name, pseudotumor cerebri, has gained popularity recently. The strongest and most consistent risk factors of IIH are obesity and female gender. Infrequently, IIH may present in the absence of papilledema showing a headache profile similar to chronic daily headache with migrainous features. There have been several proposed mechanisms to explain the etiology of this disorder associated with various clinical conditions. In recent years, some inflammatory factors, natriuretic peptides and aquaporins have been proposed as possible contributors of the pathogenesis. On the other hand, some investigators have reported that bilateral transverse sinus stenosis is seen in the majority of IIH patients; therefore, dural sinus stent placement is used in some patients. No single theory has been able to provide a comprehensive answer, and there is no consensus about the exact cause of IIH. The aim of this review was to discuss the new insights on the mysterious pathogenesis of IIH.
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... The underlying mechanism is still not fully understood but presumably a mismatch develops between cerebrospinal fluid production by the choroid plexus and its absorption by the arachnoid granulations. Several case reports and case series have repeatedly implicated other drugs as a contributory factor in intracranial hypertension including minocycline [5], tetracycline [6], nalidixic acid [7], danazol [8], Reversible bilateral optic disc swelling in a renal patient 345 ciprofloxacin [9], vitamin A [10], isotretinoin [11], corticosteroids [12] and sulphasalazine [13]. ...
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Patients with idiopathic intracranial hypertension may occasionally present with coexisting lower motor neuron facial weakness. This study reviews a 6-year experience at Mayo Clinic. The aim of this study was to determine the possible association of idiopathic intracranial hypertension and facial paresis. Two cases were identified. Both fulfilled the modified Dandy's diagnostic criteria for idiopathic intracranial hypertension. Treatment consisted of steroids in one, and emergent optic nerve sheath fenestration in the other. The cranial nerve palsies resolved in both cases.
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Intracranial dermoid cysts are rare congenital neoplasms that are believed to arise from ectopic cell rests incorporated in the closing neural tube. The rupture of an intracranial dermoid cyst is a relatively rare event that typically occurs spontaneously. In the past it was believed that rupture is always fatal, a hypothesis that is not supported by more recently reported cases. The symptoms associated with rupture vary from no symptoms to sudden death. The present paper analyzes published cases of ruptured intracranial dermoid cysts in terms of their age profile and their clinical presentation and describes an additional case. Analysis of published cases revealed headache (14 out of 44 patients; 31.8%) and seizures (13 out of 44 patients; 29.5%), to be the most common signs of rupture followed by, often temporary, sensory or motor hemisyndrome (7 out of 44 patients; 15.9%), and chemical meningitis (3 out of 44 patients; 6.9%). Headache occurred primarily in younger patients (mean age 23.5 +/- 9.3 years), whereas seizures primarily occurred in older patients (mean age 42.8 +/- 11.3 years). The patients with sensory or motor hemisyndrome associated with rupture of an intracranial dermoid cyst showed a more homogeneous age distribution (mean age 38.4 +/- 23.5 years).
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