VCOM Research Day Program Book 2023

Medical Student Research Case Reports

11 Neuro Behcet’s – The Line Between Definite and Probable

Caitlin Yee, Beatrice Byrne, DO, Jeri Lantz, MD Corresponding author: cyee@vcom.edu

Edward Via College of Osteopathic Medicine-Virginia Campus VTC Carilion Roanoke Memorial Hospital

Behcet’s Syndrome (BS) is a multisystemic vasculitis that may be termed Neuro-Behcet’s Syndrome (NBS) when there is neurovascular involvement. While BS has well-established criteria for diagnosis, NBS has recently proposed criteria that introduce the subtypes of Definite NBS and Probable NBS . This case supplements the existing literature of previously diagnosed Probable NBS cases. We report a 54-year-old man who was admitted to our hospital for progressive multifocal strokes. His hospital course would involve 3 admissions over 39 days (33 days while admitted), with evolving discharge diagnoses that would eventually lead to Probable NBS. Upon the first admission, significant findings included a digital subtraction angiogram showing beading of the cerebrovasculature, which indicated primary CNS vasculitis. Considering etiologies of CNS vasculitis: bacterial, viral, connective tissue disorders, and systemic vasculitides were all ruled out; the exception was Behcet’s due to a positive HLA-B51 gene. Additionally, transesophageal echocardiography revealed a small patent foramen ovale (PFO). He was discharged with a diagnosis of CVA and probable cerebral vasculitis and started on prednisone 60 mg with plans to taper 10 mg every month. Upon the second admission, the patient returned due to altered mental status. MRI revealed new infarcts and the

patient was started on apixaban due to a possible association between PFO and cryptogenic strokes. The patient was alert and conversant upon discharge until he returned the next day presenting acutely with vertigo, nausea, and vomiting with a mild left-sided facial droop. Brain MRI upon admission showed new infarcts, thus PFO closure was performed. However, several days later, a repeat MRI showed still more numerous new infarcts. At this point, NBS was presumed and the patient was started on 1 week of pulse dose IV methylprednisolone followed by prednisone taper. The patient was eventually discharged to rehab for several weeks before returning home. He continues to be managed clinically by neurology and rheumatology with the diagnosis of Probable Neuro-Behcet’s Syndrome and has not experienced new infarcts since. Our patient presented with a neurological syndrome that meets the proposed criteria for Probable NBS . Thus, NBS may be considered in patients that present with nonspecific neurological disease even if ISG criteria are not satisfied. While NBS is rare, it continues to make its way into the differentials of young patients presenting with CNS vasculitis and stroke. Nevertheless, further studies are needed to validate the recommended criteria so that necessary treatment protocols may be executed.

Table 1 International consensus recommendation criteria for NBS Definite NBS – meets the following criteria 1. Satisfy the ISG criteria for BS 2. Neurologic syndrome is recognized to be caused by BS and supported by relevant and characteristic abnormalities seen on neuroimaging or CSF 3. No better explanation for neuro findings Probable NBS – meets one of the following criteria in the absence of a better explanation 1. Neurological syndrome as in definite NBS, with systemic BS features but not satisfying the ISG criteria A non-characteristic neurological syndrome occurring in the context of ISG criteria-supported BS

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