Related Experiment Video
Updated: Jan 17, 2026

Human Serum Anti-aquaporin-4 Immunoglobulin G Detection by Cell-based Assay
Published on: April 5, 2019
The cascade sign in aquaporin-4-IgG positive (AQP4-IgG+) NMOSD
Luigi Bonan1, Federico Camilli2, Marcello Bombardi1
1Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna, Bologna, Italy.
Background:
The cascade sign was reported in 2003 as pathognomonic of the parenchymal form of neuro-Behçet's. At the time, diencephalo-mesencephalic lesions in neuromyelitis optica (NMO) were largely ignored. Only with the 2015 revision of NMO spectrum disorders diagnostic criteria, were localizations in these areas fully acknowledged.
Case Report:
Here we report a case of AQP4-IgG+ NMOSD presenting with acute transverse myelitis, bilateral optic neuritis, with early brain magnetic resonance imaging (MRI) showing confluent diencephalo-mesencephalic lesions evoking the cascade sign (Figure 1, a-b).
Conclusion:
MRI presentation evoking the cascade sign might hinder the correct differential diagnosis and delay appropriate treatment in AQP4-IgG+ NMOSD.
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