Neuro-Behçet disease (NBD) is a multisystem inflammatory disorder characterized by oral lesions, genital lesions, uveitis, and neurological deficits. If left untreated, it may lead to worsening neurological function and can be fatal. Here we present a case of a 52-year-old woman who was diagnosed with Behçet disease (BD) as a teenager and had a relatively mild disease course. Decades later after her initial DB diagnosis, she presented to our hospital with a chief complaint of headache. She did not have focal neurological deficits or any active mucosal lesions. Upon further investigation, the patient was found to have multiple inflammatory changes on neuroimaging and abnormal cerebrospinal fluid (CSF), consistent with the diagnosis of NBD. She was treated with intravenous corticosteroid therapy and her symptoms resolved. Although our patient presented with minimal symptoms decades after her initial diagnosis, any neurological complaint warranted a thorough investigation for a proper diagnosis and treatment given the multisystem involvement of BD.
Behçet disease (BD) is a multisystem disorder that is characterized by oral-genital ulcers and uveitis. In Neuro-Behçet disease (NBD), the central nervous system involvement can be categorized as either parenchymal or nonparenchymal [
Our patient is a 52-year-old Caucasian woman of German descent with a remote history of BD diagnosed as a teenager with recurrent oral lesions, genital lesions, and uveitis with only mild exacerbations over the past 30 years, who presented to us with a new onset headache. She reported that the headache started about 4 weeks before and it was intermittent at first but became constant 7 days prior to admission. She described the headache as pressure-like pain at the occipital regions bilaterally radiating to the vertex. Her headaches were associated with intermittent bilateral ear pain. She denied any focal weakness, nausea, vomiting, photophobia, or phonophobia. Aggravating factors for the headache included staring at the computer screen at work. She tried over-the-counter anti-inflammatories along with baclofen which helped initially. She denied any recent travel outside the US, had no recent sick contacts, reported infrequent alcohol use, and denied any toxic habits such as smoking and illicit drug use. She had no previous history of similar headaches personally or a family history of headaches. Physical examination, including a detailed neurologic examination, was within normal limits.
Of note, eight months prior to her presentation for evaluation of her headache, she had an episode of acute word-finding difficulties. She presented to our hospital and was admitted for a workup of suspected ischemic stroke. A brain MRI showed a focal T2 hyperintensity within the posterior left temporal lobe assumed to be due to an acute ischemic stroke. She was admitted and further workup was unrevealing of the etiology of her suspected ischemic stroke. As an outpatient, a repeat brain MRI one month after hospitalization showed resolution of this left temporal lesion. Clinically, the patient reported complete resolution of her word-finding difficulties.
On the admission to evaluate her headache, a repeat brain MRI showed T2 hyperintensities in the left thalamus, right mesial occipital lobe, and left brachium pontis (Figure
MRI brain T2 axial FLAIR sequence showed hyperintensities in the left thalamus and right mesial occipital lobe.
MRI cervical spine T2 sagittal sequence showed a hyperintensity extending from C1 to C2.
In regard to her past medical history of BD, she initially presented with recurrent oral ulcers and then a short time later developed recurrent genital ulcers and uveitis at age of 17. She was evaluated by multiple medical specialists and underwent an extensive workup. Subsequently, she was diagnosed with BD. She was started on oral prednisone, which she took for approximately 3 years and eventually self-discontinued because of prednisone side effects (weight gain, mood fluctuations). She had not had exacerbation with oral or eye lesions in over 30 years but did have infrequent bouts of genital ulcers. These exacerbations typically occurred in the setting of a stressor, such as an upper respiratory infection. Her last genital outbreak was one month prior to admission for headache. She had been tested before for HLA-B51, whose carriers have an increased risk of developing BD, but was negative and has no known family history of BD.
NBD disease is classified as either parenchymal or nonparenchymal. Parenchymal disease accounts for 75% of cases, and headache is the most common neurological symptom, occurring in about 70% of patients [
Just like clinical symptoms, MRI findings are also nonspecific in NBD. Typically, there is a single lesion during the acute phase, but the lesions can be diffuse and widespread during the chronic phase [
CSF analysis in NBD typically reveals increased cell counts and protein. CSF neutrophilia can be seen in the early stages with lymphocytosis being seen in the later stage [
Estimation of prognosis for NBD is based on many different factors. In patients with chronic progressive NBD, the presence of brainstem atrophy and abnormal CSF findings have a poorer prognosis [
Here we presented a patient with a myriad of uncommon NBD findings. She was a female over the age of 50 years who has had a relatively benign disease course of her BD for several decades. As mentioned above, NBD typically is diagnosed in the 3rd or 4th decade of life, presentation is 3 to 6 years after the onset of BD and it occurs more often in men. She did not present with systemic mucosal lesions or uveitis at the time of admission, contrary to the typical presentation of parenchymal NBD which often has concurrent systemic features of BD [
In conclusion, the diagnosis of NBD should be made cautiously in patients above the age of 50 years. Specifically excluding other more common neurological disorders, particularly stroke, is critical in obtaining a proper diagnosis. NBD should be considered in the differential diagnosis of any BD patient with new headache or other neurological complaints regardless of the prior status of their BD including disease duration and level of disability.
Wai Wai Miller and Demetrios Konstas declare that there are no competing interests regarding the publication of this paper. Chetan Gandhy has served as a consult for Genzyme and Teva Neuroscience and is on the speakers’ bureaus of EMD Serono and Genzyme. Derrick Robertson has served as a consultant for Biogen, Genzyme, Teva Neuroscience, and Pfizer, is on the speakers’ bureaus of Biogen, Pfizer, EMD Serono, Genzyme, Teva Neuroscience, Mallinckrodt, and Acorda; and has received grant support from Biogen, Genzyme, Novartis, Sun Pharma, MedImmune, Actelion, Mallinckrodt, EMD Serono, and Genetech.