Neuro-Behçet's Syndrome Without Genital Ulcers: A Case Report and Literature Review

Abstract

Behçet's disease is a rare multisystemic vasculitis characterized by oral ulcers, genital ulcers, and skin and ocular lesions. Neuro-Behçet's syndrome is a condition in which individuals with Behçet's disease experience neurological symptoms that cannot be attributed to other neurological diseases. We present a rare case of neuro-Behçet's syndrome with acute internuclear ophthalmoplegia and deteriorating neurological function with a prior history of recurrent oral ulcers with pathergy-like features, acneiform papulopustular rash, retinal hemorrhages, and recurrent epididymitis without genital ulcers. Patient improved with cyclophosphamide. This case underscores the importance of diagnosing and managing neuro-Behçet's syndrome in the absence of genital ulcers.

Keywords: behçet's disease; epididymitis; genital ulcers; internuclear ophthalmoplegia; neuro-behçet's syndrome.

Figure 1. Brain MRI at initial presentation

A, B: hyperintensity in the pontine tegmentum along the dorsal pons in the fluid attenuated inversion recovery (FLAIR) sequence. C: subtle contrast enhancement in the dorsal pons in the post-contrast T1 sequence. D: mild hyperintensity in the right cerebral peduncle in the FLAIR sequence. E: a small focus of hyperintensity in the right thalamo-capsular junction in the FLAIR sequence.

Figure 2. Repeat brain MRI on Day 4 of methylprednisolone treatment

Repeat brain MRI on Day 4 of methylprednisolone treatment showing hyperintensities in the anterior medulla, dorsal pons, and dorsal midbrain in the fluid attenuated inversion recovery (FLAIR) sequence compared to MRI at initial presentation.