Idiopathic Intracranial Hypertension in a Girl with Juvenile Idiopathic Arthritis-Associated Uveitis: a case report

Background Idiopathic intracranial hypertension (IIH) is a rare disorder of elevated pressure of cerebrospinal uid (CSF) with no evident cause. It has several associated conditions and medications and it may present asymptomatically. Case presentation We here present the case of a 14-year-old girl with juvenile idiopathic arthritis (JIA)-associated uveitis who also had papilledema. She was diagnosed later with IIH as she developed headaches. To the best of our knowledge, this is the fth case to have uveitis and IIH among children and the only one with no obvious risk factors for IIH, as all were excluded including corticosteroids, tetracyclines and weight gain Conclusions our case suggests to suspect IIH in children when optic disc edema accompany uveitis even in asymptomatic patients, considering that papilledema doesn't resolve despite uveitis recovery in this case. anti-HCV, Brucella IgG Ab, Brucella IgM Ab, and blood culture were all negative. Tuberculin test was negative after 48 -72 hours. X-ray of the hands showed malalignment with joint space narrowing in the proximal interphalangeal joints of the fth ngers in both hands. Chest, pelvis and lumbosacral spine X-ray images were within normal limits. Echocardiogram was normal with no evidence of endocarditis. fever, eyes redness articular manifestations Seven days later, returned for follow-up new complaint of generalized, persistent, tension that on analgesics, severe blurred vision and decreased visual acuity. Ophthalmic examination exhibited three grade papilledema in both eyes with no are in the anterior chamber and vitreous. Vital signs at the second presentation were all normal. Brain computed tomography (CT) was normal and lumbar puncture disclosed an increased opening pressure of 300 mm H2O. CSF analysis revealed no cells, a CSF protein of 40 mg/dL (normal up to 45 mg/dL), and a CSF glucose of 56 mg/dL with serum glucose of 79 mg/dL. Total blood leukocyte count was 10.4 K/mm3 with a differential count of neutrophil 86%. ESR was 20 mm at the end of the rst other laboratory normal. Magnetic resonance imaging (MRI) of the brain with magnetic resonance venography (MRV) normal.

Background Idiopathic intracranial hypertension (IIH) is a rare disorder of elevated pressure of cerebrospinal uid (CSF) with no evident cause.It has several associated conditions and medications and it may present asymptomatically.Case presentation We here present the case of a 14-year-old girl with juvenile idiopathic arthritis (JIA)-associated uveitis who also had papilledema.She was diagnosed later with IIH as she developed headaches.To the best of our knowledge, this is the fth case to have uveitis and IIH among children and the only one with no obvious risk factors for IIH, as all were excluded including corticosteroids, tetracyclines and weight gain Conclusions our case suggests to suspect IIH in children when optic disc edema accompany uveitis even in asymptomatic patients, considering that papilledema doesn't resolve despite uveitis recovery in this case.

Background:
Idiopathic intracranial hypertension (IIH), also called pseudotumor cerebri, is a disorder of elevated pressure of cerebrospinal uid (CSF) with no evident cause (i.e.normal neuroimaging and CSF tests) (1,2).IIH is a rare condition with an incidence of 0.1-0.9 per 100,000 population; it predominantly affects obese women of childbearing age; a signi cantly smaller percentage occur in children (3)(4)(5)(6).

Case Presentation:
A 14-year-old girl presented to our hospital with a two-day complaint of bilateral painful red eyes with mild blurred vision preceded by a month of persistent low back pain that did not improve on rest.She also had a two-year history of recurrent in ammatory arthritis and a half-hour morning stiffness with no obvious swelling.Moreover, the joint pain had become continuous and more intense ten days before presenting to our center with fever peaks several times a day.She had been treated with prednisolone 20 mg daily for just three days, then with gentamicin, doxycycline and rifampicin for ve days to no avail.She had no relevant past medical, travel or family history.
On physical examination, her blood pressure was 120/80 mm Hg, pulse was 105 bpm and regular, respiratory rate was 23 bpm, temperature was 38.5c°, and body mass index was 20 kg/m2.She had red eyes, tenderness in the right elbow and both ankles, as well as boutonniere deformity in the fth nger in both hands.The rest of the physical examination was normal.
On ophthalmic examination, her distance visual acuity without correction was 20/20 in both eyes.Slit-lamp examination revealed bilateral precipitates on the endothelium of cornea and anterior surface of lens, cells (3+) in both anterior chambers with are in anterior chamber and vitreous, which demonstrated anterior uveitis.She also had grade one papilledema bilaterally.The macula and vessels in each eye were normal.Intraocular Pressure (IOP) was normal.
Laboratory investigations revealed a blood leukocyte count of 12.4 K/mm3 with 85% neutrophils, a hemoglobin concentration of 12.2 g/dL, and a platelet count of 340 K/mm3.Erythrocyte sedimentation rate (ESR) was 50 mm at the end of the rst hour and C-reactive protein (CRP) was 4.6 mg/L.Liver function tests, creatinine, urea, urinalysis and microscopy were within normal limits.Antinuclear antibody (ANA), rheumatoid factor (RF), HIV antibodies, VDRL, HBsAg, anti-HCV, Brucella IgG Ab, Brucella IgM Ab, and blood culture were all negative.Tuberculin test was negative after 48 -72 hours.X-ray of the hands showed malalignment with joint space narrowing in the proximal interphalangeal joints of the fth ngers in both hands.Chest, pelvis and lumbosacral spine X-ray images were within normal limits.Echocardiogram was normal with no evidence of endocarditis.
After ruling out infectious causes, malignancies, and other systemic autoimmune diseases, the patient was diagnosed with enthesitis-related JIA based on the International League of Associations for Rheumatology (ILAR) classi cation criteria (20).She was treated with prednisolone 0.5 mg/kg/day, which led to improvement in fever, eyes redness and articular manifestations within three days.On discharge, we added methotrexate 10 mg once a week.Seven days later, she returned for follow-up with a new complaint of generalized, persistent, tension headache that improved partially on analgesics, accompanied by severe blurred vision and decreased visual acuity.Ophthalmic examination exhibited three grade papilledema in both eyes with no are in the anterior chamber and vitreous.Vital signs at the second presentation were all normal.Brain computed tomography (CT) was normal and lumbar puncture disclosed an increased opening pressure of 300 mm H2O.CSF analysis revealed no cells, a CSF protein of 40 mg/dL (normal up to 45 mg/dL), and a CSF glucose of 56 mg/dL with serum glucose of 79 mg/dL.Total blood leukocyte count was 10.4 K/mm3 with a differential count of neutrophil 86%.ESR was 20 mm at the end of the rst hour.All other laboratory studies were normal.Magnetic resonance imaging (MRI) of the brain with magnetic resonance venography (MRV) were normal.
Based on Revised diagnostic criteria the patient was diagnosed with IIH and started on acetazolamide 500 mg/day increased up to 750 mg daily (2).Three weeks later, the papilledema had resolved and then she was kept on the same dose of acetazolamide for one month.After that, acetazolamide and prednisolone were tapered off over one year.Currently, she is doing well on a 2.5 mg methotrexate maintenance dose weekly.

Discussion And Conclusion:
We presented the case of a girl with arthritis, uveitis, and optic disc edema, which was diagnosed later with IIH.Uveitis is an intraocular in ammation which could be idiopathic or associated with underlying systemic diseases.Therefore, we started a thorough investigation that eventually excluded other systemic etiologies and she was diagnosed with JIA associated uveitis.Since optic disc edema may be found in patients with uveitis and resolves after the treatment of uveitis (21,22), we initially opted to only monitor it.However, in this case, the optic disc edema was actually due to IIH evident by the headaches the patient