A 29-year-old man presented with subacute new-onset headache, confusion, and hallucinations. On examination, he had obtundation and bilateral decreased visual acuity. Brain MRI revealed a T2/fluid-attenuated inversion recovery (FLAIR) hyperintense signal of the diencephalon and basal ganglia. Subsequently, he developed anterior uveitis with mobile hypopyon (Figure). Further questioning of his wife disclosed previous episodes of “red eyes,” acne-like skin lesions, and oral ulcers.
Figure. Ocular Findings and Neuroimaging.
External eye photograph depicts the mobile hypopyon (black arrow) and its movement within the anterior chamber right after the patient changed the position from standing to right lateral decubitus (curved arrow) (A). Brain MRI axial FLAIR-weighted image shows confluent hyperintense lesions in the diencephalon (B).
He was diagnosed with Behçet disease (BD) and fully recovered after a methylprednisolone pulse. The hallmarks of BD are oral and genital ulcers and uveitis. Neuro-BD often presents with cerebral venous thrombosis, aseptic meningitis, or mesodiencephalic venulitis. Although confluent diencephalic lesions on neuroimaging strongly indicate Neuro-BD, the diagnosis also relies on clinical findings.1 Hypopyon is the sedimentation of leukocytes in the anterior chamber of the eye, which manifests as a whitish fluid level. Mobile hypopyon is typical of NB and a relevant diagnostic clue.2
Appendix. Authors
Footnotes
Teaching slides links.lww.com/WNL/C476
Study Funding
The authors report no targeted funding.
Disclosure
The authors report no relevant disclosures. Go to Neurology.org/N for full disclosures.
References
- 1.Kalra S, Silman A, Akman-Demir G, et al. Diagnosis and management of Neuro-Behçet's disease: international consensus recommendations. J Neurol. 2014;261(9):1662-1676. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Zakka FR, Chang PY, Giuliari GP, Foster CS. Current trends in the management of ocular symptoms in Adamantiades-Behçet's disease. Clin Ophthalmol. 2009;3:567-579. doi: 10.2147/opth.s4445". [DOI] [PMC free article] [PubMed] [Google Scholar]