SHM Converge Scientific Abstract ePoster Gallery-Isolated Abducens Nerve Palsy as an Initial Manifestation of Neuro-Behçet's Disease

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This case report describes a rare initial neurologic manifestation of Neuro-Behçet’s disease (NBD): isolated abducens nerve palsy. Behçet’s disease is a systemic variable-vessel vasculitis typically causing oral/genital ulcers and uveitis, and NBD occurs in about 10% of patients. Although brainstem involvement is common in NBD, isolated cranial nerve VI palsy is distinctly uncommon.<br /><br />A 38-year-old woman with established Behçet’s disease, treated with hydroxychloroquine, developed 6 days of horizontal binocular diplopia on rightward gaze. She had no active oral or genital ulcers, uveitis, or other systemic flare symptoms. Examination showed isolated right abducens nerve palsy. Initial CT head, CTA, and CSF studies were unrevealing, and serum anti-MOG and AQP4 antibodies were negative. Brain MRI revealed a focal T2/FLAIR hyperintensity in the posterior right pons, localized to the facial colliculus at the level of the right abducens nucleus.<br /><br />The differential diagnosis included multiple sclerosis, neuromyelitis optica, ischemic stroke, and NBD. MS and NMO were considered unlikely because CSF and antibody testing were negative and there were no disseminated lesions. Stroke was also less likely given the lesion’s appearance and the rapid steroid responsiveness. The combination of known Behçet’s disease, a characteristic pontine lesion, and clinical improvement supported a diagnosis of parenchymal NBD.<br /><br />She was treated as an acute NBD flare with IV methylprednisolone 1 g/day for 5 days, followed by a 1-month oral prednisone taper. Her diplopia fully resolved by day 4. She remained symptom-free at 3- and 9-month follow-up, and 9-month MRI showed complete radiologic resolution of the pontine lesion.<br /><br />Key takeaway: in patients with known Behçet’s disease, unexplained isolated cranial neuropathy—especially CN VI palsy—should prompt evaluation for neuro-Behçet’s disease and early corticosteroid treatment.

Asset Subtitle

Nilesh Patel

Meta Tag

Author List Nilesh Patel

Category Clinical Vignettes

Concept Behçet Disease

Concept Neuro-Behçet Disease

Concept Abducens Nerve Palsy

Concept Parenchymal Neuro-Behçet Disease

Concept IV Methylprednisolone

Distinguished Non-Finalist

Presenter Organization University of Tennessee Medical Center

Presenting Author Nilesh Patel

Track Adult

Keywords

Neuro-Behçet’s disease

abducens nerve palsy

cranial nerve VI palsy

Behçet’s disease

pontine lesion

brainstem involvement

diplopia

methylprednisolone

corticosteroid treatment

facial colliculus

Behçet Disease

Neuro-Behçet Disease

Abducens Nerve Palsy

Parenchymal Neuro-Behçet Disease

IV Methylprednisolone