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Recovery of vision in fulminant idiopathic intracranial hypertension

Andrew Micieli and Jonathan A. Micieli
CMAJ June 10, 2019 191 (23) E639; DOI: https://doi.org/10.1503/cmaj.190024
Andrew Micieli
Division of Neurology, Department of Medicine (A. Micieli and J.A. Micieli), Department of Ophthalmology and Vision Sciences (J.A. Micieli), University of Toronto, Toronto, Ont.; Kensington Vision and Research Centre (J.A. Micieli), Toronto, Ont.
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Jonathan A. Micieli
Division of Neurology, Department of Medicine (A. Micieli and J.A. Micieli), Department of Ophthalmology and Vision Sciences (J.A. Micieli), University of Toronto, Toronto, Ont.; Kensington Vision and Research Centre (J.A. Micieli), Toronto, Ont.
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  • Idiopathic intracranial hypertension: need to rule out cerebral venous sinus thrombosis
    Aravind Ganesh
    Published on: 10 June 2019
  • Published on: (10 June 2019)
    Idiopathic intracranial hypertension: need to rule out cerebral venous sinus thrombosis
    • Aravind Ganesh, Neurology resident, Associate fellow, Department of Clinical Neurosciences, University of Calgary; Centre for Prevention of Stroke and Dementia, University of Oxford

    I thank Drs Micieli for their report on fulminant idiopathic intracranial hypertension (IIH) which highlighted the importance of promptly considering interventions like optic nerve sheath fenestration or shunting in the setting of vision loss, rather than just standard medical therapy with acetazolamide.[1] However, a key caveat in the diagnostic process for IIH that is worth noting is the need to rule out secondary causes of intracranial hypertension, the most important one being cerebral venous sinus thrombosis (CVST) which can exactly mimic the clinical symptoms and exam findings of IIH. There are critical implications for management, as patients with CVST require anticoagulation. Consequently, the diagnostic criteria for IIH require normal neuroimaging including venography with no evidence of abnormal meningeal enhancement or venous thrombosis.[2] This can be accomplished using CT or MRI with venography, but the latter has the added advantage of also potentially demonstrating supportive features of IIH like flattening of the posterior globes, tortuous optic nerves, enlargement of the pre-optic optic subarachnoid space, empty sella turcica, or narrowing of the transverse venous sinuses. In the presence of three or more of these findings is required to make the diagnosis of suspected IIH in the absence of papilledema or sixth nerve palsy, although IIH without papilledema (IIHWOP) is a controversial entity.[3]

    References
    1. Micieli A, Micieli JA. Recovery of...

    Show More

    I thank Drs Micieli for their report on fulminant idiopathic intracranial hypertension (IIH) which highlighted the importance of promptly considering interventions like optic nerve sheath fenestration or shunting in the setting of vision loss, rather than just standard medical therapy with acetazolamide.[1] However, a key caveat in the diagnostic process for IIH that is worth noting is the need to rule out secondary causes of intracranial hypertension, the most important one being cerebral venous sinus thrombosis (CVST) which can exactly mimic the clinical symptoms and exam findings of IIH. There are critical implications for management, as patients with CVST require anticoagulation. Consequently, the diagnostic criteria for IIH require normal neuroimaging including venography with no evidence of abnormal meningeal enhancement or venous thrombosis.[2] This can be accomplished using CT or MRI with venography, but the latter has the added advantage of also potentially demonstrating supportive features of IIH like flattening of the posterior globes, tortuous optic nerves, enlargement of the pre-optic optic subarachnoid space, empty sella turcica, or narrowing of the transverse venous sinuses. In the presence of three or more of these findings is required to make the diagnosis of suspected IIH in the absence of papilledema or sixth nerve palsy, although IIH without papilledema (IIHWOP) is a controversial entity.[3]

    References
    1. Micieli A, Micieli JA. Recovery of vision in fulminant idiopathic intracranial hypertension. CMAJ 2019;191(23):E639.
    2. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013;81(13):1159–1165.
    3. Markey KA, Mollan SP, Jensen RH, Sinclair AJ. Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. Lancet Neurol 2016;15(1):78-91.

    Show Less
    Competing Interests: None declared.
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Canadian Medical Association Journal: 191 (23)
CMAJ
Vol. 191, Issue 23
10 Jun 2019
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Recovery of vision in fulminant idiopathic intracranial hypertension
Andrew Micieli, Jonathan A. Micieli
CMAJ Jun 2019, 191 (23) E639; DOI: 10.1503/cmaj.190024

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Recovery of vision in fulminant idiopathic intracranial hypertension
Andrew Micieli, Jonathan A. Micieli
CMAJ Jun 2019, 191 (23) E639; DOI: 10.1503/cmaj.190024
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