Clinical Characterization of Neuro-Behcet’s Disease in a North American Cohort
Avanteeka Ganguly1, Joao Vitor Mahler1, Philippe-Antoine Bilodeau2, Shamik Bhattacharyya3
1Neurology, Mass General Brigham, 2Massachusetts General Hospital, 3Brigham and Women's Hospital
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.
Disclaimer: Abstracts were not reviewed by Neurology® and do not reflect the views of Neurology® editors or staff.