Abstract Details

Title Clinical Characterization of Neuro-Behcet’s Disease in a North American Cohort

Topic Autoimmune Neurology

Presentation(s) P3 - Poster Session 3 (5:00 PM-6:00 PM)

Poster/Presentation
Number 2-010

Objective To characterize NBD in a US cohort and describe the clinical trajectory of the disease.

Background Neuro-Behçet’s disease (NBD) is a rare manifestation of Behçet’s disease with limited studies from non-endemic populations.

Design/Methods

Retrospective observational study of patients evaluated at Mass General Brigham hospital system with systemic Behcet’s disease (International Criteria for Behcet’s Disease) and neurological syndromes. Patients were also included if they had neuropathology and/or imaging consistent with Behcet disease with no alternate diagnoses on follow-up but did not meet scoring criteria.

Results 29 confirmed cases of neuro-Behcet’s disease (NBD) were identified (males 58.6%, females 41.4%) with median age of 34.3 years (IQR 28.0–48.5) at neurological syndrome onset. Of these, 20.7% had Mediterranean ancestry, 3.4% were Japanese, and 6.9% were of Middle Eastern origin, while 41.4% lacked a defined ethnic background. HLA-B51 positivity was observed in 29.2%. In 35.5% of patients, neurological manifestations preceded the diagnosis of systemic Behcet’s disease. There was a total of 65 relapses reported across all the patients. Hemiparesis or other motor deficits (55.2%) and headache (51.7%) were the most common presentations. Cerebral syndrome or encephalitis was the predominant phenotype (29.2%), followed by meningitis (27.7%), peripheral neurologic syndrome (18.5%), brainstem syndrome (15.4%), optic neuritis (5.2%), cerebral venous sinus thrombosis and intracranial hypertension (7.7% each). Cerebrospinal fluid showed pleocytosis (72.2%), lymphocytic predominance (69.2%) and elevated protein (75%) with no oligoclonal bands. Radiologically, the brainstem was most frequently involved (44.8%), followed by the thalamus and basal ganglia (34.5%), with contrast enhancement in 52.4% of lesions.

Conclusions Neuro-Behcet’s disease in this North American cohort typically affected younger adults with low prevalence of HLA-B51 and often of unclear ethnic background. NBD was frequently the initial presentation before known systemic Behcet’s. Along with cerebral syndromes and rhombencephalitis, peripheral nervous system syndromes were a common finding.

Authors/Disclosures
Avanteeka Ganguly PRESENTER	Miss Ganguly has nothing to disclose.
Joao Vitor Mahler, MD	Dr. Mahler has received research support from The Sumaira Foundation.
Philippe-Antoine Bilodeau, MD (Massachusetts General Hospital)	Dr. Bilodeau has or had stock in Roche. The institution of Dr. Bilodeau has received research support from Alexion Pharmaceuticals. The institution of Dr. Bilodeau has received research support from Department of Defense. Dr. Bilodeau has received research support from Canadian Institutes of Health Research. The institution of Dr. Bilodeau has received research support from Ann Theodore Foundation. Dr. Bilodeau has received research support from The MOG Project.
Shamik Bhattacharyya, MD, FAAN (Brigham and Women's Hospital)	Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NeuroLambda. Dr. Bhattacharyya has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion Pharmaceuticals. Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Bhattacharyya has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Continuum. Dr. Bhattacharyya has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Wiley. Dr. Bhattacharyya has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Merck. The institution of Dr. Bhattacharyya has received research support from Alexion Pharmaceuticals. The institution of Dr. Bhattacharyya has received research support from National Institute of Health. The institution of Dr. Bhattacharyya has received research support from UCB. The institution of Dr. Bhattacharyya has received research support from Genentech. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care.