Abstract Details

Title Neurobehçet presenting with sympathetic ophthalmia and pseudotumoral brainstem lesion in afro-american patient.

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-048

Objective NA

Background Neurological involvement in Behçet disease (BD) has been reported to be 5%. Although common in Eurasia, BD is extremely rare in afro-american population in which presentation is typically severe with negative HLA B51.

Design/Methods NA

Results 37 year-old afro-american male with history of perforating trauma to the left eye in childhood, developed recurrent episodes of bilateral panuveitis when he was diagnosed with sympathetic ophtalmia at age 27. Patient was then started on systemic immunosuppression but had multiple relapses throughout the years due to poor compliance. At age 37, patient presented with subacute headaches, fatigue, nausea, vomiting and gait instability. Physical exam notable for dysarthria, mild upper extremity dysmetria and wide-based gait. Brain MRI demonstrated diffuse T2 and FLAIR hyperintensity in brainstem with multifocal nodular enhancements concerning for malignancy or inflammatory lesion. CSF demonstrated lymphocytic pleocytosis (32 total cells) and elevated protein (69 md/dl). Cytology and flow cytometry were negative for malignancy. Infectious, paraneoplastic and sarcoidosis work-up were negative. Patient was started on methylprednisolone for 3 days with partial recovery and was discharged. Two weeks later, patient was readmitted with same symptoms with interval MRI showing improvement of prior lesions and development of new enhancing lesions in midbrain. Systemic inflamatory markers were elevated. General exam was revealing for oral ulcers and lower extremitiy sterile pustules and a clinical diagnosis of BD was made. There was no evidence of genital ulcers or scars. HLA B51 and pathergy test were negative. Patient was started on infliximab with improvement of lesions.

Conclusions

Behçet's disease is rare in afro-american population and diagnosis can be delayed due to atypical presentation and negative HLA B51. Sympathetic ophthalmia is a devastating bilateral panuveitis that may represent the spectrum of uveitis in BD. Neurobehçet remains a challenging diagnosis and must be considered in patients with brainstem lesions and systemic inflammatory signs.

Authors/Disclosures
Thiago S. Carneiro, MD PRESENTER	Dr. Carneiro has nothing to disclose.
Melissa B. Pergakis, MD	Dr. Pergakis has nothing to disclose.
	No disclosure on file
Shuhan Zhu, MD	Dr. Zhu has nothing to disclose.

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