|Year : 2016 | Volume
| Issue : 1 | Page : 25-29
Idiopathic intracranial hypertension: Clinical profile and outcome
Shobha G Pai1, Trisha Sharma1, Richa Gupta2
1 Department of Ophthalmology, Kasturba Medical College, Mangalore, Karnataka, India
2 Department of Ophthalmology, Sankara Nethralaya, Chennai, Tamil Nadu, India
|Date of Submission||01-Nov-2014|
|Date of Acceptance||26-Jun-2015|
|Date of Web Publication||19-Jan-2016|
Room number 401, KMC PG Ladies Hostel, Lalbagh, Mangalore - 575 003, Karnataka
Source of Support: None, Conflict of Interest: None
Background: It has been a long standing challenge to clinicians and investigators to explain the pathophysiology of Idiopathic Intracranial Hypertension (IIH).Therefore, the goal of this study is to delineate the clinical course of this disorder. Aim: To delineate the clinical course of Idiopathic Intracranial Hypertension with an emphasis on visual prognosis. Settings and Design: Prospective, observational study of 18 patients. Materials and Methods: Patients with unilateral or bilateral disc oedema; CSF pressure >25 cm H 2 O non- focal neurological examination, and normal CT/MRI/MRV scans were included in the study, while those with concurrent ocular disease were excluded .Ocular examination, visual acuity, fundus photography and visual fields evaluation was done at presentation and during follow up visits for a period of two years. Statistical analysis: Descriptive study. Results: Of the 18 patients, 16 were females. The average age of onset was 31.5 years. Hypertension was the most common systemic comorbidity, seen in 5 patients. Headache was the most common presenting symptom, seen in all 18 patients .Diminution of vision was seen in 16 eyes, out of which 14 improved while 2 showed progressive impairment of vision. Abnormal visual field tests included an enlarged blind spot in 11 out of 36 eyes and peripheral constriction in 14 out of 36 eyes .17 patients had a bilateral and symmetric disc swelling. A CSF opening pressure of more than 40 cm of H 2 O was noted in 6 patients. All the patients were managed medically with diuretics therapy (acetazolamide) and weight reduction. Conclusion: A strong suspicion of Idiopathic Intracranial Hypertension in chronic severe headaches and immediate investigations followed by proper treatment can salvage vision of patients even in cases with established papilledema.
Keywords: Idiopathic Intracranial Hypertension, Pseudotumor cerebri, papilledema, headache
|How to cite this article:|
Pai SG, Sharma T, Gupta R. Idiopathic intracranial hypertension: Clinical profile and outcome. J Clin Ophthalmol Res 2016;4:25-9
|How to cite this URL:|
Pai SG, Sharma T, Gupta R. Idiopathic intracranial hypertension: Clinical profile and outcome. J Clin Ophthalmol Res [serial online] 2016 [cited 2022 Jul 4];4:25-9. Available from: https://www.jcor.in/text.asp?2016/4/1/25/174402
It has been a long standing challenge to clinicians and investigators to explain the pathophysiology of the diverse symptoms, collectively termed as idiopathic intracranial hypertension (IIH) or pseudotumor cerebri. It may be associated with serious visual loss. , Unfortunately, current knowledge of IIH is limited. Therefore, the goal of this study is to delineate the clinical course of this disorder with an emphasis on visual prognosis.
| Materials and Methods|| |
We conducted a prospective study of 18 patients, who presented to the Neurology and Ophthalmology Departments of our hospital over a period of 5 years and were diagnosed as IIH. Approval for this study was obtained from the Institutional Ethics Committee. Written informed consent was obtained from all participants before enrolment in the study. All clinical investigations were conducted according to the principles of Declaration of Helsinki.
The inclusion criteria were unilateral or bilateral disc edema, nonfocal neurological examination, except for isolated abducens nerve palsy, computed tomography (CT)/magnetic resonance venography (MRV) scans and cerebrospinal fluid (CSF) opening pressure ≥25 cm H 2 O. Patients with concurrent ocular diseases were excluded.
All patients underwent a complete medical evaluation including careful history taking, ophthalmic examination, complete blood count, CT scan, MRV scan, and CSF analysis (including opening pressure). Ocular examination consists of visual acuity measurement with Snellen's chart, anterior segment examination using slit lamp biomicroscopy, applanation tonometry, stereoscopic fundus photography and visual fields evaluation using automated perimetry with the Humphrey 30-2 program. The degree of papilledema was graded using Frisen's scheme. ,
Visual acuity, optic disc changes, and visual field defects were checked in all the patients during follow-up, which was done every 2 weeks for a month, monthly for 3 months, and after that every 6 months for 2 years.
| Results|| |
Of the 18 patients, 16 were females. The average age of onset was 31.5 years (range 9-52 years). Systemic hypertension was observed in 5 patients, all of whom were on medications for the same. Other systemic associations included hyperthyroidism, on treatment, in 1 patient and chronic obstructive pulmonary disease in another [Figure 1]. Headache was the most common presenting symptom seen in all 18 patients, followed by blurring of vision in 8 patients, nausea and vomiting in 7 patients, diplopia in 1 patient, tinnitus in 1 patient and transient obscuration of vision in 1 patient [Figure 2]. Visual acuity ranged from 6/6 to 6/24 at the last visit. Totally, 29 out of 36 eyes had a visual acuity of 6/6 [Table 1]. Only 1 out of 36 eyes had a visual acuity of 6/24. All patients are improved during the course of treatment except for 1 patient whose vision decreased from 6/12 to 6/24 right eye and 6/6-6/18 left eye which was associated with worsening of edema. This patient was initially started on oral acetazolamide 500 mg twice a day. However, the patients showed visual deterioration despite medical therapy and was referred to the neurosurgeon and to consider ventriculoperitoneal (VP) shunt but the patient was not willing for surgery and was lost to follow-up [Figure 3]. While eight subjects was showed no visual field defects, abnormal visual field tests included an enlarged blind spot in 11 out of 36 eyes, peripheral constriction in 14 out of 36 eyes, paracentral scotoma in 1 out of 36 eyes, arcuate scotoma in 1 patient and generalized reduction in sensitivity in 1 patient [Figure 4]. Bilateral and symmetric disc swelling was found in 17 patients [Figure 5]. Neuroimaging like CT scan and MRV were normal for all patients. None of the patients had abnormal ventricular sizes. Lumbar puncture revealed a CSF opening pressure of more than 40 cm of H 2 O in 6 patients, 25-40 cm of H 2 O in 12 patients. CSF composition was normal in all the cases. All the patients were managed medically with acetazolamide, which was started at 500 mg twice a day and monitored for side effects. The dosage was increased up to 2 g/day, depending on the response of patients and any adverse events. In spite of medical treatment, visual function deteriorated in 1 patient, as mentioned above. No recurrences were noted in other patients.
|Table 1: Comparison of visual acuities of all subjects pre- and post-treatment|
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| Discussion|| |
IIH is a condition defined by elevated intracranial pressure but no clinical, laboratory, or radiographic evidence of responsible infection, vascular abnormality, space occupying lesion or hydrocephalus.  The diagnosis is formally established when the modified Dandy criteria are fulfilled [Table 2]. , The pathophysiology of this disorder is unclear. Studies suggest that there exists a relative resistance to the absorption of CSF across the arachnoid villi or through the extracranial lymphatics. , Other theories are supported to an abnormality in the cerebral circulation with a resulting increase in brain's water content and this raised intracranial pressure is then transmitted to the optic nerves. Another accepted theory is that elevated intracranial venous pressure, that was found universally in most cases, leads to a rise in CSF and intracranial pressure by resisting CSF absorption. ,,,,, If not treated appropriately, chronic interruption of the axoplasmic flow of the optic nerves with ensuing papilledema may lead to irreversible optic neuropathy. 
The annual incidence of IIH is 0.9/100,000 and 3.5/100,000 in females between 15 and 44 years of age,  most being obese. In our study, of the 18 patients, 16 were females (89%) which was found in accordance with the results of previously conducted studies. , The average age of onset was 31.5 years (range 9-52 years). A study by Daniels et al.  reported that the mean age as 32 years in their subjects.
Headache is the most frequently reported symptom in IIH , and was the presenting complaint of all the subjects in our study. It was reportedly seen in 78% of IIH patients by Ozer et al.  and in more than 90% cases by Binder et al.  It is usually severe and of throbbing type, generalized, continuous, and associated with neck pain.  It worsens in the morning and is increased by Valsalva manoeuvre.  However, it may conspicuously be absent in some cases. It was observed in all our patients as opposed to 76% reported by a previous Indian study.  Diminution of vision was the second most common presenting symptom in our study, seen in 16 eyes as the presenting symptom, in correspondence with the results of a study done by Ambika et al.  Out of these, 14 eyes showed improvement while both eyes of a single patient showed progressive impairment of vision. Other studies reported that the progressive, permanent worsening of visual acuity in up to 25% of cases. ,, While transient visual obscurations were reported in high numbers in previous studies, they were noted in just 10 patients. They are said to occur due to deficient maintenance of perfusion of swollen optic nerve head even with slight changes in blood pressure. ,
Pulsatile intracranial noises/pulse-synchronous tinnitus that is, described as hearing a swishing heartbeat in one/both ears, is ascribed to flow disturbances in the cerebral venous system.  While, it was reported by a single patient in our study, Schlosser et al.  report it to be a presenting symptom in 45% of patients. Another symptom encountered is diplopia, mainly horizontal, owing to unilateral or bilateral abducens nerve paresis. Vertical diplopia rarely occurs due to trochlear or oculomotor nerve palsies , or skew deviation. Diplopia was seen in just 1 patient in our study while it was reported to be present in about 8% of patients as per another Indian study  and as high as 60% by Binder et al. 
Important concerns in the patient's history include medications taken over the previous year (oral contraceptive pills, corticosteroids), history of systemic disorders (such as anemia, chronic respiratory insufficiency, sarcoidosis, systemic lupus erythematosus and thrombocytopenic purpura), history of weight gain or weight loss and previous head trauma or any intracranial surgeries. Although steroid withdrawal and Addison's disease are clearly associated with IIH, ,, the role of other endocrine abnormalities as risk factors remain unproven. Our study investigated these associated risk factors and found that eight cases demonstrated systemic risk factors, the most common one being hypertension. This is supported by a study conducted by Bruce et al. 
Bilateral and symmetrical papilledema was seen in 17 patients. Similar results were reported in studies by Ambika et al It is also worth noting that though papilledema is a cardinal feature of this condition, there have been case reports suggesting occurrence without papilledema , or pseudopapilledema.  The most important entities to address in differential diagnosis of papilledema include brain tumors, ventricular system obstruction, and dural sinus thrombosis, thus warranting neuroimaging with CT scan and MRV. It was found normal for all patients in our study.
In IIH, enlarged blind spots and peripheral field defects are early visual losses. , A careful evaluation and monitoring of visual field defects are required using quantitative perimetry. Out of 36 eyes, 11 showed blind spot enlargement while 14 showed peripheral constriction. Blind spot enlargement was also reported by Suh and Kim. 
Lumbar puncture revealed a CSF opening pressure of more than 40 cm of H 2 O in 6 patients and between 25 and 40 cm of H 2 O in 12 patients. The Median CSF opening pressure was found to be 35 cm H 2 O in a study by Riggeal et al.  CSF composition was normal in all cases.
IIH patients with persistent signs and symptoms can be treated either medically or surgically. Carbonic anhydrase inhibitors, such as, acetazolamide along with weight reduction was the initial line of treatment for our all subjects. Indications for surgery include progressive visual loss despite medical therapy, severe or rapid visual loss, and severe papilledema causing macular edema or exudates. ,, Accordingly, 1 patient was referred for surgery but was lost to follow-up. The procedures include optic nerve sheath decompression and CSF diversion procedures ,,,,,, like lumbo-peritoneal shunting  and wVP shunting. ,, Though these procedures are deemed to be effective treatment options, they do not actually treat the problem so much as just reduce the chances of complication till there is spontaneous resolution. 
Limitations in our study were small sample size and no documentation of body mass index which is one of the contributing factors as per research. However, all patients were advised weight loss along with medical therapy.
| Conclusion|| |
The diagnosis of IIH is made on identifying the typical symptoms of the disease along with documentation of papilledema, high CSF pressure, normal neuroimaging and neurologic examination, except occasional abducens nerve palsy. The best way to prevent visual loss is to test visual acuity regularly as well as assess visual fields. The course of IIH may be short, benign or may progress to a more aggressive form that ultimately results in blindness over a short period of time.  Follow-up of the patients forms an integral part of patient care in IIH as there have been several reports of delayed worsening, recurrences, and late occurrence of raised intracranial tension, some even after several years of initial presentation, , A strong suspicion of IIH in chronic severe headaches and immediate investigations followed by proper treatment can salvage vision of patients even in established papilledema.
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Conflicts of interest
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| References|| |
Nithyanandam S, Manayath GJ, Battu RR. Optic nerve sheath decompression for visual loss in intracranial hypertension: Report from a tertiary care center in South India. Indian J Ophthalmol 2008;56:115-20.
Bruce BB, Kedar S, Van Stavern GP, Monaghan D, Acierno MD, Braswell RA, et al.
Idiopathic intracranial hypertension in men. Neurology 2009;72:304-9.
Sinclair AJ, Burdon MA, Nightingale PG, Matthews TD, Jacks A, Lawden M, et al.
Rating papilloedema: An evaluation of the Frisén classification in idiopathic intracranial hypertension. J Neurol 2012;259:1406-12.
Scott CJ, Kardon RH, Lee AG, Frisen L, Wall M. Diagnosis and grading of papilledema in patients with raised intracranial pressure using optical coherence tomography(OCT) compared to clinical expert assessment using a clinical staging scale. Arch Ophthalmol 2010;128:705-11.
Rangwala LM, Liu GT. Pediatric idiopathic intracranial hypertension. Surv Ophthalmol 2007;52:597-617.
Agarwal P, Kumar M, Arora V. Clinical profile of cerebral venous sinus thrombosis and the role of imaging in its diagnosis in patients with presumed idiopathic intracranial hypertension. Indian J Ophthalmol 2010;58:153-5.
Acheson JF. Idiopathic intracranial hypertension and visual function. Br Med Bull 2006;79-80:233-44.
Edwards LJ, Sharrack B, Ismail A, Tench CR, Gran B, Dhungana S, et al.
Increased levels of interleukins 2 and 17 in the cerebrospinal fluid of patients with idiopathic intracranial hypertension. Am J Clin Exp Immunol 2013;2:234-44.
Kapoor KG, Katza SE, Grzybowskia DM, Lubowa M. Cerebrospinal ﬂuid outﬂow: An evolving perspective. Brain Res Bull 2008;77:327-34.
Digre KB. Idiopathic intracranial hypertension. BMJ 2010;341:c2836.
Kapoor KG. More than meets the eye? Redefining idiopathic intracranial hypertension. Int J Neurosci 2010;120:471-82.
Strydom MA, Briers N, Bosman MC, Steyn S. The anatomical basis of venographic filling defects of the transverse sinus. Clin Anat 2010;23:153-9.
Bateman GA, Stevens SA, Stimpson J. A mathematical model of idiopathic intracranial hypertension incorporating increased arterial inflow and variable venous outflow collapsibility. J Neurosurg 2009;110:446-56.
Sander K, Poppert H, Etgen T, Hemmer B, Sander D. Dynamics of intracranial venous flow patterns in patients with idiopathic intracranial hypertension. Eur Neurol 2011;66:334-8.
Lin J, Fernandes JK, Faria EC, Pinho RS, Masruha MR, Vilanova LC. Fulminant idiopathic intracranial hypertension in a pediatric patient following a minor head trauma. Arq Neuropsiquiatr 2009;67:519-22.
Wall M. Idiopathic intracranial hypertension. Neurol Clin 2010;28:593-617.
Raoof N, Sharrack B, Pepper IM, Hickman SJ. The incidence and prevalence of idiopathic intracranial hypertension in Sheffield, UK. Eur J Neurol 2011;18:1266-8.
Shah VA, Kardon RH, Lee AG, Corbett JJ, Wall M. Long-term follow-up of idiopathic intracranial hypertension: The Iowa experience. Neurology 2008;70:634-40
Daniels AB, Liu GT, Volpe NJ, Galetta SL, Moster ML, Newman NJ, et al.
Profiles of obesity, weight gain, and quality of life in idiopathic intracranial hypertension (pseudotumor cerebri). Am J Ophthalmol 2007;143:635-41.
Bruce BB, Biousse V, Newman NJ. Update on idiopathic intracranial hypertension. Am J Ophthalmol 2011;152:163-9.
Ozer S, Ozer PA, Kaya SC, Atas A, Altýparmak E, Atay G, et al.
Results of audiological evaluation in patients with idiopathic intracranial hypertension. Int Adv Otol 2013;9:193-202.
Binder DK, Horton HC, Lawton MT, McDermott MW. Idiopathic intracranial hypertension. Neurosurgery 2004;54:538-52.
Tasdemir HA, Dilber C, Totan M, Onder A. Pseudotumor cerebri complicating measles: A case report and literature review. Brain Dev 2006;28:395-7.
Chiarella G, Bono F, Cassandro C, Lopolito M, Quattrone A, Cassandro E. Bilateral transverse sinus stenosis in patients with tinnitus. Acta Otorhinolaryngol Ital 2012;32:238-43.
Ambika S, Arjundas D, Noronha V, Anshuman. Clinical profile, evaluation, management and visual outcome of idiopathic intracranial hypertension in a neuro-ophthalmology clinic of a tertiary referral ophthalmic centre in India.
Ann Indian Acad Neurol 2010;13:37-41.
Kim JS. Clinical reasoning: A 22-year-old woman with headache and diplopia. Neurology 2009;73:e1-7.
Ball AK, Clarke CE. Idiopathic intracranial hypertension. Lancet Neurol 2006;5:433-42.
Dhungana S, Sharrack B, Woodroofe N. Idiopathic intracranial hypertension. Acta Neurol Scand 2010;121:71-82.
Cole A, George ND. Unilateral papilloedema with transient visual obscurations. Eye (Lond) 2006;20:1095-7.
Hofmann E, Behr R, Neumann-Haefelin T, Schwager K. Pulsatile tinnitus: Imaging and differential diagnosis. Dtsch Arztebl Int 2013;110:451-8.
Schlosser RJ, Woodworth BA, Wilensky EM, Grady MS, Bolger WE. Spontaneous cerebrospinal fluid leaks: A variant of benign intracranial hypertension. Ann Otol Rhinol Laryngol 2006;115:495-500.
Tan H. Bilateral oculomotor palsy secondary to pseudotumor cerebri. Pediatr Neurol 2010;42:141-2.
Chansoria M, Agrawal A, Ganghoriya P, Raghu Raman B. Pseudotumor cerebri with transient oculomotor palsy. Indian J Pediatr 2005;72:1047-8.
Nguyen HS, Haider KM, Ackerman LL. Unusual causes of papilledema: Two illustrative cases. Surg Neurol Int 2013;4:60.
Sharma D, Mukherjee R, Moore P, Cuthbertson DJ. Addison's disease presenting with idiopathic intracranial hypertension in 24-year-old woman: A case report. J Med Case Rep 2010;4:60.
Barnett M, Sinha MD, Morrison D, Lim M. Intracranial hypertension presenting with severe visual failure, without concurrent headache, in a child with nephrotic syndrome. BMC Pediatr 2013;13:167.
Bruce BB, Preechawat P, Newman NJ, Lynn MJ, Biousse V. Racial differences in idiopathic intracranial hypertension. Neurology 2008;70:861-7.
Bono F, Messina D, Giliberto C, Cristiano D, Broussard G, Fera F, et al.
Bilateral transverse sinus stenosis predicts IIH without papilledema in patients with migraine. Neurology 2006;67:419-23.
Bono F, Messina D, Giliberto C, Cristiano D, Broussard G, D'Asero S, et al.
Bilateral transverse sinus stenosis and idiopathic intracranial hypertension without papilledema in chronic tension-type headache. J Neurol 2008;255:807-12.
Liu B, Murphy RK, Mercer D, Tychsen L, Smyth MD. Pseudopapilledema and association with idiopathic intracranial hypertension. Childs Nerv Syst 2014;30:1197-200.
Thurtell MJ, Wall M. Idiopathic intracranial hypertension (pseudotumor cerebri): Recognition, treatment, and ongoing management. Curr Treat Options Neurol 2013;15:1-12.
Best J, Silvestri G, Burton B, Foot B, Acheson J. The incidence of blindness due to idiopathic intracranial hypertension in the UK. Open Ophthalmol J 2013;7:26-9.
Suh SY, Kim SJ. IIH with normal CSF pressures? Indian J Ophthalmol 2013;61:681-2.
Riggeal BD, Bruce BB, Saindane AM, Ridha MA, Kelly LP, Newman NJ, et al.
Clinical course of idiopathic intracranial hypertension with transverse sinus stenosis. Neurology 2013;80:289-95.
Ament JD, Hoffman C, Black P. Idiopathic intracranial hypertension: A neurosurgical perspective. Contemp Neurosurg 2008;30:1-6.
Mukherjee N, Bhatti MT. Update on the surgical management of idiopathic intracranial hypertension. Curr Neurol Neurosci Rep 2014;14:438.
Malik A, Golnik K. Cerebrospinal fluid diversion procedures in the treatment of patients with idiopathic intracranial hypertension. Int Ophthalmol Clin 2014;54:51-9.
Yadav YR, Parihar V, Agarwal M, Bhatele PR, Saxena N. Lumbar peritoneal shunt in idiopathic intracranial hypertension. Turk Neurosurg 2012;22:21-6.
Ochalski PG, Horowitz MB, Mintz AH, Hughes SJ, Okonkwo DO, Kassam AB, et al.
Minimal-access technique for distal catheter insertion during ventricular peritoneal shunt procedures: A review of 100 cases. J Neurosurg 2009;111:623-7.
Tarnaris A, Toma AK, Watkins LD, Kitchen ND. Is there a difference in outcomes of patients with idiopathic intracranial hypertension with the choice of cerebrospinal fluid diversion site: A single centre experience. Clin Neurol Neurosurg 2011;113:477-9.
Abubaker K, Ali Z, Raza K, Bolger C, Rawluk D, O'Brien D. Idiopathic intracranial hypertension: Lumboperitoneal shunts versus ventriculoperitoneal shunts-Case series and literature review. Br J Neurosurg 2011;25:94-9.
Walsh TJ. Idiopathic Intracranial Hypertension (IIH). Oman Med J 2008;23:70-1.
Baheti NN, Nair M, Thomas SV. Long-term visual outcome in idiopathic intracranial hypertension. Ann Indian Acad Neurol 2011;14:19-22.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
[Table 1], [Table 2]