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The expanding spectrum of idiopathic intracranial hypertension

Abstract

Once considered a rare and often difficult diagnosis in the era predating routine MRI, idiopathic intracranial hypertension has become an everyday concern in ophthalmology and neurology clinics where, especially in the latter, essentially every young overweight woman with headaches is initially presumed to have IIH. Has the diagnosis of IIH become too easy, and are we over-diagnosing IIH in this period of an accelerating obesity crisis? Or are we actually missing cases of IIH because they do not fit the classic clinical profile? We think it is both: at the same time IIH is being diagnosed in excess in obese women without papilledema, often resulting in unnecessary procedures, inappropriate treatment and even iatrogenic complications, the spectrum of this disorder is expanding to include a broad array of clinical presentations that involve multiple specialists beyond just the ophthalmologist and neurologist.

摘要

在常规核磁共振成像出现前的时代, 特发性颅内高压 (IIH) 被认为是一种罕见且通常难以诊断的疾病, 如今已经成为眼科和神经科诊所日常的关注点, 特别是在后者, 基本上每个患有头痛的年轻超重女性最初都被推定为IIH。IIH的诊断是否变得过于简单, 在这个肥胖危机加速的时期, 我们是否过度诊断了IIH? 还是我们实际上遗漏了IIH的病例, 因为他们不符合经典的临床特征? 我们认为两者都是: 同时, IIH在无视神经乳头水肿的肥胖女性中被过度诊断, 往往导致不必要的操作、不恰当的治疗甚至是医源性并发症, 这种疾病疾病诊断的范围正在扩大, 包括广泛的临床表现。IIH的诊断除了与眼科医生和神经科医生有关之外, 还涉及到了多个领域的专家。

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Fig. 1: Spectrum of idiopathic intracranial hypertension.

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References

  1. Fisayo A, Bruce BB, Newman NJ, Biousse V. Overdiagnosis of idiopathic intracranial hypertension. Neurology. 2016;86:341–50.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Mollan SP, Mytton J, Tsermoulas G, Sinclair AJ. idiopathic intracranial hypertension: evaluation of admissions and emergency readmissions through the hospital episode statistic dataset between 2002-2020. Life. 2021;11:417.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Urbach H, Duman IE, Altenmüller DM, Fung C, Lützen N, Elsheikh S, et al. Idiopathic intracranial hypertension - a wider spectrum than headaches and blurred vision. Neuroradiol J. 2022;35:183–92.

    Article  CAS  PubMed  Google Scholar 

  4. Mollan SP, Aguiar M, Evison F, Frew E, Sinclair AJ. The expanding burden of idiopathic intracranial hypertension. Eye. 2019;33:478–85.

    Article  PubMed  Google Scholar 

  5. Miah L, Strafford H, Fonferko-Shadrach B, Hollinghurst J, Sawhney IM, Hadjikoutis S, et al. Incidence, prevalence and healthcare outcomes in idiopathic intracranial hypertension: a population study. Neurology. 2021;96:e1251–1261.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Ward ZJ, Bleich SN, Cradock AL, Barrett JL, Giles CM, Flax C, et al. Projected U.S. state-level prevalence of adult obesity and severe obesity. N Engl J Med. 2019;381:2440–50.

    Article  PubMed  Google Scholar 

  7. Ducros A, Biousse V. Headache arising from idiopathic changes in CSF pressure. Lancet Neurol. 2015;14:655–68.

    Article  PubMed  Google Scholar 

  8. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults in children. Neurology. 2013;81:1159–65.

    Article  PubMed  Google Scholar 

  9. Bidot S, Saindane AM, Peragallo JH, Bruce BB, Newman NJ, Biousse V. Brain imaging in idiopathic intracranial hypertension. J Neuroophthalmol. 2015;35:400–11.

    Article  PubMed  Google Scholar 

  10. Mallery RM, Rehmani OF, Woo JH, Chen YJ, Reddi S, Salzman KL, et al. Utility of magnetic resonance imaging features for improving the diagnosis of idiopathic intracranial hypertension without papilledema. J Neuroophthalmol. 2019;39:299–307.

    Article  PubMed  Google Scholar 

  11. Kwee RM, Kwee TC. Systematic review and meta-analysis of MRI signs for diagnosis of idiopathic intracranial hypertension. Eur J Radiol. 2019;116:106–15.

    Article  PubMed  Google Scholar 

  12. Chen BS, Newman NJ, Biousse V. Unusual manifestations of idiopathic intracranial hypertension. Taiwan J Ophthalmol. 2020;11:25–38.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Bidot S, Levy JM, Saindane AM, Oyesiku NM, Newman NJ, Biousse V. Do most patients with a spontaneous cerebrospinal fluid leak have idiopathic intracranial hypertension? J Neuroophthalmol. 2019;39:487–95.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Bidot S, Levy JM, Saindane AM, Narayana KM, Dattilo M, DelGaudio JM, et al. Spontaneous skull base cerebrospinal fluid leaks and their relationship to idiopathic intracranial hypertension. Am J Rhinol Allergy. 2021;35:36–43.

    Article  PubMed  Google Scholar 

  15. Vosoughi AR, Margolin EA, Micieli JA. Idiopathic intracranial hypertension: incidental discovery versus symptomatic presentation. J Neuro Ophthalmol. 2022;42:187–91.

    Article  Google Scholar 

  16. Chen BS, Meyer BI, Saindane AM, Bruce BB, Newman NJ, Biousse V. Prevalence of incidentally detected signs of intracranial hypertension on magnetic resonance imaging and their association with papilledema. JAMA Neurol. 2021;78:718–25.

    Article  PubMed  Google Scholar 

  17. Aung AB, Chen BS, Wicks J, Bruce BB, Meyer BI, Dattilo M, et al. presumptive idiopathic intracranial hypertension based on neuroimaging findings: a referral pattern study. J Neuroophthalmol. 2022. https://doi.org/10.1097/WNO.0000000000001660.

  18. Bouffard MA. Fulminant idiopathic intracranial hypertension. Curr Neurol Neurosci Rep. 2020;20:8.

    Article  PubMed  Google Scholar 

  19. Mollan SP, Moss HE, Hamann S. Evolving evidence in idiopathic intracranial hypertension. Life. 2021;11:1225.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Kuner AD, Kennedy TA. Beyond the AJR: isolated incidentally detected imaging findings associated with idiopathic intracranial hypertension are common in patients without papilledema. Am J Roentgenol. 2021. https://doi.org/10.2214/AJR.21.26469.

  21. Biousse V, Danesh-Meyer, HV, Saindane AM, Lamirel C, Newman NJ. Imaging of the optic nerve: technological advances and future prospects. Lancet Neurol. 2022. https://doi.org/10.1016/S1474-4422(22)00173-9.

  22. Aaron G, Doyle J, Vaphiades MS, Riley KO, Woodworth BA. Increased intracranial pressure in spontaneous CSF leak patients is not associated with papilledema. Otolaryngol Head Neck Surg. 2014;151:1061–6.

    Article  PubMed  Google Scholar 

  23. Buchowicz B, Chen BS, Bidot S, Bruce BB, Newman NJ, Saindane AM, et al. CSF-Leak Study Group. Prediction of postoperative risk of raised intracranial pressure after spontaneous skull base cerebrospinal fluid leak repair. J Neuroophthalmol. 2021;41:e490–e497.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Mollan SP, Grech O, Sinclair AJ. Headache attributed to idiopathic intracranial hypertension and persistent post-idiopathic intracranial hypertension headache: a narrative review. Headache. 2021;61:808–16.

    Article  PubMed  Google Scholar 

  25. Mollan SP, Mitchell JL, Ottridge RS, Aguiar M, Yiangou A, Alimajstorovic Z, et al. Effectiveness of bariatric surgery vs community weight management intervention for the treatment of idiopathic intracranial hypertension: a randomized clinical trial. JAMA Neurol. 2021;78:678–86.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Mollan SP, Mitchell JL, Yiangou A, Ottridge RS, Alimajstorovic Z, Cartwright DM, et al. Association of amount of weight lost after bariatric surgery with intracranial pressure in women with idiopathic intracranial hypertension. Neurology. 2022;99:e1090–e1099.

    Article  PubMed  PubMed Central  Google Scholar 

  27. Totten DJ, Schueth E, Saltagi MZ, Rabbani C, Harris AH, Tressman D, et al. Trends in spontaneous cerebrospinal fluid leak repairs in the United States, 2009-2018. Otol Neurotol Open. 2022;2:e021.

    Article  Google Scholar 

  28. Thaller M, Homer V, Hyder Y, Yiangou A, Liczkowski A, Fong AW, et al. The idiopathic intracranial hypertension prospective cohort study: evaluation of prognostic factors and outcomes. J Neurol. https://doi.org/10.1007/s00415-022-11402-6.

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Funding

There is no funding for this manuscript. VB and NJN are supported in part by NIH/NEI core grant P30-EY06360 (Department of Ophthalmology, Emory University School of Medicine), by and by a departmental grant from Research to Prevent Blindness.

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VB and NJN have equally contributed to the preparation of the manuscript, including review and selection of relevant publications, collection of data, preparation of the first draft, review of the final draft, and preparation of the figure.

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Correspondence to Nancy J. Newman.

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Biousse, V., Newman, N.J. The expanding spectrum of idiopathic intracranial hypertension. Eye 37, 2361–2364 (2023). https://doi.org/10.1038/s41433-022-02361-3

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