Wendel, Eva-Maria and Tibussek, Daniel and Barisic, Nina and Bertolini, Annikki and Panzer, Andreas and Chang, Petrus and Geis, Tobias and Knierim, Ellen and Nikolaus, Marc and Nosadini, Margherita and Sartori, Stefano and Schoene-Bake, Jan-Christoph and Yilmaz, Deniz and Reindl, Markus and Pakeerathan, Thivya and Ayzenberg, Ilya and Rostasy, Kevin (2025) Children with MOG-IgG positive bilateral optic neuritis misdiagnosed as fulminant idiopathic intracranial hypertension. MULTIPLE SCLEROSIS AND RELATED DISORDERS, 93: 106205. ISSN 2211-0348, 2211-0356
Full text not available from this repository. (Request a copy)Abstract
Background: Fulminant idiopathic intracranial hypertension (IIH) is characterized by headache, rapid decrease of vision and elevated CSF-opening pressure. Objective: To delineate a subgroup of MOGAD mimicking fulminant IIH. Methods: In this case series children with MOGAD with vision loss, optic disc swelling and elevated CSF opening pressure, initially diagnosed with fulminant IIH, were included. Results: 8 MOGAD patients (median age 12.5y, f:m = 7:1) with an initial diagnosis of fulminant IIH were included. Rapid bilateral visual loss and headache was present in all patients in addition to bilateral optic disc swelling and decreased visual acuity. In 2 patients cMRI showed prominent optic discs or an empty sella sign. In 7 patients the CSF opening pressure was markedly elevated. Patients were treated with Acetazolamide (n = 6), CSF external drainage (n = 2) or implantation of VP shunt (n = 1). Intravenous Methylprednisolone was administered in all patients because of worsening of symptoms, positive MOG-ab titers and/or new MRI white matter lesions. Visual recovery was good with residuals in 1/8 children. OCT revealed thickening of pRNFL in the acute stage in all patients with a rapid declining of pRNFL and retinal atrophy thereafter. Conclusion: Children with bilateral MOGAD-ON presenting with loss of vision and elevated CSF opening pressure are at risk of being misdiagnosed as fulminant IIH. The role of elevated CSF opening pressure in MOGAD warrants further investigations. We recommend to include measurement of CSF opening pressure in the work up of neuroinflammatory diseases, in particular in patients with suspected MOGAD, to consider adapted pressure management.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | PSEUDOTUMOR CEREBRI SYNDROME; FLUID OPENING PRESSURE; DEMYELINATING DISEASE; CHILDHOOD; RESPONSES; Mogad; Bilateral on; Children; Idiopathic intracranial hypertension; Acquired demyelinating syndrome |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Kinder- und Jugendmedizin |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 31 Mar 2026 08:34 |
| Last Modified: | 31 Mar 2026 08:34 |
| URI: | https://pred.uni-regensburg.de/id/eprint/65289 |
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