Pay attention to the diagnosis and treatment of idiopathic intracranial hypertension_Chinese Journal of Ocular Fundus Diseases

Authors：

Li Zhiqing ¹ , Wang Zixun ¹ ,  Wei Shihui ²

1. Tianjin Key Laboratory of Retinal Functions and Diseases, Tianjin Branch of National Clinical Research Center for Ocular Disease, Eye Institute and School of Optometry, Tianjin Medical University Eye Hospital, Tianjin 300384, China;
2. Department of Ophthalmology, The Third Medical Center, The Chinese PLA General Hospital, Beijing 100853, China;

Corresponding author：

Wei Shihui, Email: weishihui706@hotmail.com

Keywords：

Idiopathic intracranial hypertension; Multidisciplinary collaborative diagnosis and treatment; Diagnosis; Differential diagnosis; Treatment; Editorial

DOI：

10.3760/cma.j.cn511434-20241206-00471

Video：

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Idiopathic intracranial hypertension (IIH) is a neurological disorder characterized by an unexplained increase in intracranial pressure that primarily affects obese women of childbearing age, but individuals of any age, gender, or weight may also be affected. Its signature symptoms include disc edema, headache, visual disturbance, and throbbing tinnitus. Due to potentially serious complications, such as vision loss, accurate diagnosis and appropriate treatment management are critical to improving patients' quality of life. Ophthalmologists play a key role in the treatment process, as about half of patients first visit the eye department. Diagnosis of IIH depends not only on clinical presentation, but also on the exclusion of other diseases that may cause similar symptoms, and imaging and other tests to ensure an accurate diagnosis. In order to improve diagnostic accuracy and treatment efficiency, multidisciplinary collaborative diagnosis and treatment mode is advocated, especially in the face of patients with complex trauma or systemic diseases, which can effectively shorten the treatment time and ensure patient safety. Future research directions include establishing China's IIH epidemiological database, exploring clinical diagnosis and treatment methods and basic scientific research, aiming at forming diagnosis and treatment standards suitable for China's national conditions, improving medical level and improving patient prognosis. At the same time, a deeper understanding of the different forms of IIH will better serve the affected populations.

1.	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.
2.	Thurtell MJ, Kawasaki A. Update in the management of idiopathic intracranial hypertension[J]. Neurol Clin, 2021, 39(1): 147-161. DOI: 10.1016/j.ncl.2020.09.008.
3.	Mollan SP, Davies B, Silver NC, et al. Idiopathic intracranial hypertension: consensus guidelines on management[J]. J Neurol Neurosurg Psychiatry, 2018, 89(10): 1088-1010. DOI: 10.1136/jnnp-2017-317440.
4.	Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children[J]. Neurology, 2013, 81(13): 1159-1165. DOI: 10.1212/WNL.0b013e3182a55f17.
5.	Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension[J]. Neurology, 2002, 59(10): 1492-1495. DOI: 10.1212/01.wnl.0000029570.69134.1b.
6.	Sinclair AJ, Burdon MA, Nightingale PG, et al. Rating papilloedema: an evaluation of the Frisén classification in idiopathic intracranial hypertension[J]. J Neurol, 2012, 259(7): 1406-1412. DOI: 10.1007/s00415-011-6365-6.
7.	Chen BS, Meyer BI, Saindane AM, et al. Prevalence of incidentally detected signs of intracranial hypertension on magnetic resonance imaging and their association with papilledema[J]. JAMA Neurol, 2021, 78(6): 718-725. DOI: 10.1001/jamaneurol.2021.0710.
8.	Fisayo A, Bruce BB, Newman NJ, et al. Overdiagnosis of idiopathic intracranial hypertension[J]. Neurology, 2016, 86(4): 341-350. DOI: 10.1212/WNL.0000000000002318.
9.	Potter O, Menon V, Mollan SP. Risk factors and disease associations in people living with idiopathic intracranial hypertension[J]. Expert Rev Neurother, 2024, 24(7): 681-689. DOI: 10.1080/14737175.2024.2359420.
10.	Yiangou A, Mitchell JL, Nicholls M, et al. Obstructive sleep apnoea in women with idiopathic intracranial hypertension: a sub-study of the idiopathic intracranial hypertension weight randomised controlled trial (IIH: WT)[J]. J Neurol, 2022, 269(4): 1945-1956. DOI: 10.1007/s00415-021-10700-9.
11.	Lee AG, Golnik K, Kardon R, et al. Sleep apnea and intracranial hypertension in men[J]. Ophthalmology, 2002, 109(3): 482-485. DOI: 10.1016/s0161-6420(01)00987-3.
12.	Fishman RA. Polar bear liver, vitamin A, aquaporins, and pseudotumor cerebri[J]. Ann Neurol, 2002, 52(5): 531-533. DOI: 10.1002/ana.10389.
13.	Thambisetty M, Lavin PJ, Newman NJ, et al. Fulminant idiopathic intracranial hypertension[J]. Neurology, 2007, 68(3): 229-232. DOI: 10.1212/01.wnl.0000251312.19452.ec.
14.	Sowmini PR, Kumar SP, Velayutham SS, et al. Fulminant idiopathic intracranial hypertension with atypical presentation[J]. Ann Indian Acad Neurol, 2023, 26(6): 1026-1028. DOI: 10.4103/aian.aian_655_23.
15.	NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee, Wall M, McDermott MP, et al. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial[J]. JAMA, 2014, 311(16): 1641-1651. DOI: 10.1001/jama.2014.3312.
16.	Yalcinkaya Cakir G, Solmaz B, Cakir I, et al. Optical coherence tomography angiography findings in optic disc drusen and idiopathic intracranial hypertension[J]. Eur J Ophthalmol, 2024, 34(2): 566-573. DOI: 10.1177/11206721231187664.
17.	Fard MA, Sahraiyan A, Jalili J, et al. Optical coherence tomography angiography in papilledema compared with pseudopapilledema[J]. Invest Ophthalmol Vis Sci, 2019, 60(1): 168-175. DOI: 10.1167/iovs.18-25453.
18.	Geeraerts T, Newcombe VF, Coles JP, et al. Use of T2-weighted magnetic resonance imaging of the optic nerve sheath to detect raised intracranial pressure[J]. Crit Care, 2008, 12(5): R114. DOI: 10.1186/cc7006.
19.	Hyder YF, Homer V, Thaller M, et al. Defining the phenotype and prognosis of people with idiopathic intracranial hypertension after cerebrospinal fluid diversion surgery[J]. Am J Ophthalmol, 2023, 250: 70-81. DOI: 10.1016/j.ajo.2023.01.016.
20.	Corecha Santos R, Gupta B, Santiago RB, et al. Endoscopic endonasal optic nerve sheath decompression (EONSD) for idiopathic intracranial hypertension: technical details and meta-analysis[J/OL]. Clin Neurol Neurosurg, 2023, 229: 107750[2023-04-30]. https://pubmed.ncbi.nlm.nih.gov/37146367/. DOI: 10.1016/j.clineuro.2023.107750.
21.	Gates P, McNeill P. A possible role for temporary lumbar drainage in the management of idiopathic intracranial hypertension[J]. Neuroophthalmology, 2016, 40(6): 277-280. DOI: 10.1080/01658107.2016.1220954.
22.	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.
23.	Mollan SP, Chong YJ, Grech O, et al. Current perspectives on idiopathic intracranial hypertension without papilloedema[J]. Life (Basel), 2021, 11(6): 472. DOI: 10.3390/life11060472.
24.	Mollan SP, Tahrani AA, Sinclair AJ. The potentially modifiable risk factor in idiopathic intracranial hypertension: body weight[J/OL]. Neurol Clin Pract, 2021, 1(4): e504-e507[2021-08-01]. https://pubmed.ncbi.nlm.nih.gov/34484948/. DOI: 10.1212/CPJ.0000000000001063.
25.	Ko MW, Liu GT. Pediatric idiopathic intracranial hypertension (pseudotumor cerebri)[J]. Horm Res Paediatr, 2010, 74(6): 381-389. DOI: 10.1159/000321180.
26.	Balcer LJ, Liu GT, Forman S, et al. Idiopathic intracranial hypertension: relation of age and obesity in children[J]. Neurology, 1999, 52(4): 870-872. DOI: 10.1212/wnl.52.4.870.
27.	Genizi J, Lahat E, Zelnik N, et al. Childhood-onset idiopathic intracranial hypertension: relation of sex and obesity[J]. Pediatr Neurol, 2007, 36(4): 247-249. DOI: 10.1016/j.pediatrneurol.2007.01.002.
28.	Malem A, Sheth T, Muthusamy B. Paediatric idiopathic intracranial hypertension (IIH)-A review[J]. Life (Basel), 2021, 11(7): 632. DOI: 10.3390/life11070632.
29.	张奇, 吴洁, 魏世辉, 等. 特发性颅高压的研究现状及进展[J]. 国际眼科纵览, 2024, 48(5): 349-356. DOI: 10.3760/cma.j.issn.1673-5803.2024.05.005.Zhang Q, Wu J, Wei SH, et al. Current status and progress of idiopathic intracranial hypertension[J]. Int Rev Ophthalmol, 2024, 48(5): 349-356. DOI: 10.3760/cma.j.issn.1673-5803.2024.05.005.
30.	Vitaliti G, Pavone P, Matin N, et al. Therapeutic approaches to pediatric pseudotumor cerebri: new insights from literature data[J]. Int J Immunopathol Pharmacol, 2017, 30(1): 94-97. DOI: 10.1177/0394632016681578.
31.	Markey KA, Uldall M, Botfield H, et al. Idiopathic intracranial hypertension, hormones, and 11β-hydroxysteroid dehydrogenases[J]. J Pain Res, 2016, 9: 223-232. DOI: 10.2147/JPR.S80824.

1. 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.
2. Thurtell MJ, Kawasaki A. Update in the management of idiopathic intracranial hypertension[J]. Neurol Clin, 2021, 39(1): 147-161. DOI: 10.1016/j.ncl.2020.09.008.
3. Mollan SP, Davies B, Silver NC, et al. Idiopathic intracranial hypertension: consensus guidelines on management[J]. J Neurol Neurosurg Psychiatry, 2018, 89(10): 1088-1010. DOI: 10.1136/jnnp-2017-317440.
4. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children[J]. Neurology, 2013, 81(13): 1159-1165. DOI: 10.1212/WNL.0b013e3182a55f17.
5. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension[J]. Neurology, 2002, 59(10): 1492-1495. DOI: 10.1212/01.wnl.0000029570.69134.1b.
6. Sinclair AJ, Burdon MA, Nightingale PG, et al. Rating papilloedema: an evaluation of the Frisén classification in idiopathic intracranial hypertension[J]. J Neurol, 2012, 259(7): 1406-1412. DOI: 10.1007/s00415-011-6365-6.
7. Chen BS, Meyer BI, Saindane AM, et al. Prevalence of incidentally detected signs of intracranial hypertension on magnetic resonance imaging and their association with papilledema[J]. JAMA Neurol, 2021, 78(6): 718-725. DOI: 10.1001/jamaneurol.2021.0710.
8. Fisayo A, Bruce BB, Newman NJ, et al. Overdiagnosis of idiopathic intracranial hypertension[J]. Neurology, 2016, 86(4): 341-350. DOI: 10.1212/WNL.0000000000002318.
9. Potter O, Menon V, Mollan SP. Risk factors and disease associations in people living with idiopathic intracranial hypertension[J]. Expert Rev Neurother, 2024, 24(7): 681-689. DOI: 10.1080/14737175.2024.2359420.
10. Yiangou A, Mitchell JL, Nicholls M, et al. Obstructive sleep apnoea in women with idiopathic intracranial hypertension: a sub-study of the idiopathic intracranial hypertension weight randomised controlled trial (IIH: WT)[J]. J Neurol, 2022, 269(4): 1945-1956. DOI: 10.1007/s00415-021-10700-9.
11. Lee AG, Golnik K, Kardon R, et al. Sleep apnea and intracranial hypertension in men[J]. Ophthalmology, 2002, 109(3): 482-485. DOI: 10.1016/s0161-6420(01)00987-3.
12. Fishman RA. Polar bear liver, vitamin A, aquaporins, and pseudotumor cerebri[J]. Ann Neurol, 2002, 52(5): 531-533. DOI: 10.1002/ana.10389.
13. Thambisetty M, Lavin PJ, Newman NJ, et al. Fulminant idiopathic intracranial hypertension[J]. Neurology, 2007, 68(3): 229-232. DOI: 10.1212/01.wnl.0000251312.19452.ec.
14. Sowmini PR, Kumar SP, Velayutham SS, et al. Fulminant idiopathic intracranial hypertension with atypical presentation[J]. Ann Indian Acad Neurol, 2023, 26(6): 1026-1028. DOI: 10.4103/aian.aian_655_23.
15. NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee, Wall M, McDermott MP, et al. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial[J]. JAMA, 2014, 311(16): 1641-1651. DOI: 10.1001/jama.2014.3312.
16. Yalcinkaya Cakir G, Solmaz B, Cakir I, et al. Optical coherence tomography angiography findings in optic disc drusen and idiopathic intracranial hypertension[J]. Eur J Ophthalmol, 2024, 34(2): 566-573. DOI: 10.1177/11206721231187664.
17. Fard MA, Sahraiyan A, Jalili J, et al. Optical coherence tomography angiography in papilledema compared with pseudopapilledema[J]. Invest Ophthalmol Vis Sci, 2019, 60(1): 168-175. DOI: 10.1167/iovs.18-25453.
18. Geeraerts T, Newcombe VF, Coles JP, et al. Use of T2-weighted magnetic resonance imaging of the optic nerve sheath to detect raised intracranial pressure[J]. Crit Care, 2008, 12(5): R114. DOI: 10.1186/cc7006.
19. Hyder YF, Homer V, Thaller M, et al. Defining the phenotype and prognosis of people with idiopathic intracranial hypertension after cerebrospinal fluid diversion surgery[J]. Am J Ophthalmol, 2023, 250: 70-81. DOI: 10.1016/j.ajo.2023.01.016.
20. Corecha Santos R, Gupta B, Santiago RB, et al. Endoscopic endonasal optic nerve sheath decompression (EONSD) for idiopathic intracranial hypertension: technical details and meta-analysis[J/OL]. Clin Neurol Neurosurg, 2023, 229: 107750[2023-04-30]. https://pubmed.ncbi.nlm.nih.gov/37146367/. DOI: 10.1016/j.clineuro.2023.107750.
21. Gates P, McNeill P. A possible role for temporary lumbar drainage in the management of idiopathic intracranial hypertension[J]. Neuroophthalmology, 2016, 40(6): 277-280. DOI: 10.1080/01658107.2016.1220954.
22. 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.
23. Mollan SP, Chong YJ, Grech O, et al. Current perspectives on idiopathic intracranial hypertension without papilloedema[J]. Life (Basel), 2021, 11(6): 472. DOI: 10.3390/life11060472.
24. Mollan SP, Tahrani AA, Sinclair AJ. The potentially modifiable risk factor in idiopathic intracranial hypertension: body weight[J/OL]. Neurol Clin Pract, 2021, 1(4): e504-e507[2021-08-01]. https://pubmed.ncbi.nlm.nih.gov/34484948/. DOI: 10.1212/CPJ.0000000000001063.
25. Ko MW, Liu GT. Pediatric idiopathic intracranial hypertension (pseudotumor cerebri)[J]. Horm Res Paediatr, 2010, 74(6): 381-389. DOI: 10.1159/000321180.
26. Balcer LJ, Liu GT, Forman S, et al. Idiopathic intracranial hypertension: relation of age and obesity in children[J]. Neurology, 1999, 52(4): 870-872. DOI: 10.1212/wnl.52.4.870.
27. Genizi J, Lahat E, Zelnik N, et al. Childhood-onset idiopathic intracranial hypertension: relation of sex and obesity[J]. Pediatr Neurol, 2007, 36(4): 247-249. DOI: 10.1016/j.pediatrneurol.2007.01.002.
28. Malem A, Sheth T, Muthusamy B. Paediatric idiopathic intracranial hypertension (IIH)-A review[J]. Life (Basel), 2021, 11(7): 632. DOI: 10.3390/life11070632.
29. 张奇, 吴洁, 魏世辉, 等. 特发性颅高压的研究现状及进展[J]. 国际眼科纵览, 2024, 48(5): 349-356. DOI: 10.3760/cma.j.issn.1673-5803.2024.05.005.Zhang Q, Wu J, Wei SH, et al. Current status and progress of idiopathic intracranial hypertension[J]. Int Rev Ophthalmol, 2024, 48(5): 349-356. DOI: 10.3760/cma.j.issn.1673-5803.2024.05.005.
30. Vitaliti G, Pavone P, Matin N, et al. Therapeutic approaches to pediatric pseudotumor cerebri: new insights from literature data[J]. Int J Immunopathol Pharmacol, 2017, 30(1): 94-97. DOI: 10.1177/0394632016681578.
31. Markey KA, Uldall M, Botfield H, et al. Idiopathic intracranial hypertension, hormones, and 11β-hydroxysteroid dehydrogenases[J]. J Pain Res, 2016, 9: 223-232. DOI: 10.2147/JPR.S80824.

Chinese Journal of Ocular Fundus Diseases

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