Blurred Vision, Clear Clue: The Cascade Sign in a Child with Recurrent Optic Neuritis

Abstract

Neuro-Behçet’s disease (NBD) refers to a constellation of neurological signs and symptoms occurring in patients with Behçet's disease (BD). Pediatric NBD (PNBD) is extremely rare and can present with headache, meningitis, stroke, cerebral venous sinus thrombosis (CVST), psychiatric symptoms, and recurrent fever. Optic nerve involvement in BD has been reported. However, it almost always occurs alongside other features of BD or NBD, which either precede or follow ocular symptoms. Isolated ocular manifestations of PNBD are extremely rare. NBD can be associated with characteristic abnormalities in neuroimaging.
We present an unusual PNBD case with recurrent unilateral (left) optic neuritis (ON) without any systemic features or neurological manifestations. The diagnosis was made based on the Cascade sign visualized on Brain Magnetic Resonance Imaging (MRI), highlighting the importance of recognizing neuroimaging patterns. A differential diagnosis of PNBD should be considered in such unusual cases in which other causes of ON have been ruled out, and there are supporting diagnostic markers, such as human leukocyte antigen B51 (HLA-B51) positivity, with or without neuroimaging features of NBD. Early diagnosis ensures disease remission and prevents PNBD-associated morbidity.