Diagnosis and treatment of papilledema due to idiopathic intracranial hypertension_Chinese Journal of Ocular Fundus Diseases

Authors：

Yu Mingyi ,  Jiang Libin

Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology & Visual Sciences Key Laboratory, Beijing 100730, China;

Corresponding author：

Jiang Libin, Email: jlbjlb@sina.com

Keywords：

Papilledema; Nervous system diseases/diagnosis; Nervous system diseases/therapy; Review

DOI：

10.3760/cma.j.issn.1005-1015.2017.05.032

Video：

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Abstract Full text Figures/Tables Video References Cited by

Idiopathic intracranial hypertension (IIH) is a neurological disease, characterized by increased intracranial pressure and papilledema, and often associated with headache, transient loss of vision and pulsatile tinnitus. IIH typically occurs in women of childbearing age. Over 90.0% of patients are with obesity or over weighted. Loss of sensory visual function is the major morbidity associated with IIH and some patients even develop into blindness. Most patients will have varied degrees of visual impairment, or even a few become blind. Frisén grading system, visual field examination and spectral-domain optical coherence tomography can be used to evaluate and monitor the IIH papilledema functionally and morphologically. In recent years, IIH treatment trials in other countries confirmed that, weight loss and low-salt diet combined with acetazolamide treatment has a clear improvement for IIH patients with mild visual impairment. In-depth understanding of the etiology, clinical manifestations, diagnostic criteria and the main treatment has important clinical significance for IIH patients

Citation： Yu Mingyi, Jiang Libin. Diagnosis and treatment of papilledema due to idiopathic intracranial hypertension. Chinese Journal of Ocular Fundus Diseases, 2017, 33(5): 551-554. doi: 10.3760/cma.j.issn.1005-1015.2017.05.032 Copy

1.	Wall M, Falardeau J, Fletcher WA, et al. Risk factors for poor visual outcome in patients with idiopathic intracranial hypertension[J]. Neurology, 2015, 85(9): 799-805. DOI: 10.1212/WNL.0000000000001896.
2.	Markey KA, Mollan SP, Jensen RH, et al. Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions[J]. Lancet Neurol, 2016, 15(1): 78-91. DOI: 10.1016/S1474-4422(15)00298-7.
3.	Ko MW, Chang SC, Ridha MA, et al. Weight gain and recurrence in idiopathic intracranial hypertension: a case-control study[J]. Neurology, 2011, 76(18): 1564-1567. DOI: 10.1212/WNL.0b013e3182190f51.
4.	Szewka AJ, Bruce BB, Newman NJ, et al. Idiopathic intracranial hypertension: relation between obesity and visual outcomes[J]. Neuroophthalmol, 2013, 33(1): 4-8. DOI: 10.1097/WNO.0b013e31823f852d.
5.	Alperin N, Lam BL, Tain RW, et al. Evidence for altered spinal canal compliance and cerebral venous drainage in untreated idiopathic intracranial hypertension[J]. Acta Neurochir Suppl, 2012, 114: 201-205. DOI: 10.1007/978-3-7091-0956-4_39.
6.	Ball AK, Sinclair AJ, Curnow SJ, et al. Elevated cerebrospinal fluid (CSF) leptin in idiopathic intracranial hypertension (IIH): evidence for hypothalamic leptin resistance[J]. Clin Endocrinol (Oxf), 2009, 70(6): 863-869. DOI: 10.1111/j.1365-2265.2008.03401.
7.	Bodary PF, Westrick RJ, Wickenheiser KJ, et al. Effect of leptin on arterial thrombosis following vascular injury in mice[J]. JAMA, 2002, 287(13): 1706-1709.
8.	Song HJ, Cho YG, Lee HJ. Dietary sodium intake and prevalence of overweight in adults[J]. Metabolism, 2013, 62(5): 703-708. DOI: 10.1016/j.metabol.2012.11.009.
9.	Gideon P, Sorensen PS, Thomsen C, et al. Assessment of CSF dynamics and venous flow in the superior sagittal sinus by MRI in idiopathic intracranial hypertension: a preliminary study[J]. Neuroradiology, 1994, 36(5): 350-354.
10.	Sinclair AJ, Ball AK, Burdon MA, et al. Exploring the pathogenesis of IIH: an inflammatory perspective[J]. Neuroimmunol, 2008, 15: 212-220. DOI: 10.1016/j.jneuroim.2008.06.029.
11.	Panagiotakos DB, Pitsavos C, Yannakoulia M, et al. The implication of obesity and central fat on markers of chronic inflammation: The ATTICA Study[J]. Atherosclerosis, 2005, 183(2): 308-315.
12.	Kesler A, Kliper E, Shenkerman G, et al. Idiopathic intracranial hypertension is associated with lower body adiposity[J]. Ophthalmology, 2010, 117(1): 169-174. DOI: 10.1016/j.ophtha.2009.06.030.
13.	Kosmorsky GS. Idiopathic intracranial hypertension: pseudotumor cerebri[J]. Headache, 2014, 54(2): 389-393. DOI: 10.1111/head.12284.
14.	Glueck CJ, Aregawi D, Goldenberg N, et al. Idiopathic intracranial hypertension, polycystic-ovary syndrome, and thrombophilia[J]. Lab Clin Med, 2005, 145(2): 72-82.
15.	Hayreh SS. Pathogenesis of optic disc edema in raised intracranial pressure[J]. Prog Retin Eye Res, 2016, 50: 108-144. DOI: 10.1016/j.preteyeres.2015.10.001.
16.	Bortoluzzi M, Di Lauro L, Marini G. Benign intracranial hypertension with spinal and radicular pain. Case report[J]. Neurosurg, 1982, 57(6): 833-836.
17.	OCT Sub-Study Committee for NORDIC Idiopathic Intracranial Hypertension Study Group, Auinger P, Durbin M, et al. Baseline OCT measurements in the idiopathic intracranial hypertension treatment trial, part Ⅰ: quality control, comparisons, and variability[J]. Invest Ophthalmol Vis Sci, 2014, 55(12): 8180-8188. DOI: 10.1167/iovs.14-14960.
18.	Wall M, Kupersmith MJ, Kieburtz KD, et al. The idiopathic intracranial hypertension treatment trial: clinical profile at baseline[J]. JAMA Neurol, 2014, 71(6): 693-701. DOI: 10.1001/jamaneurol.2014.133.
19.	Gospe SM 3rd, Bhatti MT, El-Dairi MA. Anatomic and visual function outcomes in paediatric idiopathic intracranial hypertension[J]. Br J Ophthalmol, 2016, 100(4): 505-509. DOI: 10.1136/bjophthalmol-2015-307043.
20.	Scott CJ, Kardon RH, Lee AG. Diagnosis and grading of papilledema in patients with raised intracranial pressure using optical coherence tomography vs clinical expert assessment using a clinical staging scale[J]. Arch Ophthalmol, 2010, 128(6): 705-711. DOI: 10.1001/archophthalmol.2010.94.
21.	OCT Sub-Study Committee for NORDIC Idiopathic Intracranial Hypertension Study Group, Auinger P, Durbin M, et al. Baseline OCT measurements in the idiopathic intracranial hypertension treatment trial, part Ⅱ: correlations and relationship to clinical features[J]. Invest Ophthalmol Vis Sci, 2014, 55(12): 8173-8179. DOI: 10.1167/iovs.14-14961.
22.	Wall M, George D. Idiopathic intracranial hypertension: a prospective study of 50 patients[J]. Brain, 1991, 114(1): 155-180.
23.	Heckmann JG, Weber M, Jünemann AG. Laser scanning tomography of the optic nerve vs CSF opening pressure in idiopathic intracranial hypertension[J]. Neurology, 2004, 62(7): 1221-1223.
24.	Kupersmith MJ, Sibony P, Mandel G, et al. Optical coherence tomography of the swollen optic nerve head: deformation of the peripapillary retinal pigment epithelium layer in papilledema[J]. Invest Ophthalmol Vis Sci, 2011, 52(9): 6558-6564. DOI: 10.1167/iovs.10-6782.
25.	Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension[J]. Neurology, 2002, 59(10): 1492-1495.
26.	Handley JD, Baruah BP, Williams DM. Bariatric surgery as a treatment for idiopathic intracranial hypertension: a systematic review[J]. Surg Obes Relat Dis, 2015, 11(6): 1396-1403. DOI: 10.1016/j.soard.2015.08.497.
27.	Yadav YR, Parihar V, Agarwal M. Lumbar peritoneal shunt in idiopathic intracranial hypertension[J]. Turk Neurosurg, 2012, 22(1): 21-26. DOI: 10.5137/1019-5149.JTN.4307-11.1.
28.	Levin AA, Hess D, Hohler AD. Treatment of idiopathic intracranial hypertension with gastric bypass surgery[J]. Int J Neurosci, 2015, 125(1): 78-80. DOI: 10.3109/00207454.2014.898144.
29.	Kwon YJ, Allen JL, Liu GT, et al. Presumed pseudotumor cerebri syndrome after withdrawal of inhaled glucocorticoids[J/OL]. Pediatrics, 2016, 137(6): 20152091[2016-05-17]. http://pediatrics.aappublications.org/cgi/pmidlookup?view=long&pmid=27244842. DOI: 10.1542/peds.2015-2091.
30.	Johnson LN, Krohel GB, Madsen RW, et al. The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri)[J]. Ophthalmology, 1998, 105(12): 2313-2317. DOI: 10.1016/S0161-6420(98)91234-9.
31.	Wong R, Madill SA, Pandey P, et al. Idiopathic intracranial hypertension: the association between weight loss and the requirement for systemic treatment[J]. BMC Ophthalmol, 2007, 21(7): 15. DOI: 10.1186/1471-2415-7-15.
32.	ten Hove MW, Friedman DI, Patel AD, et al. Safety and tolerability of acetazolamide in the idiopathic intracranial hypertension treatment trial[J]. Neuroophthalmol, 2016, 36(1): 13-19. DOI: 10.1097/WNO.0000000000000322.
33.	Worsham F Jr, Beckman EN, Mitchell EH. Sacrococcygeal teratoma in a neonate: association with maternal use of acetazolamide[J]. JAMA, 1978, 240(3): 251-252.
34.	Wang JK, Kardon RH, Ledolter J, et al. Peripapillary retinal pigment epithelium layer shape changes from acetazolamide treatment in the idiopathic intracranial hypertension treatment trial[J]. Invest Ophthalmol Vis Sci, 2017, 58(5): 2554-2565. DOI: 10.1167/iovs.16-21089.

1. Wall M, Falardeau J, Fletcher WA, et al. Risk factors for poor visual outcome in patients with idiopathic intracranial hypertension[J]. Neurology, 2015, 85(9): 799-805. DOI: 10.1212/WNL.0000000000001896.
2. Markey KA, Mollan SP, Jensen RH, et al. Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions[J]. Lancet Neurol, 2016, 15(1): 78-91. DOI: 10.1016/S1474-4422(15)00298-7.
3. Ko MW, Chang SC, Ridha MA, et al. Weight gain and recurrence in idiopathic intracranial hypertension: a case-control study[J]. Neurology, 2011, 76(18): 1564-1567. DOI: 10.1212/WNL.0b013e3182190f51.
4. Szewka AJ, Bruce BB, Newman NJ, et al. Idiopathic intracranial hypertension: relation between obesity and visual outcomes[J]. Neuroophthalmol, 2013, 33(1): 4-8. DOI: 10.1097/WNO.0b013e31823f852d.
5. Alperin N, Lam BL, Tain RW, et al. Evidence for altered spinal canal compliance and cerebral venous drainage in untreated idiopathic intracranial hypertension[J]. Acta Neurochir Suppl, 2012, 114: 201-205. DOI: 10.1007/978-3-7091-0956-4_39.
6. Ball AK, Sinclair AJ, Curnow SJ, et al. Elevated cerebrospinal fluid (CSF) leptin in idiopathic intracranial hypertension (IIH): evidence for hypothalamic leptin resistance[J]. Clin Endocrinol (Oxf), 2009, 70(6): 863-869. DOI: 10.1111/j.1365-2265.2008.03401.
7. Bodary PF, Westrick RJ, Wickenheiser KJ, et al. Effect of leptin on arterial thrombosis following vascular injury in mice[J]. JAMA, 2002, 287(13): 1706-1709.
8. Song HJ, Cho YG, Lee HJ. Dietary sodium intake and prevalence of overweight in adults[J]. Metabolism, 2013, 62(5): 703-708. DOI: 10.1016/j.metabol.2012.11.009.
9. Gideon P, Sorensen PS, Thomsen C, et al. Assessment of CSF dynamics and venous flow in the superior sagittal sinus by MRI in idiopathic intracranial hypertension: a preliminary study[J]. Neuroradiology, 1994, 36(5): 350-354.
10. Sinclair AJ, Ball AK, Burdon MA, et al. Exploring the pathogenesis of IIH: an inflammatory perspective[J]. Neuroimmunol, 2008, 15: 212-220. DOI: 10.1016/j.jneuroim.2008.06.029.
11. Panagiotakos DB, Pitsavos C, Yannakoulia M, et al. The implication of obesity and central fat on markers of chronic inflammation: The ATTICA Study[J]. Atherosclerosis, 2005, 183(2): 308-315.
12. Kesler A, Kliper E, Shenkerman G, et al. Idiopathic intracranial hypertension is associated with lower body adiposity[J]. Ophthalmology, 2010, 117(1): 169-174. DOI: 10.1016/j.ophtha.2009.06.030.
13. Kosmorsky GS. Idiopathic intracranial hypertension: pseudotumor cerebri[J]. Headache, 2014, 54(2): 389-393. DOI: 10.1111/head.12284.
14. Glueck CJ, Aregawi D, Goldenberg N, et al. Idiopathic intracranial hypertension, polycystic-ovary syndrome, and thrombophilia[J]. Lab Clin Med, 2005, 145(2): 72-82.
15. Hayreh SS. Pathogenesis of optic disc edema in raised intracranial pressure[J]. Prog Retin Eye Res, 2016, 50: 108-144. DOI: 10.1016/j.preteyeres.2015.10.001.
16. Bortoluzzi M, Di Lauro L, Marini G. Benign intracranial hypertension with spinal and radicular pain. Case report[J]. Neurosurg, 1982, 57(6): 833-836.
17. OCT Sub-Study Committee for NORDIC Idiopathic Intracranial Hypertension Study Group, Auinger P, Durbin M, et al. Baseline OCT measurements in the idiopathic intracranial hypertension treatment trial, part Ⅰ: quality control, comparisons, and variability[J]. Invest Ophthalmol Vis Sci, 2014, 55(12): 8180-8188. DOI: 10.1167/iovs.14-14960.
18. Wall M, Kupersmith MJ, Kieburtz KD, et al. The idiopathic intracranial hypertension treatment trial: clinical profile at baseline[J]. JAMA Neurol, 2014, 71(6): 693-701. DOI: 10.1001/jamaneurol.2014.133.
19. Gospe SM 3rd, Bhatti MT, El-Dairi MA. Anatomic and visual function outcomes in paediatric idiopathic intracranial hypertension[J]. Br J Ophthalmol, 2016, 100(4): 505-509. DOI: 10.1136/bjophthalmol-2015-307043.
20. Scott CJ, Kardon RH, Lee AG. Diagnosis and grading of papilledema in patients with raised intracranial pressure using optical coherence tomography vs clinical expert assessment using a clinical staging scale[J]. Arch Ophthalmol, 2010, 128(6): 705-711. DOI: 10.1001/archophthalmol.2010.94.
21. OCT Sub-Study Committee for NORDIC Idiopathic Intracranial Hypertension Study Group, Auinger P, Durbin M, et al. Baseline OCT measurements in the idiopathic intracranial hypertension treatment trial, part Ⅱ: correlations and relationship to clinical features[J]. Invest Ophthalmol Vis Sci, 2014, 55(12): 8173-8179. DOI: 10.1167/iovs.14-14961.
22. Wall M, George D. Idiopathic intracranial hypertension: a prospective study of 50 patients[J]. Brain, 1991, 114(1): 155-180.
23. Heckmann JG, Weber M, Jünemann AG. Laser scanning tomography of the optic nerve vs CSF opening pressure in idiopathic intracranial hypertension[J]. Neurology, 2004, 62(7): 1221-1223.
24. Kupersmith MJ, Sibony P, Mandel G, et al. Optical coherence tomography of the swollen optic nerve head: deformation of the peripapillary retinal pigment epithelium layer in papilledema[J]. Invest Ophthalmol Vis Sci, 2011, 52(9): 6558-6564. DOI: 10.1167/iovs.10-6782.
25. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension[J]. Neurology, 2002, 59(10): 1492-1495.
26. Handley JD, Baruah BP, Williams DM. Bariatric surgery as a treatment for idiopathic intracranial hypertension: a systematic review[J]. Surg Obes Relat Dis, 2015, 11(6): 1396-1403. DOI: 10.1016/j.soard.2015.08.497.
27. Yadav YR, Parihar V, Agarwal M. Lumbar peritoneal shunt in idiopathic intracranial hypertension[J]. Turk Neurosurg, 2012, 22(1): 21-26. DOI: 10.5137/1019-5149.JTN.4307-11.1.
28. Levin AA, Hess D, Hohler AD. Treatment of idiopathic intracranial hypertension with gastric bypass surgery[J]. Int J Neurosci, 2015, 125(1): 78-80. DOI: 10.3109/00207454.2014.898144.
29. Kwon YJ, Allen JL, Liu GT, et al. Presumed pseudotumor cerebri syndrome after withdrawal of inhaled glucocorticoids[J/OL]. Pediatrics, 2016, 137(6): 20152091[2016-05-17]. http://pediatrics.aappublications.org/cgi/pmidlookup?view=long&pmid=27244842. DOI: 10.1542/peds.2015-2091.
30. Johnson LN, Krohel GB, Madsen RW, et al. The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri)[J]. Ophthalmology, 1998, 105(12): 2313-2317. DOI: 10.1016/S0161-6420(98)91234-9.
31. Wong R, Madill SA, Pandey P, et al. Idiopathic intracranial hypertension: the association between weight loss and the requirement for systemic treatment[J]. BMC Ophthalmol, 2007, 21(7): 15. DOI: 10.1186/1471-2415-7-15.
32. ten Hove MW, Friedman DI, Patel AD, et al. Safety and tolerability of acetazolamide in the idiopathic intracranial hypertension treatment trial[J]. Neuroophthalmol, 2016, 36(1): 13-19. DOI: 10.1097/WNO.0000000000000322.
33. Worsham F Jr, Beckman EN, Mitchell EH. Sacrococcygeal teratoma in a neonate: association with maternal use of acetazolamide[J]. JAMA, 1978, 240(3): 251-252.
34. Wang JK, Kardon RH, Ledolter J, et al. Peripapillary retinal pigment epithelium layer shape changes from acetazolamide treatment in the idiopathic intracranial hypertension treatment trial[J]. Invest Ophthalmol Vis Sci, 2017, 58(5): 2554-2565. DOI: 10.1167/iovs.16-21089.

Chinese Journal of Ocular Fundus Diseases

Diagnosis and treatment of papilledema due to idiopathic intracranial hypertension

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