A Case of Neuro-Behcet's Syndrome Without Genital Ulcers
Yongzhen Chen1, Sumona Banerjee1, Farid Khasiyev2, Benjamin Kiaei2, Sanhitha Valasareddy3, Adam Kilian3, Momina Soudagar-Turkey2
1Saint Louis University School of Medicine, 2Department of Neurology, 3Division of Rheumatology, Department of Internal Medicine, Saint Louis University School of Medicine
Objective:
Emphasize the significance of recognizing neuro-Behcet’s syndrome, even when genital ulcers are absent.
Background:

Behcet's disease is a recurring, multisystemic inflammatory disorder characterized by oral ulcers, genital ulcers, skin lesions, and ocular lesions. Neuro-Behcet’s syndrome is a condition in which individuals with Behcet's disease experience neurological symptoms that cannot be attributed to other neurological diseases. Very few reports in the literature document Behcet's disease without evidence of current or prior genital ulcers at the time of diagnosis.

Design/Methods:

We present a case of a 25-year-old male with past medical history notable for recurrent epididymitis, low-grade fevers, night sweats, recurrent painful oral ulcers, thrush, unintentional weight loss, and acneiform papulopustular rashes. He presented with acute-onset right internuclear ophthalmoplegia and binocular diplopia for one day. He had no genital ulcers on physical exam and denied a history of such ulcers.

Results:

Labs were unremarkable except for elevated serum C-reactive protein and erythrocyte sedimentation rate and cerebrospinal fluid white blood cells of 13 x 10^6/L. Brain MRI was notable for a hyperintensity noted on FLAIR and T1 in the pontine tegmentum region along the dorsal aspect of the pons, possibly involving the bilateral sixth nerve nuclei and facial colliculi. Given the constellation of symptoms along with the imaging findings, the patient was diagnosed with neuro-Behcet’s syndrome and started on a monthly cyclophosphamide infusion for a total of three doses as well as bi-monthly Adalimumab. Upon follow-up one month after discharge, his neurological symptoms had improved. 

Conclusions:
This rare case highlights that the presence of genital ulcers is not mandatory for diagnosing Behcet's diseases, despite being considered the second most common symptom at the time of diagnosis. In situations where patients exhibit neurological symptoms in the setting of recurrent oral ulcers, cutaneous lesions, and recurrent epididymitis, neuro-Behcet's syndrome should be promptly considered despite the lack of genital ulcers.
10.1212/WNL.0000000000205572