Symptoms and Etiologies of Pseudotumor Cerebri

J Millichap; John Millichap

doi:10.15844/pedneurbriefs-28-10-2

Authors:

J Gordon Millichap ,

Northwestern University Feinberg School of Medicine, US

John J Millichap

Northwestern University Feinberg School of Medicine, US

Abstract

How to Cite: Millichap, J.G. and Millichap, J.J., 2014. Symptoms and Etiologies of Pseudotumor Cerebri. Pediatric Neurology Briefs, 28(10), pp.74–75. DOI: http://doi.org/10.15844/pedneurbriefs-28-10-2

Published on 01 Oct 2014

CC BY 4.0

Investigators from Ankara Pediatrics, Turkey, evaluate the clinical symptoms and etiology in records of 53 patients (32 female) diagnosed with pseudotumor cerebri (PTC) in a child neurology department between 2005 and 2012. Mean age at presentation was 10.9 years (range 3-17) and one half were age 11 years or younger. Prepubertal patients (under 12 years old) were male in >50%, while 74% patients at puberty were girls. Etiology was undetermined or idiopathic in 30 and symptomatic in 23. Obesity rate was 41% for pubertal patients and 31% for prepubertal patients. Obesity was not related to etiology or puberty. In idiopathic cases, headache was the most common symptom (in 88%), nausea and/or vomiting in 30%, diplopia in 28%, and dizziness in 9%. Papilledema was found in 100%, and VI or VII nerve palsy in 11.3%. An etiologic factor for symptomatic PTC was identified in 43% of patients and included cerebral venous sinus thrombosis in 6 patients, upper respiratory tract infections in 4, iron deficiency anemia in 3, steroid withdrawal in 3 epilepsy patients, risperidone or cyclosporine usage in 3, Brucella infection, post-traumatic and slit ventricle syndrome in one each of the secondary PTC group. Comorbid disorders in the idiopathic group were related to obesity (hypertension, diabetes), and in the symptomatic group, epilepsy, and vitamin D deficiency. Papilledema was lessened by acetazolamide in 72%. [1]

COMMENTARY. Idiopathic intracranial hypertension (IIH) is defined as intracranial pressure increase with no intracranial pathology and a normal CSF content [2]. The term, “pseudotumor cerebri,” is used where an etiology for intracranial hypertension is identified or suspected [3]. Olfactory impairment, an under-recognized complication of idiopathic intracranial hypertension, is studied in relation to astronauts in head-down tilt positions [4]. Many long-duration astronauts develop signs of elevated intracranial pressure and have olfactory threshold dysfunction.

Controversy regarding efficacy of acetazolamide in IIH. The efficacy of acetazolamide in IIH is questioned since a randomized controlled trial failed to show a significant difference in lumbar puncture pressure, headache disability and visual acuity in the acetazolamide vs placebo groups [5]. A prospective cohort study found that weight loss is an effective therapy in IIH [6, 7], leading to the proposition that weight loss may be the reason for the small improvement in the acetazolamide cohort in a most recent IIH Treatment Trial [7]. Proponents of a positive effect of acetazolamide in IIH argue that the effect of acetazolamide on visual field function is independent of its effect on weight loss and does not relate to the anorexigenic effect of acetazolamide [8].

References

Degerliyurt, A Teber, S Karakaya, G Güven, A Seker, ED Arhan, EP et al. (2014). Pseudotumor cerebri/idiopathic intracranial hypertension in children: An experience of a tertiary care hospital. Brain Dev Sep 201436(8): 690–9, DOI: https://doi.org/10.1016/j.braindev.2013.09.007 [PubMed]

Skau, M, Brennum, J, Gjerris, F and Jensen, R (2006). What is new about idiopathic intracranial hypertension? An updated review of mechanism and treatment. Cephalalgia Apr 200626(4): 384–99, DOI: https://doi.org/10.1111/j.1468-2982.2005.01055.x [PubMed]

Spennato, P Ruggiero, C Parlato, RS Buonocore, MC Varone, A Cianciulli, E et al. (2011). Pseudotumor cerebri. Childs Nerv Syst Feb 201127(2): 215–35, DOI: https://doi.org/10.1007/s00381-010-1268-x [PubMed]

Bershad, EM Urfy, MZ Calvillo, E Tang, R Cajavilca, C Lee, AG et al. (2014). Marked olfactory impairment in idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry Sep 201485(9): 959–64, DOI: https://doi.org/10.1136/jnnp-2013-307232 [PubMed]

Ball, AK Howman, A Wheatley, K Burdon, MA Matthews, T Jacks, AS et al. (2011). A randomised controlled trial of treatment for idiopathic intracranial hypertension. J Neurol May 2011258(5): 874–81, DOI: https://doi.org/10.1007/s00415-010-5861-4 [PubMed]

Sinclair, AJ Burdon, MA Nightingale, PG Ball, AK Good, P Matthews, TD et al. (2010). Low energy diet and intracranial pressure in women with idiopathic intracranial hypertension: prospective cohort study. BMJ Jul 7 2010341: c2701.DOI: https://doi.org/10.1136/bmj.c2701 [PubMed]

Sinclair, AJ, Woolley, R and Mollan, SP (2014). Idiopathic intracranial hypertension. JAMA Sep 10 2014312(10): 1059–60, DOI: https://doi.org/10.1001/jama.2014.8894 [PubMed]

Wall, M McDermott, MP Kieburtz, KD Corbett, JJ Feldon, SE Friedman, DI et al. (2014). Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. JAMA Apr 23-30 2014311(16): 1641–51, DOI: https://doi.org/10.1001/jama.2014.3312 [PubMed]

[CIT0001] Degerliyurt, A Teber, S Karakaya, G Güven, A Seker, ED Arhan, EP et al. (2014). Pseudotumor cerebri/idiopathic intracranial hypertension in children: An experience of a tertiary care hospital. Brain Dev Sep 201436(8): 690–9, DOI: https://doi.org/10.1016/j.braindev.2013.09.007 [PubMed]

[CIT0002] Skau, M, Brennum, J, Gjerris, F and Jensen, R (2006). What is new about idiopathic intracranial hypertension? An updated review of mechanism and treatment. Cephalalgia Apr 200626(4): 384–99, DOI: https://doi.org/10.1111/j.1468-2982.2005.01055.x [PubMed]

[CIT0003] Spennato, P Ruggiero, C Parlato, RS Buonocore, MC Varone, A Cianciulli, E et al. (2011). Pseudotumor cerebri. Childs Nerv Syst Feb 201127(2): 215–35, DOI: https://doi.org/10.1007/s00381-010-1268-x [PubMed]

[CIT0004] Bershad, EM Urfy, MZ Calvillo, E Tang, R Cajavilca, C Lee, AG et al. (2014). Marked olfactory impairment in idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry Sep 201485(9): 959–64, DOI: https://doi.org/10.1136/jnnp-2013-307232 [PubMed]

[CIT0005] Ball, AK Howman, A Wheatley, K Burdon, MA Matthews, T Jacks, AS et al. (2011). A randomised controlled trial of treatment for idiopathic intracranial hypertension. J Neurol May 2011258(5): 874–81, DOI: https://doi.org/10.1007/s00415-010-5861-4 [PubMed]

[CIT0006] Sinclair, AJ Burdon, MA Nightingale, PG Ball, AK Good, P Matthews, TD et al. (2010). Low energy diet and intracranial pressure in women with idiopathic intracranial hypertension: prospective cohort study. BMJ Jul 7 2010341: c2701.DOI: https://doi.org/10.1136/bmj.c2701 [PubMed]

[CIT0007] Sinclair, AJ, Woolley, R and Mollan, SP (2014). Idiopathic intracranial hypertension. JAMA Sep 10 2014312(10): 1059–60, DOI: https://doi.org/10.1001/jama.2014.8894 [PubMed]

[CIT0008] Wall, M McDermott, MP Kieburtz, KD Corbett, JJ Feldon, SE Friedman, DI et al. (2014). Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. JAMA Apr 23-30 2014311(16): 1641–51, DOI: https://doi.org/10.1001/jama.2014.3312 [PubMed]

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Intracranial Hypertension

Symptoms and Etiologies of Pseudotumor Cerebri

Authors:

J Gordon Millichap ,

Northwestern University Feinberg School of Medicine, US

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John J Millichap

Northwestern University Feinberg School of Medicine, US

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Abstract

References