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IIH Provoke

CGRP-provoked headache is associated with a neuroimmune signature in idiopathic intracranial hypertension

Year of Publication: 2026

Authors: Yiangou A, Do TP, Grech O, ..., Sinclair AJ

Journal: J Headache Pain

Citation: Yiangou A et al. J Headache Pain. 2026;27(1). DOI: 10.1186/s10194-026-02385-0

Link: https://doi.org/10.1186/s10194-026-02385-0

Bottom Line

In a small (n=16) double-blind, placebo-controlled, two-way crossover provocation study, IV CGRP triggered typical IIH headache in 44% of women and was accompanied by elevations in 8 pro-nociceptive and immunoregulatory cytokines (CCL4, CCL8, IL-6, IL-17C, IL-27, OSM, HGF, TGF-α) that were not seen after placebo or in CGRP recipients without headache, supporting a neuroimmune component of CGRP-induced IIH headache.

Major Points

  • Double-blind, placebo-controlled, two-way crossover provocation study (parent trial IIH Provoke, ISRCTN13251508) in 16 women with IIH; 4 enrolled but did not complete both visits.
  • IV CGRP (1.5 µg/min x 20 min) provoked a typical IIH headache attack in 7/16 (44%); no participant developed headache during the placebo visit.
  • Eight cytokines were significantly elevated during the ictal phase vs baseline after CGRP: CCL4 (P=0.038), CCL8 (P=0.049), IL-6 (P=0.014), IL-17C (P=0.044), IL-27 (P<0.001), OSM (P=0.011), HGF (P=0.007) and TGF-α (P=0.042).
  • These cytokine changes were absent after placebo and in CGRP recipients who did not develop headache (H-), supporting specificity to the CGRP-provoked ictal phase rather than a direct CGRP pharmacologic effect.
  • FLT3LG decreased after both CGRP (P=0.022) and placebo (P=0.038) and CCL8 decreased after placebo (P=0.04), highlighting non-specific procedural/temporal effects.
  • Mechanistic interpretation: glial-immune activation, BBB modulation, vascular/tissue remodelling and trigeminal sensitisation in a primed neuroimmune state in IIH; exploratory and hypothesis-generating only.

Design

Study Type: Randomised, double-blind, placebo-controlled, two-way crossover mechanistic provocation RCT

Randomization: 1

Blinding: Double-blind (participants and investigators masked; drug prepared/administered by independent staff)

Enrollment Period: Parent trial IIH Provoke registered 24 Oct 2022 (ISRCTN13251508)

Follow-up Duration: Two inpatient visits ≥6 days apart; sampling baseline and peak headache or ~4 h post-infusion

Centers: 1

Countries: United Kingdom

Sample Size: 16

Analysis: Within-participant paired t-tests for baseline vs ictal/post-infusion cytokine comparisons; Pearson correlations for clinical associations; post-hoc Benjamini–Hochberg FDR sensitivity analysis


Inclusion Criteria

  • Female sex, age 18–60 years
  • Prior diagnosis of IIH per IIH consensus guideline criteria (papilloedema, lumbar puncture opening pressure ≥25 cm H₂O, normal neuroimaging except markers of raised intracranial pressure, normal CSF constituents, normal neurological examination except possible CN VI palsy)
  • Headache attributed to IIH per ICHD-3 criteria
  • Venous sinus thrombosis excluded on neuroimaging
  • Able to attend two inpatient visits and provide written informed consent

Exclusion Criteria

  • History of migraine predating IIH diagnosis
  • >1 day/month of non-IIH headache
  • Pregnancy
  • Significant comorbidity, including cardiovascular or cerebrovascular disease
  • Medication overuse
  • Use of GLP-1 receptor agonists or other immune-modulating medications
  • Spontaneous headache exacerbation within 72 h of a scheduled visit (visit deferred)

Baseline Characteristics

All Participants (n=16, crossover design):

  • Mean Age (years): 26.2 (SD 6.3)
  • Mean Weight (kg): 107.1 (SD 23.0)
  • Mean BMI (kg/m²): 39.2 (SD 8.4)
  • Time from IIH diagnosis (months): 17.4 (SD 19.6)
  • Papilloedema OCT RNFL worst eye (µm): 145.5 (SD 58.5)
  • Monthly headache days: 24.9 (SD 8.3)
  • Non-migrainous headache severity (NRS): 5.9 (SD 2.9)
  • Monthly migraine-like days: 16.3 (SD 9.4)
  • Migraine-like day severity (NRS): 9.0 (SD 1.5)
  • Ethnicity - White British: 13 (81%)
  • Ethnicity - Mixed White & Black Caribbean: 3 (19%)
  • Headache phenotype - Migraine-like overall: 15 (94%)
  • Headache phenotype - Episodic migraine-like: 2 (13%)
  • Headache phenotype - Chronic migraine-like: 13 (81%)
  • Headache phenotype - Tension-type-like: 1 (6%)
  • Family history of migraine: 4 (25%)
  • Acetazolamide use: 3 (19%)
  • Migraine preventative use: 3 (19%)
  • Triptan use: 5 (31%)
  • Simple analgesic use: 12 (75%)

Arms

FieldCGRPControl
n1616
InterventionIV human α-CGRP 1.5 µg/min infusion for 20 minutesIV isotonic saline matched infusion for 20 minutes
DurationSingle 20-minute infusion; inpatient observation ~4 hSingle 20-minute infusion; inpatient observation ~4 h

Outcomes

OutcomeTypeControlInterventionHR / OR / RRP-value
Change in plasma cytokine levels (Olink Target 48 PEA panel, 45 proteins; 38 passing QC) from baseline (BL) to peak headache (H+) during the ictal phase after CGRP infusion in participants who developed a typical IIH headache (n=7)PrimaryMultiple; see description
CGRP-provoked typical IIH headache attack within sampling periodSecondaryNot formally tested
Mean time from CGRP infusion start to peak headache sample (H+)SecondaryDescriptive
Mean time to end-of-visit sample (H-) in headache-free participantsSecondaryDescriptive
Cytokine changes after CGRP in participants without headache (H-, n=9)SecondaryNon-significant
Correlation between cytokine changes and headache severity (NRS) in H+ groupSecondaryNon-significant
Period/carryover effect (baseline cytokines by visit order)SecondaryNon-significant
CommentAdverseNo formal safety table was presented in this exploratory mechanistic sub-analysis. The 44% incidence of provoked IIH headache after CGRP (vs 0% after placebo) is the studied physiological response rather than an adverse event. No serious adverse events were reported. 7 of 45 cytokines (CSF2, IL-17A, IL-17F, IL-1β, IL-2, IL-33, TSLP) were below the lower limit of quantification and excluded from analysis.

Subgroup Analysis

Within-CGRP comparison of headache responders (H+, n=7) vs non-responders (H-, n=9) showed no baseline cytokine differences. Comparison of baseline cytokines by randomisation order showed no carryover/period effects.


Criticisms

  • Very small sample size (n=16 overall, n=7 in the primary ictal analysis) limits statistical power and precludes correction-robust inferences
  • Single-centre (UK) and all-female, predominantly White British population — limits generalisability across sex, ethnicity, and healthcare setting
  • Provoked rather than spontaneous IIH headache attacks; experimental ictal pathophysiology may differ from naturally occurring attacks
  • Peripheral plasma cytokines may not reflect central CSF/meningeal neuroimmune events
  • Exploratory analysis without prespecified correction for multiple comparisons; FDR adjustments provided only as post-hoc sensitivity analysis
  • 7 of 45 cytokines below the limit of quantification (including IL-17A, IL-1β, IL-33, TSLP) restricted the breadth of the inflammatory profile
  • Study design cannot establish whether cytokine elevations cause headache or are downstream consequences of CGRP-induced trigeminovascular activation

Funding

Parent IIH Provoke trial (ISRCTN13251508). Author affiliations include NIHR Birmingham Biomedical Research Centre. Detailed funding source and conflicts of interest declared in the full paper.

Based on: IIH Provoke (J Headache Pain, 2026)

Authors: Yiangou A, Do TP, Grech O, ..., Sinclair AJ

Citation: Yiangou A et al. J Headache Pain. 2026;27(1). DOI: 10.1186/s10194-026-02385-0

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