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. 2024 Jan;136(1-2):32-39.
doi: 10.1007/s00508-023-02252-x. Epub 2023 Aug 31.

The Vienna idiopathic intracranial hypertension database-An Austrian registry

Philip Pruckner  1   2 , Christoph Mitsch  3 , Stefan Macher  1   2 , Nik Krajnc  1   2 , Wolfgang Marik  4 , Klaus Novak  5 , Christian Wöber  1   2 , Berthold Pemp  3 , Gabriel Bsteh  6   7
Affiliations

The Vienna idiopathic intracranial hypertension database-An Austrian registry

Philip Pruckner et al. Wien Klin Wochenschr. 2024 Jan.

Abstract

Background: Idiopathic intracranial hypertension (IIH) is becoming increasingly more prevalent bearing the risk of visual impairment and affecting the quality of life. Clinical presentation and outcome are heterogeneous. Large, well-characterized cohorts are scarce.

Objective: To characterize the clinical spectrum, diagnostic findings, therapeutic management, and outcome of IIH.

Methods: We identified patients with IIH according to modified Friedman criteria treated at our center between 2014 and 2021. The Vienna IIH database is described in detail.

Results: Of 113 patients 89% were female (mean age 32.3 years). Median body mass index (BMI) was 31.8, with 85% overweight (BMI > 25) and 5% were classified as IIH without papilledema. Headache was present in 84% and showed migraine features in 43%. Median opening pressure in lumbar puncture was 31 cmH2O. Pharmacotherapy (predominantly acetazolamide) was established in 99%, 56% required at least 1 therapeutic lumbar puncture and 13% a surgical intervention. After a median 3.7 years follow-up, 57% had achieved significant weight loss, papilledema was present in 59% and headache in 76% (58% improved). Comparing initial presentation to follow-up, perimetry was abnormal in 67% vs. 50% (8% worsened, 24% improved) and transorbital sonography in 87% vs. 65% with a median optic nerve sheath diameter of 5.4 mm vs. 4.9 mm. Median peripapillary retinal nerve fiber layer thickness decreased from 199 µm to 99 µm and ganglion cell layer volume from 1.13 mm3 to 1.05 mm3.

Conclusion: The large representative Vienna IIH cohort characterizes IIH-related symptoms, diagnostic findings, treatment, and outcome emphasizing substantial long-term sequelae of IIH. Future analyses will aim to refine phenotyping and identify factors predicting outcome.

Keywords: Database; Diagnostic findings; Outcome; Pseudotumor cerebri; Treatment.

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Conflict of interest statement

P. Pruckner, C. Mitsch, S. Macher, W. Marik, K. Novak and B. Pemp declare that they have no competing interests. N. Krajnc: has participated in meetings sponsored by and received speaker honoraria or travel funding from Merck, Novartis and Roche, and held a grant for a Multiple Sclerosis Clinical Training Fellowship Programme from the European Committee for Treatment and Research in Multiple Sclerosis (ECTRIMS). C. Wöber: has received honoraria consultancy/speaking from Apomedica, Curelator, Eli Lilly, Grünenthal, Hermes, Novartis, Pfizer, and Ratiopharm/Teva. G. Bsteh: has participated in meetings sponsored by and received speaker honoraria or travel funding from Biogen, Celgene/BMS, Lilly, Merck, Novartis, Roche, Sanofi-Genzyme and Teva, and received honoraria for consulting from Biogen, Celgene/BMS, Novartis, Roche, Sanofi-Genzyme and Teva. He has received unrestricted research grants from Celgene/BMS and Novartis.

Figures

Fig. 1
Fig. 1
Overview outcome
See this image and copyright information in PMC

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