Skip to main content

Idiopathic intracranial hypertension presenting as iron deficiency anemia: a case report



The presentation of idiopathic intracranial hypertension (IIH) in association with iron deficiency anemia (IDA) is rare.

Case presentation

This case report depicts the unusual case of a 31-year-old woman of mixed Jamaican and English heritage with IIH who presented initially as IDA in the context of menorrhagia. Subsequent ophthalmic review, lumbar puncture, cerebrospinal fluid analysis and neuroimaging studies revealed severe bilateral optic disc swelling and raised intracranial pressure in keeping with IIH. Prompt treatment of IDA with blood transfusion and orally administered iron supplements, in addition to medical treatment for IIH, contributed to significant improvement of symptoms and prevented long-term visual deficits.


The possibility of IDA, albeit rare, should always be considered and investigated appropriately in all patients with IIH, as the treatment of the anemia alone may be sight-saving.

Peer Review reports


Idiopathic intracranial hypertension (IIH) is clinically defined as raised intracranial pressure (ICP) without evidence of a detectable cause. Disease prevalence is greatest in obese women of childbearing age, with a global incidence of 12–20 per 100,000 per year in this category. In comparison, the incidence in the general population is only 0.5–2 per 100,000 people per year [1]. IIH was originally referred to as benign intracranial hypertension, but this is now obsolete given its potential to cause severe and permanent blindness [2]. The exact pathogenesis behind IIH remains unknown; however, there is speculation about whether abnormal pressure gradients seen in venous hypertension may contribute to IIH [2].

Iron deficiency anemia (IDA) has been reported as a rare association with IIH. Since its first description in 1840 by Praël [3], fewer than 100 such cases have been reported in the literature [4]. Although cases with isolated IIH and anemia have been described in which full symptomatic resolution was achieved with the treatment of anemia alone [5,6,7,8,9,10], the causal relationship between these two entities remains questionable. Several mechanisms have been proposed, including a hyperviscous state, which leads to increased venous pressure without true venous sinus thrombosis [4], and a hypoxic state with resultant cerebral edema due to underlying anemia [3].

We present a rare case of IIH presenting initially as IDA in which significant resolution of papilledema was achieved with the prompt and simple correction of anemia, preventing long-term visual deficits. This illustrates the importance of clinician awareness of the rare association of IDA with IIH, especially given that treatment of anemia may be pivotal in optimizing visual outcome.

Case presentation

Our patient, a 31-year-old woman of mixed Jamaican and English heritage, first presented to the general accident and emergency department with a 4-week history of menorrhagia and a 3-week history of lethargy, palpitations, exertional dyspnea, postural dizziness, syncopal episodes, headache and vomiting (Fig. 1). She initially denied any visual symptoms and was admitted to the gynecology unit for treatment and monitoring. She had a normal body mass index of 24, and her medical, drug and family history were unremarkable, apart from a congenital left esotropia corrected with bilateral medial rectus recession as a child.

Fig. 1
figure 1

Clinical timeline of a 31-year-old woman of mixed Jamaican and English heritage diagnosed with IIH and IDA. OCT optical coherence tomography, MRI magnetic resonance imaging, MRV magnetic resonance venography, CVST cerebral venous sinus thrombosis, IIH idiopathic intracranial hypertension, IDA iron deficiency anemia

The next day she revealed that over the past 2 weeks she had developed pulsatile tinnitus, intermittent blurred vision and transient visual obscurations. Three days prior to admission she had developed neck pain and photophobia, and 2 days prior to admission mild clumsiness of the right arm and hand and numbness on the right scalp and right fingertips. She underwent an urgent ophthalmic and orthoptic examination, which revealed severe papilledema (grade 5 on Frisén scale) and a partial right lateral rectus palsy. She was afebrile and mildly hypotensive (blood pressure 87/62 mmHg). Best-corrected visual acuity (BCVA) was 6/9 in the right eye and 6/6 in the left eye. Standard color vision test with Ishihara plates was 16/17 in the right eye and 15/17 in the left eye. No relative afferent pupillary defect was demonstrated. Intraocular pressure and anterior segment were unremarkable. The peripheral retina, vessels and other cranial nerves were normal. She also underwent an urgent inpatient neurological review, and peripheral neurological examination was normal.

Goldmann visual field testing revealed an enlarged left blind spot and constriction of peripheral fields in both eyes (Fig. 2). Ultra-widefield imaging (Optos®) confirmed severe papilledema (Fig. 3), and optical coherence tomography (OCT) demonstrated subretinal fluid in the peripapillary and macular area (Fig. 2).

Fig. 2
figure 2

Initial optical coherence tomography (OCT) demonstrating peripapillary and macular subretinal fluid (white arrows). Color fundus photographs (b right; d left) highlighting the position of the line scan for the OCT images (a right; c left). Initial Goldmann visual field maps (e right; f left) showing constricted fields and an enlarged left blind spot

Fig. 3
figure 3

Severe papilledema as demonstrated on Optos ultra-widefield imaging. a Right eye. b Left eye

Blood tests revealed a hemoglobin level of 74 gm/L and mean cell volume of 63.0 fL consistent with microcytic anemia, as well as a platelet count of 584 × 109/L consistent with thrombocytosis. Peripheral smear showed microcytic hypochromic red blood cells with anisopoikilocytosis. Iron studies in keeping with iron deficiency were as follows: serum iron 2.2 μmol/L, serum total iron binding capacity 94.7 μmol/L, transferrin saturation 2.3% and ferritin 2 μg/L. The remainder of the results of the full blood count, serum chemistry, liver function, renal function, thyroid function, erythrocyte sedimentation rate, clotting screen, C-reactive protein, vitamin B12 and folate were normal. Transvaginal ultrasound revealed no uterine abnormalities. Comprehensive infection and autoimmune screens were negative. Apart from menorrhagia, no other etiology for IDA was found.

An urgent CT head and CT venogram were performed, which showed no intracerebral lesions but there was bilateral focal narrowing at the transverse and sigmoid junctions, more conspicuous on the left. There were filling defects at the proximal right sigmoid sinus which were felt to be consistent with arachnoid granulations, and there was no evidence of venous sinus thrombosis. There was prominence of the perioptic cerebrospinal fluid (CSF) spaces with minimal flattening of the sclera, especially on the left. CSF opening pressure on lumbar puncture was 63 cm H2O and closing pressure 27 cm H2O. Cytological and biochemical analyses of CSF were normal. Bacterial culture, viral polymerase chain reaction and malignant cytology were also negative. No other hormonal or metabolic cause of raised ICP was found. Subsequent magnetic resonance imaging (MRI) of the brain with venography revealed findings of bilateral transverse sigmoid sinus narrowing, prominence of the perioptic nerve spaces and an empty sella, consistent with IIH and excluding venous sinus thrombosis.

The patient was transfused with 2 units of red blood cells and commenced on orally administered iron supplementation and acetazolamide 1 g once a day. All symptoms rapidly improved following this, with return of hemoglobin level to 105 gm/L at day 10. Repeat lumbar puncture at 1-month follow-up revealed a significantly reduced opening pressure of 27 cm H2O. There was a gradual resolution of papilledema over the following 2 months (Fig. 4), with preserved BCVA and improved visual field (Fig. 4). Following tapering of acetazolamide treatment, the patient remains asymptomatic whilst solely on iron replacement and is under regular follow-up in our neuro-ophthalmology clinic.

Fig. 4
figure 4

Follow-up optical coherence tomography (OCT) and color fundus photography demonstrating resolution of papilledema. Color fundus photographs (b right; d left) highlighting the position of the line scan for the OCT images (a right; c left). Follow-up Goldmann visual field maps (e right; f left) showing improvement of visual field in both eyes, with a residual central defect in the right eye

Discussion and conclusions

Given that IDA is extremely common and is the most prevalent cause of anemia worldwide [11], our case highlights the importance of considering, detecting and treating concomitant IDA in patients with IIH. This is supported by the recent diagnostic criteria for IIH proposed by Friedman and Jacobson that specify severe IDA as a condition that can result in intracranial hypertension and mimic IIH [12]. However, the authors acknowledge that the establishment of a definite association between these two entities is limited by the paucity of reported cases. Our case adds to the growing evidence that IDA is a key etiological factor in a subset of patients with raised ICP, and it may be arguable that such cases should not be termed “true” IIH, but rather secondary intracranial hypertension.

The differential diagnosis of IIH includes masquerading conditions that may produce raised ICP. These include a number of medical disorders (for example, cerebral venous sinus thrombosis, renal failure) and medications (for example, tetracycline, vitamin A and related compounds) [12]. The valid diagnosis of IIH therefore requires the exclusion of secondary causes through a thorough workup, all of which was fulfilled in our case. It remains unclear whether IDA is causative or simply coincidental in the context of our patient with IIH. However, it is likely to be the main etiological factor given the exclusion of all other possible diagnoses with an extensive workup, and significant clinical improvement and maintenance upon normalization of hemoglobin and iron levels. We speculate that the short course of oral acetazolamide acted as a temporizing measure while allowing iron-related homeostatic mechanisms to slowly regain control in normalizing ICP.

Although the causal relationship between IDA and IIH remains speculative [13,14,15], a recent large retrospective cohort study of 607 patients with IIH found an independent association between IIH and anemia in patients with obesity [16]. This is corroborated by a consecutive case series that found a clinically significant proportion of cases of IDA in patients with IIH [6, 10], as well as observations from isolated case reports where correction of anemia alone corresponded with full resolution of symptoms and signs of IIH [4, 5, 8].

The exact etiological role of anemia in raised ICP remains unclear. Different mechanisms have been posited by different authors, including (1) increased cerebral venous pressure secondary to a relative hyperviscosity state that ultimately leads to raised ICP [4], (2) altered cerebral hemodynamics due to tissue hypoxia which increases brain capillary permeability and therefore ICP [3], and (3) dysfunctional iron hemostasis causing altered CSF dynamics and raised ICP [6].

Of these, the hyperviscosity theory is likely to be the most plausible. IDA is considered a hypercoagulable state and has been associated with venous and arterial cerebral thrombosis [17, 18]. Pathogenic processes in IDA contributing to this may include abnormal blood flow patterns within the vessels as a result of reduced deformability and increased viscosity of microcytic red blood cells [19]. In addition, IDA may result in reactive thrombocytosis due to the lack of inhibition of thrombopoiesis [20, 21]. These processes have been hypothesized to lead to increased venous pressure which decreases the rate of CSF resorption, thereby resulting in raised ICP [4]. Interestingly, cerebral venous sinus thrombosis is often excluded in such cases [4, 6], including our patient, which could suggest IDA-associated hyperviscosity to be a milder variant lying on a similar disease spectrum with true venous sinus thrombosis.

In conclusion, clinicians should be aware of IDA as a rare but important association in patients with IIH. Consequently, the workup of patients presenting with signs of raised ICP should include a full blood count, and if appropriate, hematinics including iron studies, especially in the absence of other known associated factors such as obesity or medications. Prompt evaluation and treatment of IDA should be achieved to reduce, if not prevent, long-term ocular morbidity and mortality.

Availability of data and materials

Not applicable.



Cerebrospinal fluid


Computed tomography


Best corrected visual acuity


Intracranial pressure


Iron deficiency anemia


Idiopathic intracranial hypertension


Magnetic resonance imaging


Optical coherence tomography


  1. Markey KA, Mollan SP, Jensen RH, Sinclair AJ. Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. Lancet Neurol. 2016;15(1):78–91.

    Article  Google Scholar 

  2. Acheson JF. Idiopathic intracranial hypertension and visual function. Br Med Bull. 2006;79–80(1):233–44.

    Article  Google Scholar 

  3. Capriles LF. Intracranial hypertension and iron-deficiency anemia: report of four cases. Arch Neurol. 1963;9(2):147–53.

    Article  CAS  Google Scholar 

  4. Biousse V, Rucker JC, Vignal C, Crassard I, Katz BJ, Newman NJ. Anemia and papilledema. Am J Ophthalmol. 2003;135(4):437–46.

    Article  Google Scholar 

  5. Stoebner R, Kiser R, Alperin JB. Iron deficiency anemia and papilledema. Rapid resolution with oral iron therapy. Am J Dig Dis. 1970;15(10):919–22.

    Article  CAS  Google Scholar 

  6. Mollan SP, Ball AK, Sinclair AJ, Madill SA, Clarke CE, Jacks AS, et al. Idiopathic intracranial hypertension associated with iron deficiency anaemia: a lesson for management. Eur Neurol. 2009;62(2):105–8.

    Article  CAS  Google Scholar 

  7. Mangat SS. Papilledema due to iron-deficiency anaemia. Philipp J Ophthalmol. 2010;35(2):76–8.

    Google Scholar 

  8. Kaul B, Ramanarayanan S, Mahapatra H, Sethi TK, Ahlawat R. Iron deficiency masquerading as idiopathic intracranial hypertension. BMJ Case Rep. 2009;bcr06.2008.0346.

  9. Forman EB, O’Byrne JJ, Capra L, McElnea E, King MD. Idiopathic intracranial hypertension associated with iron-deficiency anaemia. Arch Dis Child. 2013;98(6):418.

    Article  Google Scholar 

  10. Meng C, Lai CT, Jing Y, Jiang HQ, Sun HL, Ma ZH, et al. Clinical analysis of 36 cases of idiopathic intracranial hypertension complicated with iron deficiency anemia. Natl Med J China. 2019;99(33):2592–6.

    CAS  Google Scholar 

  11. Johnson-Wimbley TD, Graham DY. Diagnosis and management of iron deficiency anemia in the 21st century. Therap Adv Gastroenterol. 2011;4(3):177–84.

    Article  Google Scholar 

  12. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology. 2002;59(10):1492–5.

    Article  Google Scholar 

  13. Giuseffi V, Wall M, Siegel PZ, Rojas PB. Symptoms and disease associations in idiopathic intracranial hypertension (pseudotumor cerebri): a case-control study. Neurology. 1991;41(2):239–44.

    Article  CAS  Google Scholar 

  14. Wall M, George D. Idiopathic intracranial hypertension. A prospective study of 50 patients. Brain. 1991;114:155–80.

    Article  Google Scholar 

  15. Ireland B, Corbett JJ, Wallace RB. The search for causes of idiopathic intracranial hypertension. A preliminary case-control study. Arch Neurol. 1990;47(3):315–20.

    Article  CAS  Google Scholar 

  16. Ardissino M, Moussa O, Tang A, Muttoni E, Ziprin P, Purkayastha S. Idiopathic intracranial hypertension in the British population with obesity. Acta Neurochir (Wien). 2019;161(2):239–46.

    Article  Google Scholar 

  17. Knizley HJ, Noyes WD. Iron deficiency anemia, papilledema, thrombocytosis, and transient hemiparesis. Arch Intern Med. 1972;129(3):483–6.

    Article  Google Scholar 

  18. Belman AL, Roque CT, Ancona R, Anand AK, Davis RP. Cerebral venous thrombosis in a child with iron deficiency anemia and thrombocytosis. Stroke. 1990;21(3):488–93.

    Article  CAS  Google Scholar 

  19. Hartfield DS, Lowry NJ, Keene DL, Yager JY. Iron deficiency: a cause of stroke in infants and children. Pediatr Neurol. 1997;16(1):50–3.

    Article  CAS  Google Scholar 

  20. Kuku I, Kaya E, Yologlu S, Gokdeniz R, Baydin A. Platelet counts in adults with iron deficiency anemia. Platelets. 2009;20(6):401–5.

    Article  CAS  Google Scholar 

  21. Dan K. Thrombocytosis in iron deficiency anemia. Intern Med. 2005;44(10):1025–6.

    Article  Google Scholar 

Download references




Not applicable.

Author information

Authors and Affiliations



PYS, PT, IOCW, SR and DPK were involved in data acquisition, analysis and interpretation. PYS was involved in the first draft of the manuscript. PYS, PT, IOCW, SR and DPK were involved in revising and critically appraising the manuscript. PYS, PT, IOCW, SR and DPK were involved in the final approval for publication. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Peng Yong Sim.

Ethics declarations

Ethics approval and consent to participate

Not applicable.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Competing interests

The authors declare that they have no competing interests.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Sim, P.Y., Taribagil, P., Woollacott, I.O.C. et al. Idiopathic intracranial hypertension presenting as iron deficiency anemia: a case report. J Med Case Reports 15, 45 (2021).

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: