neuro-Behcet's Disease Research Articles

Neuro-Behcet's Masquerading as Status Epilepticus and Meningoencephalitis in the Emergency Department

BackgroundBehcet disease (BD) is a rare small vessel vasculitis that commonly manifests as recurrent painful oral or genital ulcerations, uveitis, and skin lesions. Some patients with BD develop neurological symptoms termed neuro-Behcet's disease. In the emergency department (ED) setting, these symptoms can be mistaken for other common acute issues including stroke, infection, epilepsy, multiple sclerosis, toxin ingestion, or psychiatric conditions. Case ReportWe present a case of a 28-year-old male with neuro-Behcet disease mimicking status epilepticus and meningoencephalitis. He was actively seizing on arrival and febrile at 103.8 F. The patient also had a history of vasculitis, uveitis and genital lesions which raised suspicion for a rheumatological process. Cerebrospinal fluid (CSF) samples were obtained and computed tomography (CT) of the head revealed no acute findings. The patient was started on treatment for seizure and meningoencephalitis and admitted to neurology for continuous electroencephalogram (EEG) monitoring with additional consults placed for rheumatology, ophthalmology, and infectious disease. The patient was ultimately diagnosed with neuro-Behcet's disease and treated appropriately.Why should an emergency physician be aware of this?: In a patient presenting with neurological symptoms and signs of rheumatological disease providers should consider neuro-Behcet's disease as a differential diagnosis. This case exemplifies the vital role of reviewing past medical history to expand differential diagnoses and early coordination with specialists so treatment can be initiated early to prevent morbidity and mortality. The case will also review different presentations of this unfamiliar diagnosis for a more comprehensive understanding.

Epidemiology of Neuro-Behçet's Disease in Northern Spain 1999-2019: A Population-Based Study.

Background/Objectives: Neuro-Behçet's disease (NBD) is one of the most severe complications of Behçet's disease (BD). The incidence of NBD varies widely worldwide. This study aimed to estimate its current incidence in Northern Spain. Methods: This was a retrospective population-based cohort study of 120 patients in Northern Spain diagnosed with BD according to the 2013 International Criteria for BD (ICBD) between 1 January 1999 and 31 December 2019. NBD diagnoses were made according to International Consensus Recommendation (ICR) criteria. Overall, 96 patients were included, and their demographic and clinical data were collected. The incidence of NBD was estimated by age, gender, and year of diagnosis between 1999-2019. Results: NBD was diagnosed in 23 of 96 (24%) patients (15 women/8 men) (mean age: 44 ± 13.9 years). HLA-B51 was positive in 5 of 13 (38.4%) cases tested. A total of 10 (43.5%) patients had parenchymatous NBD, 10 (43.5%) had non-parenchymatous NBD, and 3 (13%) had mixed NBD. Incidence during the study period was 0.13 (95% CI, 0.11-0.26) per 100,000 people-years. There were no significant differences in gender in the incidence rate stratified by age (p > 0.05). Furthermore, there was a linear relationship with a mild decrease in age at diagnosis over time. Conclusions: Epidemiological characteristics of NBD in Northern Spain are similar to those of neighboring countries, except female gender predominance.

Diffusion tensor imaging in Behcet's disease with and without neurological involvement patients: evaluation of microstructural white matter abnormality with a tract-based spatial statistical analysis.

This study aims to assess the microstructural abnormalities in white matter (WM) among Behcet's disease (BD) patients, both with and without neurological involvement, utilising tract-based spatial statistics (TBSS) to elucidate the underlying causes of WM microstructural changes. This prospective study comprised 43 BD patients without neurological involvement, 15 neuro-Behcet's disease (NBD) patients with normal conventional MRI, and 54 healthy controls matched for age and sex. TBSS was applied in this diffusion tensor imaging study to conduct a whole-brain voxel-wise analysis of fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) of WM. Compared to the control group, BD patients exhibited decreased FA and increased MD and RD in nearly all WM tracts, along with increased AD in the left corticospinal tract (CST), left inferior longitudinal fasciculus (ILF), and left superior longitudinal fasciculus (SLF). NBD patients also showed a widespread decrease in FA and increased MD and RD, similar to BD patients without neurological involvement. Additionally, NBD patients had increased AD in the left CST, left ILF, left SLF, left inferior fronto-occipital fasciculus (IFOF), and right CST. Compared to BD patients without neurological involvement, NBD patients exhibited a greater reduction in FA and an increase in MD and RD in WM tracts, with no significant differences in AD. These results suggest that the main mechanism of microstructural changes in the WM of BD patients may be related to impaired fibre integrity, demyelination, and decreased myelin sheath integrity. This study demonstrated BD patients without neurological involvement and NBD patients a decrease in FA and an increase in MD and RD were observed in larger areas of major WM tracts, while an increase in AD values was observed in fewer tracts. Our findings may be useful in understanding the pathophysiology underlying subclinical parenchymal involvement and neurological dysfunction in BD patients and the management of BD patients.

A case of neuro-Behcet's disease complicated with intra-cardiac thrombosis

Intra-cardiac thrombus is an unusual and rare complication of Behcet’s (BD) with poor prognosis. A 32-year-old man with a history of mouth and genital ulcers since 12 years ago was admitted to the neurology ward with complaints of headache and fever. Two years ago, the patient suffered from hydrocephalus due to cerebral vein thrombosis and a cerebral shunt was implanted for him. The patient was diagnosed with BD and was treated with glucocorticoids and cytotoxic drugs. During current hospitalization, with the diagnosis of shunt infection, the patient was treated with broad-spectrum antibiotics and the shunt was removed. On the 10th day of hospitalization, he developed cough and palpitation without dyspnea. Echocardiography showed a 2.5 × 4.7 mm mass in the right ventricle, tricuspid regurgitation and pulmonary arterial pressure of 32 mmHg. On computed tomography of the chest, the lung parenchyma was normal, but extension of the thrombus into the main pulmonary artery was reported.

Neuro-Behcet’s Disease Manifesting as Bilateral Fornix and Left Thalamus Infarction

Neuro-Behcet’s disease (NBD) is defined as neurological involvement in Behcet’s disease. A 32-year-old man was admitted due to sudden memory impairment and had a history of recurrent oral/genital ulcers. Brain magnetic resonance imaging (MRI) revealed ischemic lesions in the bilateral fornix and left thalamus, and high-resolution vessel wall MRI demonstrated vasculitis in the anterior communicating artery. We report a rare case of NBD that presented as vasculitis and ischemic stroke in the bilateral fornix and thalamus.

Neurological and Vascular Behçet's Disease in a Young Male without Classic Triad of Behçet's Disease: A Case Report and Literature Review.

Neuro-Behçet's disease (NBD) is an uncommon presentation in Behçet's disease (BD) with severe course and worse prognosis. Both vascular and NBD presentation without the classical triad of BD in a single patient is rarely reported. Here a 48-year-old male had an extensive aortic aneurysm eroding vertebra for which he was diagnosed as vascular BD. Two years later, he was presented with a severe headache and cerebrovascular accident, his brain imaging showed hyperintensity in the right thalamus, basal ganglia, temporal lobe, and internal capsule, suggesting the 'cascade sign' of NBD. Surprisingly, he never had oral or genital ulcers or skin and eye involvement. He had a good response to infliximab. Clustering of BD phenotype is an emerging area of interest. It is hypothesised that severe phenotype of vascular and parenchymal NBD can happen in the same patient owing to similar underlying pathology. This case is unique due to its severe phenotype with no features of the typical triad of BD.

Effect of Infliximab on Chronic Progressive Neuro-Behçet's Disease: Influence of the Timing of Introduction on the Patient Outcome.

Objectives Chronic progressive neuro-Behcet's disease (CPNB) is characterized by progressive deterioration leading to disability. Methotrexate (MTX) has been shown to have beneficial effects on CPNB. However, while infliximab has been found to be effective for patients with inadequate responses to MTX, the appropriate timing for the introduction of infliximab remains unclear. We explored the effects of intervals before the introduction of infliximab on the functional outcome. Methods A retrospective analysis was performed for patients with CPNB who received infliximab and were followed up until October 2015. Functional disability was rated by the Steinbrocker functional classification as used in rheumatoid arthritis. Correlations between the outcomes and intervals before the introduction of infliximab were then analyzed by Spearman's rank correlation test. Patients Eleven patients with CPNB [8 men, 3 women, age 35.2±9.3 years old (mean±standard deviation)] who met the international classification criteria for Behcet's disease were included. Results All 11 patients had received MTX prior to infliximab. The intervals from the onset to the introduction of infliximab and the follow-up periods were 26.6±35.1 months and 65.2±43.6 months [mean±standard deviation], respectively. Among the 11 patients, 2 still showed progression after the introduction of infliximab. The functional disability grades after infliximab treatment were significantly correlated with the intervals from the onset of CPNB to the introduction of infliximab (r=0.6177, p=0.0476). Conclusion The results indicate that the delayed introduction of infliximab leads to irreversible functional disability in CPNB. Thus, it is recommended that infliximab be administered as soon as possible for CPNB patients with inadequate responses to MTX.

Neurobehcet’s Disease with Extensive Invasion of Cerebral Parenchyma

Neuro-Behcet’s disease (NBD) is defined as a combination of neurologic symptoms and/or signs in a patient with Behcet’s disease. A 38-year-old woman was admitted due to sensory aphasia. She had past medical history of generalized tonic-clonic seizure, retinal vasculitis, oral ulcer and cerebral vasculitis. Brain magnetic resonance imaging showed broad lesion in the left parieto-occipital lobe. Brain biopsy was performed to differentiate between central nervous system lymphoma and NBD. We report a rare case of NBD with extensive involvement of cerebral parenchyma.

Indications for Magnetic Resonance Imaging in Patients With Behcet Uveitis.

Behcet disease is a systemic vasculitis, which may involve the eyes and central nervous system. The true prevalence of neurological involvement is not precisely known but may be associated with ocular involvement. This study investigates the association between Behcet uveitis and neuro-Behcet disease. A retrospective single-center analysis was conducted for consecutive patients with Behcet uveitis at the Massachusetts Eye Research and Surgery Institution. Uveitis characteristics, neurological symptoms, fluorescein fundus angiography, and MRI results were recorded. Our population included 108 patients with Behcet uveitis, and 26 (24.1%) were found to have neurological involvement associated with Behcet disease. Optic nerve leakage on fundus angiography and neurological symptoms were associated with an increased risk of neurological involvement. Three cases (11.5%) were nonparenchymal, while 23 (88.5%) were parenchymal with lesions in the cortex, subcortical white matter, thalamus, basal ganglia, and brainstem. There is a high comorbidity between ocular and neurological involvement in Behcet disease. Careful assessment of neurological symptoms and baseline fluorescein fundus angiography are recommended for patients with Behcet disease. MRI has a high diagnostic yield and should be pursued if there is concern for progressive or pre-existing neurological involvement.

Perceived Stress Associated With COVID-19 in Patients With Neuro-Behcet's Disease.

Perceived Stress Associated With COVID-19 in Patients With Neuro-Behcet's Disease.

Analysis of the clinical characteristics of 25 cases with parenchymal neuro-Behcet's disease

Objective: To retrospectively investigate the clinical data, radiological characteristics, treatment, and outcome of patients with parenchymal neuro-Behcet's disease (P-NBD) with particular emphasis on dizziness. Methods: This was a cross-sectional study of clinical data from 25 patients with a confirmed diagnosis of P-NBD who were admitted to the Department of Neurology of the First Medical Center of Chinese People's Liberation Army General Hospital between 2010 and 2022. The median age of the population was 37 years (range: 17-85 years). Clinical data were retrospectively analyzed, including gender, age of onset, disease duration, clinical manifestations, serum immune indicators, cerebrospinal fluid (CSF) routine biochemical and cytokine levels, cranial and spinal magnetic resonance imaging (MRI) findings, treatment, and outcome. Results: The majority of patients were male (16 cases; 64.0%), the mean age of onset was (28±14) (range: 4-58 years), and the disease course was acute or subacute. Fever was the most common clinical presentation, and the complaint of dizziness was not uncommon (8/25 patients). Analysis of serum immune indices, including complement (C3 and C4), erythrocyte sedimentation rate, interleukin-1 (IL-1), IL-6, IL-8 and tumor necrotic factor-alpha were abnormal in 80.0% of patients (20/25). Most of the 16/25 patients who underwent lumbar puncture tests had normal intracranial pressure and increased CSF white cell count and protein [median values were 44 (15-380) ×106/L and 0.73 (0.49-2.81) g/L, respectively]. Of the five patients who underwent CSF cytokine tests, four patients had abnormal results; of these, an elevated level of IL-6 was most common, followed by IL-1 and IL-8. The most common site of involvement in cranial MRI was the brainstem and basal ganglia (60.0% respectively), followed by white matter (48.0%) and the cortex (44.0%). Nine cases (36.0%) showed lesions with enhancement and six cases (24.0%) showed mass-like lesions. Three patients (12.0%) patients had lesions in the spinal cord, most frequently in the thoracic cord. All patients received immunological intervention therapy; during follow up, the majority had a favorable outcome. Conclusions: P-NBD is an autoimmune disease with multiple system involvement and diverse clinical manifestations. The symptom of dizziness is not uncommon and can be easily ignored. Early treatment with immunotherapy is important and can improve the outcome of these patients.

DP10 A relapsing–remitting rash with associated neurological symptoms: a rare presentation of ​­neuro-Sweet syndrome

Abstract A 46-year-old woman presented with an 8-week history of an asymptomatic eruption on her upper extremities and back. She had a history of ulcerative colitis, asthma and hyposplenism. There had been no changes to her regular medications. Over the next 3 years, she developed a relapsing and remitting course of headaches, pyrexia and feeling unwell with the associated rash. She further developed deafness and vertigo and was admitted with headaches, blurred vision and paraesthesia of the fingers. Physical examination revealed multiple erythematous, asymptomatic, annular plaques present on her upper extremities and back with no oral, genital or ophthalmological lesions. Skin biopsy from the lesion on the upper back showed a neutrophilic dermatosis with mildly acanthotic epidermis, superficial dermal collection of acute inflammatory cells, dermal oedema and endothelial swelling, with no evidence of vasculitis. This was considered in keeping with Sweet syndrome. Magnetic resonance imaging of the brain revealed meningoencephalitis and focal cerebritis. A lumbar puncture performed for infection was negative. Following this, a diagnosis of neuro-Sweet syndrome (NSS) was made. She has responded well to oral steroids and is currently well controlled on dapsone. To date, extensive malignancy screens have been negative. Sweet syndrome, or acute febrile neutrophilic dermatosis, is characterized by fever, malaise, leucocytosis and painful distinctive skin lesions and may be associated with malignancies, drugs and infection. Skin biopsy shows dense dermal infiltration of neutrophils without any histological signs of vasculitis. Sweet syndrome is known to involve multiple organs; however, involvement of the central nervous system (CNS) is rare. When the CNS is involved, it is referred to as NSS and is defined as the coexistence of characteristic skin lesions of Sweet syndrome with encephalitis or meningitis (Suh HB, Kim HJ. Recurrent neuro-Sweet disease associated with preceding upper respiratory infection: a case study. iMRI 2018; 22:187–93). Although the condition is usually steroid responsive, disease recurrences have been reported in 50% of cases in the literature (Hisanaga K, Iwasaki Y, Itoyama Y, Neuro-Sweet Study Group. Neuro-Sweet disease: clinical manifestations and criteria for diagnosis. Neurology 2005; 64:1756–61). It is important to note that the clinical features and imaging results in NSS are similar to those of neuro-Behçet disease (NBD), and it is important to distinguish between the two as CNS involvement in NSS is often transient, whereas NBD often follows a more progressive course. We report a rare case of NSS and highlight that NSS should be considered by dermatologists in patients presenting with recurrent skin lesions of Sweet syndrome and associated neurological symptoms.

Bagel sign in neuro Behçet's disease myelitis: neuroimages.

Neuro-Behçet's disease (NBD) is a chronic heterogenous autoimmune disorder. It may involve central or pripheral nervous system but rarely shows spinal cord involvement (SCI). Bagel Sign is a unique sign of SCI due to NBD. It is a central T2W hyperintense lesion with a hypointense core on axial magnetic resonance imaging (MRI). This sign may be a complete or an incomplete ring with or without post-contrast enhancement. Here we report a patient with NBD whose primary presentaion was transverse myelitis. A 14-year-old patient was admitted due to triparesis and urinary retention. He had T2W hyperintensities from the left basal ganglion down to the T10 level. A similar anteromedial spot has been described in anterior spinal cord infarction which favors an ischemic pathogenesis for NBD. To our knowledge this is the first report of Bagel Sign and longitudinally extensive transverse myelitis with gray matter involvement.

Myelin basic protein and index for neuro-Behçet's disease

Neuro-Behçet's disease (NBD) contributes to poor prognosis in BD patients which lacks reliable laboratory biomarkers in assessing intrathecal injury. This study aimed to determine the diagnostic value of myelin basic protein (MBP), an indicator of central nervous system (CNS) myelin damage, in NBD patients and disease controls. Paired samples of cerebrospinal fluid (CSF) and serum MBP were measured using ELISA, while IgG and Alb were routinely examined before the MBP index was developed. CSF and serum MBP in NBD were significantly higher than in NIND, which could distinguish NBD from NIND with a specificity exceeding 90%, moreover, they could also be excellent discriminators for acute NBD and chronic progressive ones. We found positive linkage between MBP index and IgG index. Serial MBP monitoring confirmed serum MBP's sensitive response to disease recurrences and drug effects, whereas MBP index suggests relapses prior to clinical symptoms. MBP has high diagnostic yield for NBD with demyelination and identifies CNS pathogenic processes before imaging or clinical diagnosis.

Claude Syndrome in Childhood Associated with Probable Neuro-Behcet Disease.

Claude syndrome is a rare midbrain stroke syndrome characterized by ipsilateral third cranial nerve palsy and contralateral hemiataxia. So far, only a few cases have been reported in childhood. We present two children with Claude syndrome at 9 and 15 years of age. The typical clinical picture was consistent with brain magnetic resonance imaging findings. A thorough investigation regarding the underlying etiology revealed no definite diagnosis but clues suggestive of probable neuro-Behcet disease. Awareness of pediatric neurologists on arterial ischemic stroke has been increasing over the past decades, enabling timely diagnosis and appropriate management of rare childhood cases with midbrain stroke.

Neuro-Behcet's Disease Presenting as a Psuedotumoral Brainstem Mass: A Case Report

Objective Herein, we present a rare and diagnostically challenging case of neuro-Behcet disease (NBD) manifesting as a psuedotumoral brainstem mass. Background Psuedotumoral-NBD as a neurologic manifestation of Behcet's disease is rare. Imaging is characterized by mass-like lesions that enhance with contrast, are hyperintense with T2-weighted and fluid-attenuated inversion recovery (FLAIR) and show restricted diffusion. The differential includes glial lesions, lymphomas, infectious and granulomatous lesions. Design/Methods This is a case study of a 33-year-old male of West-African descent with a history of Behcet's disease. Results A 33-year-old male of West-African descent with a history of Behcet's disease, presented two years after his diagnosis with headaches, low-grade fever, genital ulcerations, and horizontal binocular diplopia. Imaging revealed a large right-sided T2/FLAIR hyperintense abnormality in the medulla with a central area of necrosis. Cerebrospinal fluid revealed lymphocytic-predominant pleocytosis with 11·106 cells/L (reference range 0-5), and high levels of interleukin-6. His vasculitis, infectious, paraneoplastic, flow cytometry, and autoimmune panels were negative. He tested positive for hepatitis-B core antigen, and latent tuberculosis. The etiology for his presentation was believed to be parenchymal NBD and he received a 3-day course of intravenous solumedrol (eventually transitioned to prednisone and azathioprine) with significant improvement. Imaging 1-week post-treatment revealed resolution of enhancement, and at 3 months he had near complete lesion resolution. Conclusions NBD can rarely present with a psuedotumoral presentation, which can cause diagnostic uncertainty. A thorough radiologic/laboratory workup should be conducted to exclude other neurologic diagnoses; however, a high index of suspicion for NBD is required in similar cases and a spectacular response to steroids are invaluable in diagnosis.

Cerebral venous thrombosis in adolescence

Behçet's disease (BD) is a rare systemic vasculitis with multisystemic involvement. Neurological involvement, called neuro-Behçet's disease (NBD), mostly involves the central nervous system and cerebral venous thrombosis (CVT) is the predominant neurological manifestation in the pediatric age. A 12-year-old female with a past medical history of a CVT, without an identifiable etiology, was admitted with a five-day right fronto-orbital headache. Neuroimage showed a subacute thrombosis of a right superficial sylvian vein, with indirect signs of intracranial hypertension and no imaging signs of vasculitis. Prothrombotic screening and immunologic study were normal. She was started on acetazolamide and hypocoagulation with progressively improving. She had a history of frequent oral aphthae and an episode of a genital ulcer three months before admission. Pathergy test was negative. HLA-B51 was positive. She was diagnosed with NBD and started therapy with colchicine and infliximab. After discharge, the patient remains without symptoms, hypocoagulated, and on infliximab regimen, without complications to report. This case, only diagnosed in the second episode of CVT, is paradigmatic of the difficulty in establishing the diagnosis of BD.

Ghost lesions of the central nervous system: Misleading or revealing the diagnosis?

Vanishing lesions of the CNS also known as Ghost lesions can be a radiological feature of various neurological disorders mainly tumoral followed by inflammatory diseases of the CNS. Vasculitis such as Behcet Disease (BD) is considered as a leading cause of demyelinating disorders with ghost lesions on brain MRI. As a result, following a specific clinical approach is pivotal for definitive diagnosis in order to adapt the treatment and assess the outcome. Here, we report 3 cases of ghost lesions on cerebral MRI as a radiological finding in CNS vasculitis: Neurobehcet Disease (NB).

Delirium: Fallout of Neuro-Behcet’s Disease – A Case Report

Here, we present a case of an elderly male with hypertension and diabetes, who presented to ER with delirium, ataxia, and slurring of speech associated with altered psychomotor behaviour, also features such as anorexia, painful erythematous patches over extremities, tender knee, and ankle joints were conspicuous at presentation. A brain scan on admission revealed multiple lacunar cerebellar infarcts, whereas routine blood investigations did not reveal anything substantial contributions to the cause of presenting symptoms, including negative serology for ANA, DS-DNA, HAL-B51, and anticardiolipin antibodies, done to rule in, presence of systemic autoimmune causes for the gamut of clinical features at presentation. Contemplation over past and associated clinical features, such as ocular ailment, recurrent aphthous ulcers, dermatological lesions (erythema nodosum and peudofolliculitis), and past MRI showing lesions in the thalamus and pons (diencephalon predilection) a diagnosis of Behcet’s disease (BD) was considered based on ‘International Study Group Diagnostic Criteria for BD’. More so in the event of no better explanation for the neurological involvement, in a diagnosed case of BD, presenting delirium was considered to be the fallout of Neuro-Behcet’s Disease (NBD). The Patient responded to steroids and was discharged on a combination of tapering doses of steroids with Azathioprine. Emphasising the fact that the central nervous system affection in a case presenting with signs of systemic inflammation, autoimmune vasculitis as a cause of neurological involvement should be considered, as this is critical for deciding onto the course of treatment. NBD being secondary to systemic vasculitis as compared to atherosclerotic vascular affection seen in regular stroke, require steroids and immunomodulators rather than antiplatelets and anticoagulants.

Neuro-Behçet’s disease revealed by diencephalo-mesencephalic impairment

Introduction: Behçet’s disease is a systemic variable vessel vasculitis with unknown cause. Neurologic involvement known as neuro-Behcet’s disease (NBD) is often diagnosed in patients who present neurological symptoms and radiological lesions of the central nervous system. Case Report: A 42-year-old woman with a history of orogenital ulceration presented with behavioral disorders and heaviness of left hemibody. Brain magnetic resonance imaging (MRI) was performed and showed a pseudo-tumor diencephalo-mesencephalic area with T2 and Flair hypersignal, a slight hypersignal in diffusion sequence, and no contrast uptake. Conclusion: Pseudotumoral NBD is a rare but severe manifestation of Behçet’s disease. Cerebral MRI is the most efficient method to explore, detect, and monitor parenchymal lesions.