International Study Group For Behçet's Disease Research Articles

EPILEPSY IN BEHCET'S DISEASE

The etiology of epilepsy is associated with many exogenous and endogenous factors. One of the rare diseases, the pathophysiological mechanisms of which, affecting the nervous system, can guide the development of epilepsy, more often its symptomatic focal form, is Behcet's disease. Currently, the diagnosis is carried out based on the criteria developed by the International Study Group for Behcet's Disease (ISGBD). In Azerbaijan, 12.8% of cases were detected. The article presents a clinical case of epilepsy in a patient with Behcet's disease, which was identified during the period of the study conducted for the purpose of clinical and epidemiological study of epilepsy in one of the large settlements of Baku, in Mashtagi. Behcet's disease should be studied carefully. Perhaps, the results of further research will shed light on an urgent problem in epileptology, proving whether there is a connection between the development of epilepsy and ethnocultural characteristics of different populations.

Long-Term Outcome of Ustekinumab Therapy for Behçet's Disease.

Oral ulcers, the hallmark lesion of Behçet's disease (BD), can be disabling and resistant to conventional treatment, and there is a need for safe and effective treatment. We undertook this study to investigate the long-term safety and efficacy of ustekinumab therapy for BD-related oral ulcers that are resistant to colchicine. This multicenter, prospective, open-label study included 30 patients who fulfilled the criteria of the International Study Group for BD and who were diagnosed as having active oral ulcers resistant to colchicine. Patients were treated subcutaneously with ustekinumab 90 mg at inclusion, at week 4, and then once every 12 weeks. Each patient was assessed longitudinally for the presence and number of oral ulcers, and median numbers of oral ulcers (with interquartile range [IQR]) were calculated. The primary efficacy end point was the proportion of patients at week 12 who experienced complete response, defined as having no oral ulcers. The median number of oral ulcers per patient during ustekinumab therapy was significantly lower at week 12 compared to baseline (0 [IQR 0-1] versus 2 [IQR 2-3]; P < 0.0001). Complete response was achieved in 60.0% and 88.9% of patients at weeks 12 and 24, respectively. The median Behçet's Syndrome Activity Score (in which higher scores indicate more active disease) was significantly lower at weeks 12 and 24 (17.5 [IQR 10-42.5] and 10 [IQR 8-11], respectively) versus baseline (70 [IQR 50-70]; P < 0.0001). After a median follow-up of 12 months (IQR 6-16 months), 26 patients (86.7%) were still receiving ustekinumab treatment. Reasons for ustekinumab discontinuation included BD flare (n = 3) and side effects (n = 1). Seven patients (23.3%) experienced adverse events, including headaches (n = 4) and asthenia (n = 2), with no serious side effects. Ustekinumab seems to be effective in treating BD-related oral ulcers that are resistant to treatment with colchicine.

Cognitive disorders in Behçet's disease

To study the prevalence of cognitive disorders (CD) and clinical/pathogenetic correlations of CD in patients with Behçet's Disease (BD). One hundred and six BD patients were enrolled in the study. The majority of patients were natives of the North Caucasus (51.9%). Mean age was 33.3±0.98 years, mean illness duration 148.5±10.4 months. All the patients met the criteria of the International Study Group for BD (1990) classification. The disease activity was assessed by scoring system BDCAF. A diagnosis of a mental disorder (MD) was established by the psychiatrist in accordance with the ICD-10 using a semi-structured interview. The Montgomery-Asberg Depression Rating Scale (MADRS), the Hamilton Anxiety Rating Scale (HAM-A), a pathopsychological method 'Pictograms', clinical/psychological methods for assessment of cognitive functions (memory, attention concentration, logic thinking) were administered. Brain MRI was done in 44 (41.5%) BD patients. The study was conducted in the frames of the interdisciplinary program 'Stress factors and mental disorders in immune-mediated inflammatory rheumatic diseases'. CD of mild to moderate severity were diagnosed in 82 (77.4%) and anxiety-depressive disorders in 81 (76.4%) of BD patients. The patients with CD were older compared to patients without cognitive disorders (34.3±1.07 vs 29.0±2.14, p=0.006). Patients with CD were most often (84.1% vs 50.0%, p=0.001) diagnosed with anxiety-depressive disorder (anxiety, chronic/recurrent depression). MADRS scores were higher (16.1±0.74 vs 12.2±1.06, p=0.005) though did not exceed the moderate level. The impact of chronic psychosocial stressors was detected more often in CD patients. MRI results showed that the frequency of chronic multifocal, predominantly subcortical, changes in the white matter was higher in CD patients. CD are characteristic of most patients with BD. They are associated with the age, anxiety-depressive disorders, chronic stressors and minor brain multifocal subcortical parenchymal MRI lesions.

Recurrent Oral Ulcers: current and future therapeutic approaches

Recurrent Oral Ulcers: current and future therapeutic approaches

Ustekinumab for Behçet's disease

ObjectiveTo evaluate the efficacy and safety of ustekinumab in the treatment of oral ulcers (OU) in patients with Behçet's disease (BD). Patients and methodsProspective study including 14 patients [median age of 39 (34; 41) years, with 71% of men] fulfilling criteria of the International Study Group for BD and with active OU resistant to colchicine. Patients received ustekinumab 90 mg (n = 11) or 45 mg (n = 3) subcutaneously at inclusion, at week 4, and every 12 weeks. The primary efficacy endpoint was the proportion of patients with complete response (CR), defined as no oral ulcer, at week 12. ResultsAt week 12, 64% were in CR, 21% in partial response and 14% non-responders. The median number of OU decreased from 2 [2; 4] to 1 [0; 1.25] (p = 0.0005) at week 12. Mean change from baseline to week 12 of Behçet's syndrome activity score (BSAS) was 22.8 ± 0.3 (p = 0.01). The median daily corticosteroids dose decreased from 12.5 (10; 16.3) to 5 [5; 10] mg/day (p = 0.02). Three patients reported headaches, leading to discontinuation of ustekinumab in one case. After a median follow-up of 7 [3; 12] months, 10 (71%) patients were still receiving ustekinumab and four (28%) experienced a relapse. Decreased levels of circulating IL-17 and IL-12 [median [IQR]; 3.9 [1.6; 10.6] vs. 29.2 [25.2; 42.7] pg/ml, and 29.4 [23.1; 33.3] vs. 56.1 [51.1; 64.4] pg/ml, p = 0.008 for both] were observed under ustekinumab, respectively. ConclusionUstekinumab seems to be efficient and safe for patient with BD and refractory OU although relapses are frequent.

Evaluation of olfactory function in Behçet's disease.

Behçet's disease (BD) is a chronic, relapsing type of vasculitis of unknown etiology and is characterized by oral and urogenital ulcers and ocular inflammation with cutaneous, musculoskeletal, vascular, and nervous system manifestations. Few cases involving the nasal mucosa have been reported in the literature, and the true prevalence of BD remains unknown. Neurological involvement associated with BD might play a more important role in causing olfactory dysfunction than mucosal involvement, but sufficient clinical data are not available on the effect of BD on olfaction in adults. We therefore evaluated the olfactory function of patients diagnosed with BD. Patients were chosen from among a consecutive patient group population who visited the internal medicine rheumatology polyclinic and otolaryngology departments of Ondokuz Mayıs University Hospital. A total of 50 patients (both males and females) aged 18 to 60 years with a diagnosis of BD and 46 healthy controls (matched to the study group in terms of age and gender) were included. BD was diagnosed based on the criteria defined by the International Study Group for BD. A complete clinical history was taken for and a physical examination was performed in all participants. Patients with other rheumatic diseases; obstructive nasal pathologies leading to conductive-type olfactory dysfunction (e.g., septum deviation or nasal polyp); advanced systemic disease (e.g., hypertension or malignancy); a history of antithyroid, antihistamine, antidepressant, or steroid medication use within the past month; or who were current smokers, had an active upper respiratory infection, or had a history of otolaryngologic operations were excluded. The results of the "Sniffin' Sticks" (SS) olfactory test were compared between the two groups. The mean age of the 50 BD patients was 35.3±10 years; that of the 46 health controls was 36.9±11 years. There was no significant group difference in age or gender distribution (p>0.05). Odor identification and overall scores were significantly lower in the BD group than in the control group. There were no significant differences in odor discrimination scores between the BD and control groups (p>0.05). To our knowledge, this is the first study to evaluate olfactory function in patients diagnosed with BD using the SS test. Odor identification was more impaired in BD patients than in healthy controls, but there was no group difference in odor discrimination. BD patients should also be assessed for the involvement of olfactory function and may require treatment due to a malfunction of the olfactory system that affects the quality of life.

Increased Serum Antibody Titer against HPV-16 Antigen in Patients with Behçet's Disease.

Quadrivalent human papillomavirus (HPV) vaccine has been reported to be significantly associated with Behçet's disease (BD). However, no reports have described HPV infection as a possible cause for the development of BD. The objective of this study was to evaluate whether anti-HPV immunoglobulin G (IgG) antibody titer is increased in BD. Serum samples from 93 Korean BD patients, who fulfilled the diagnostic criteria of the International Study Group for BD, were used in an enzyme-linked immunosorbent assay (ELISA). The clinical activity of BD was evaluated at the time of blood sampling. HPV-16 L1 virus-like particle (VLP) antigen was used in this study for the ELISA. Patients with BD had significantly higher antibody titers against HPV-16 (optical density [OD], 0.210–3.675; mean 0.992) than that of healthy controls (OD, 0.248–0.762; mean 0.517; P < 0.001). Using a receiver operating characteristic (ROC) analysis, a cut-off value of 0.578 OD for the anti-HPV antibody titer was determined that differentiated BD patients from healthy controls. When we compared the clinical features of BD between the 2 groups, articular involvement of BD was more likely in patients with an anti-HPV-16 antibody titer < 0.578 OD (P = 0.035). In addition, patients with an anti-HPV-16 antibody titer < 0.578 were significantly younger than those with a titer ≥ 0.578 OD. HPV itself may be a possible extrinsic triggering infectious agent causing the development of BD.

Arterial Aphta : Which value in Behcet disease ?

The behcet disease is a vasculitis with polyorganic attacks whose diagnosis is established bythe international study group for behcet disease (ISGBD).The arterial ulcer or aphta has no diagnostic value in the criteria of ISGBD.its morphological similarity and most likely pathophysiological also, with bipolar ulcersdeserves attention.This article is an observation relating the history of a patient who does not respond to criteriaof ISGBD despite the presence of an arterial aphta.The tradive occurrence of minor criterion was a cause of diagnostic and therapeutic delay.

Periodontal Health Status among Patients with Behçet's Disease.

We aimed to evaluate the relationship of individual periodontal parameters with the severity of Behçet's disease (BD) and attempt to find the correlation between chronic periodontitis and BD. In this study, 74 registered subjects attending Behçet's clinic with BD symptoms were recruited. The diagnosis was based on the criteria presented by the international study group for BD (ISG) and the total clinical severity score was determined for each patient. All individuals underwent clinical examination to assess oral and periodontal status and presence of oral ulcers. Periodontal clinical parameters of bleeding on probing (BOP), probing depth (PD), and clinical attachment level (CAL) and also hygiene index (HI) and decayed, missing, and filled (DMF) teeth were noted and analyzed to assess the correlation with severity of BD. There was no significant correlation between DMF and severity of BD. The strong association was found between periodontal parameters (BOP, PD, and CAL) and the severity of BD (P < 0.001). It seems that relation of BD to oral health is higher up in severe forms of BD and periodontal diseases. Clinical association between the diseases might be due to a common underlying etiopathogenesis of periodontitis and BD.

FRI0474 The Three-Year Outcome of Infliximab (IFX), an Anti-TNF-α Antibody, in Patients with Refractory Intestinal-BehÇEt's Disease (BD)

BackgroundIntestinal involvement is one of the intractable categories of Behçet's disease (BD) and closely related with prognosis of patients with BD. Although high dose corticosteroids (CS) and various immunosuppressant are...

SAT0266 Behcet's Disease in Morocco: Report of 1484 Cases

BackgroundBehçet's disease is a complex multi-system disorder of unknown aetiology characteristically presenting with recurrent oral ulcers. It is presumed to be a vasculitis and includes involvement of the ocular, cardiovascular,...

Intestinal Behcetʼs or Crohnʼs Disease

Purpose: Intestinal Behçet's disease (BD) and Crohn's disease (CD) are chronic inflammatory bowel diseases that are difficult to distinguish from each other. We present a case of intestinal BD which was very difficult to differentiate from CD. Case: 29-year-old male with history of occasional oral ulcers presented with diarrhea, abdominal pain and fevers after eating raw oysters. His partner developed the same symptoms which resolved after taking metronidazole (MTZ) and ciprofloxacin (Cip). Patients symptoms improved after taking MTZ and Cip, but returned 1 week later with diarrhea, painful scrotal ulcers (Figure 1), right wrist pain, sore throat and oral thrush. He also had ulcers on the uvula, cervical and inguinal lymphadenopathy, and multiple small pustular lesions over the body. His albumin was 1.8 mg/dl, hemoglobin 9.7 gm/dl and stool positive for Clostridium difficle toxin. A MR enterography showed diffuse thickening of descending colon and loss of haustra. Flexible Sigmoidoscopy with biopsy showed severe active colitis with cryptitis and ulcerations in the colon and mild patchy proctitis. Patient was started on IV MTZ, oral vancomycin and IV methylprednisolone 60 mg BID. There was immediate improvement in his symptoms and he was discharged from the hospital 4 days later. Discussion: The prevalence of intestinal BD is 1-2% in BD and can have similar presentation as Crohn's disease. The ulceration can be found throughout the colon, but mostly frequently found in the ileocecal region. Our patient met the criteria by international study group for BD with having recurrent oral ulcerations, genital ulcer and involvement of the colon. HLA-B51 allele positive patients have higher prevalence in Japan and eastern Asia and more severe disease. The cummulateive use of steroids and higher number of ulcers are associated in patients with Anti Alpha Enolase Antibody. Endoscopically a round, less than 6 ulcers and absence of cobblestoning is helpful to differentiate intestinal BD from CD. Corticosteriods are the drug of choice for active disease. Azathioprine, colchicine, infliximab, dapsone and tacrolimus have been used for maintenance of intestinal BD. This case highlights the difficulty in differentiating intestinal BD from CD.Figure: [1254] Scortal ulcer.

Central Retinal Artery Occlusion and Recurrent Papillitis in a Patient With Incomplete Behçet Disease

We thank Dr Liu for the interest in our article and will respond to the questions raised (1). First, Dr Liu pointed out that a single photograph of the fluorescein angiogram does not offer enough evidence for a diagnosis of central retinal artery occlusion (CRAO). We have already extensively discussed this with the reviewers and editor of the journal before our article was accepted. When we did this, we carefully reviewed the fluorescein angiogram and found in the early phase of the study that there was good choroidal filling (Fig. 1). In the Photo Essay, Figure 2A shows a later stage of the angiogram when filling of the choroid was complete, yet there was no perfusion of the retinal vessels. Furthermore, the appearance of the vascular occlusion in Figure 2A is actually more suggestive of an occlusive vasculitis than a CRAO caused by emboli, adding further support for the diagnosis of Behçet disease (BD). Second, regarding the issue of diagnostic criteria for BD, our patient admitted to recurrent oral ulcers more than 5 times per year. Finally, we agree that CRAO and recurrent papillitis are not listed as minor features in the diagnostic criteria of BD (2), but “vasculitis” and “central nervous system symptoms” are included. If we accept that the vascular occlusion observed in each eye of our patient was caused by vasculitis and that the optic nerve is part of the central nervous system, then we have 2 minor features of BD. While recurrent oral ulcers are a major feature, it is noteworthy that diagnostic criteria for BD vary and have been revised many times. The International Study Group for BD proposed that the diagnosis be considered when recurrent oral ulcers plus 2 other features are present, in the absence of other clinical explanations (3). In our patient, we performed a wide range of ancillary tests to exclude other disorders, which can cause an obliterative vasculitis.FIG. 1: Early phase of fluorescein angiogram demonstrates choroidal filling.Most importantly, we wish to remind clinicians that CRAO and recurrent papillitis can be caused by a vasculitis, such as BD. Prompt treatment can be helpful to prevent further loss of visual function.

Association of amyotrophic lateral sclerosis and Behcet’s disease: is there a relationship? A multi-national case series

Neurological involvement may be seen in 5-30% of the patients with Behcet's disease (BD). Occasionally, parenchymal neurological involvement in BD can present as a spinal cord syndrome. However, motor neuron disease-like presentation is extremely uncommon. Here we are reporting five patients (all male; median age, 38) fulfilling both International Study Group criteria for BD and El Escorial criteria for amyotrophic lateral sclerosis (ALS). These patients were identified by a questionnaire sent to the members of the Neuro-Behcet Study Group of the International Study Group for BD. Three out of five patients had only motor presentations. In two patients, sensory and urinary manifestations were present as well. Spinal cord MRIs were normal in all, and brain MRIs were normal in four patients; one patient had nonspecific white matter changes. Two patients passed away 1-3 years after diagnosis of ALS, and two patients were lost to follow-up 3 and 11 years after admission; one patient is still alive 3 years after onset. The patients that are presented here might represent a rare form of neurological involvement in BD as well as sole coincidence. Larger prospective series are needed to further answer this issue.

Expression of Pro-inflammatory Protein S100A12 (EN-RAGE) in Behçet's Disease and Its Association with Disease Activity: A Pilot Study

BackgroundS100A12 is a member of the S100 family of calcium-binding proteins and is secreted either in inflamed tissues or in the bloodstream by activated neutrophils. Expression of S100A12 has been reported in various diseases, especially non-infectious inflammatory diseases, such as Kawasaki disease, giant cell arteritis and inflammatory bowel disease.ObjectiveThis study was conducted to determine both the tissue expression and the serum levels of S100A12 in Behçet's disease (BD) patients and the correlation of the S100A12 serum level with disease activity of BD.MethodsWe included in this study ten BD patients who fulfilled the criteria for diagnosis, according to the International Study Group for BD. The activity of BD was calculated using the BD Current Activity Form. The serum concentrations of both S100A12 and interleukin-8 were measured by the enzyme-linked immunosorbent assay, before and after treatment. Immunohistochemical studies were also performed to detect S100A12 expression in the skin.ResultsThe serum S100A12 level was significantly increased in the active BD period (p<0.001), in the inactive BD period (p=0.041) and in patients with active Kawasaki disease (p=0.028), compared with the serum level in the healthy controls. The serum S100A12 level decreased significantly from baseline, compared to post-treatment (p=0.017). The activity score of BD was significantly correlated with the serum level of S100A12 (Spearman's coefficient=0.464, p=0.039). Immunohistochemical studies showed that S100A12 was strongly expressed in the erythema nodosum-like skin lesions of patients.ConclusionS100A12 contributes to the pathogenesis of BD related to neutrophil hyperactivity and reflects the disease activity in BD patients.

Late-onset Behçet’s disease: demographic, clinical, and ocular features

To evaluate the demographic, clinical, and ocular features of patients with Behçet's disease (BD) who had first symptom onset after 40 years of age and fulfilled the diagnostic criteria afterwards. This retrospective study included 42 patients with initial BD symptom onset after 40 years of age who fulfilled the diagnostic criteria afterwards, according to the International Study Group for BD. Patients were divided into two groups based on the presence of ocular involvement: group 1 had uveitis and group 2 did not have uveitis. The ocular characteristics of patients in group 1 were further examined according to age groups. Group 1A included patients between 40-50 years of age, and group 1B included patients older than 50 years of age. Clinical and demographic features of uveitic and non-uveitic patients were compared. Ocular manifestations, treatment protocols, and ocular complications in uveitic patients were noted. The ocular characteristics of group 1A and group 1B were further evaluated. Twenty-six patients (61.9%) had uveitis (group 1) and 16 (39.1%) had no uveitis (group 2). There was no significant difference between groups according to sex, age at initial admission, age at the time of initial symptom of BD, and period between initial symptom and the diagnosis of BD. The most frequent initial manifestation was oral ulcer in both groups. No statistical difference between the groups was detected with regard to the mean age of onset of each symptom. Anterior uveitis (73.1%) was the most frequent type of uveitis followed by panuveitis (19.2%) and sclerouveitis (7.7%). Uveitis was bilateral in 80.8% of patients. The incidence of anterior uveitis was higher in group 1B than in group 1A (P = 0.023). Cataract was the most common ocular complication, followed by macular edema sequelae, glaucoma, optic disc paleness, and branch retinal vein occlusion. Late-onset BD usually affects both genders equally, and the prognosis of ocular involvement is usually good. The incidence of panuveitis decreases as age increases, while the incidence of anterior uveitis increases. Ocular involvement is usually bilateral and there is no correlation between gender and uveitis type.

Hyperhomocysteinaemia in Behçet's Disease

Objectives. The aim of this study was to investigate if hyperhomocysteinaemia is a contributive risk factor for the pathogenesis and the activity of Behçet's disease (BD). Design and Methods. Fifty four patients fullfiling the criteria of the International Study Group for BD were enrolled. Fifty healthy volunteers matched for age and sex with the BD group were included as a negative control group. Patients, with any condition that might affect plasma homocysteine concentration, were excluded. Results. Mean serum homocysteine concentration was significantly higher in patients with BD than in the healthy controls (P < .001), in patients with active disease (P = .04), and in masculine gender (P = .05). There was no significant difference between homocysteine level and clinical involvement. Conclusions. We demonstrated that plasma total homocysteine level (tHcy) is increased in BD and correlated with disease activity. No association was found between homocysteine levels and clinical involvement.

Ausência de anticorpos antilipoproteína lipase na doença de Behçet

OBJETIVO: A recente descrição de anticorpos antilipoproteína lipase (anti-LPL) associados à dislipoproteinemia levounos a analisar sua presença e possível associação com achados clínicos e laboratoriais em pacientes com doença de Behçet. PACIENTES E MÉTODOS: Trinta e oito pacientes consecutivos com doença de Behçet [critérios do Grupo de Estudos Internacional em Doença de Behçet (International Study Group on Behçet's Disease - ISGBD)] foram testados para a presença de anticorpos anti-LPL através da técnica de ELISA. Foi realizada avaliação clínica e laboratorial, incluindo perfil lipídico de jejum, pesquisa de autoanticorpos e marcadores inflamatórios (PCR, VHS) na inclusão dos pacientes. Os critérios de exclusão foram quaisquer condição que afetassem o perfil lipídico. RESULTADOS: A média de idade foi de 42 ± 9 anos, sendo 68% do sexo feminino e 68% da cor branca. O tempo médio de duração da doença foi de 9,8 ± 7,5 anos. Vinte e nove por cento dos pacientes apresentaram história de trombose. Os níveis de PCR estavam elevados em 31% dos pacientes e VHS aumentada foi detectada em 31%, com níveis médios de 5,95 ± 10,3 mcg/mL e 14,5 ± 13,2 mm/1ªhora, respectivamente. Cerca de 47% dos pacientes estavam tomando prednisona, com dose média de 7,6 ± 10,8 mg/dia. Quanto aos níveis de risco cardiovascular da NCEP/ATPIII, colesterol elevado foi verificado em 26%, triglicerídeos em 18%, HDL baixo em 15% e elevado LDL em 25% dos pacientes com Behçet. Os níveis médios de CT foram 198 ± 48 mg/dL, de triglicerídeos 121 ± 61 mg/dL, HDL 52.4 ± 14.7 mg/dL e LDL 119 ± 35 mg/dL. Anticorpos anti-LPL to subtipo IgG foram detectados em 0/30 pacientes com Behçet. CONCLUSÃO: Esses dados aqui apresentados corroboram uma ausência de ligação entre inflamação, resposta imunológica e dislipoproteinemia nos pacientes com doença de Behçet e sugerem que outros mecanismos estão associados à dislipidemia vista nestes pacientes.

Is there any relationship between serum and urine neopterin and serum interferon‐γ levels in the activity of Behcet's disease?

Behcet's disease (BD) is a chronic, inflammatory, multisystem vasculitic disorder. There is no reliable laboratory marker that indicates disease activity. Neopterin is an immunological marker of cellular immune activation, which is secreted by monocytes/macrophages as a result of interferon-gamma (IFN-gamma) secretion by activated T lymphocytes. We aimed to investigate serum and urine neopterin levels in BD patients. Forty-five patients who were diagnosed according to the criteria of the International Study Group for BD and 45 age- and sex-matched healthy controls were enrolled in the study. Disease activity was considered by clinical findings. Serum and urine neopterin levels and serum IFN-gamma levels were measured. The mean values of serum and urine neopterin levels were 12.68 +/- 4.87 nmol/L and 167.53 +/- 148.73 micromol/mol creatinine, respectively, in BD patients (P = 0.000 and P = 0.008, respectively), which were statistically significantly different from the control group. However, there was no significant statistical difference between serum and urine neopterin levels of the clinically active and inactive patients. It was also found that the mean value of serum IFN-gamma levels was higher in healthy controls than in BD patients (P = 0.000). We conclude that serum and urinary neopterin measurement can not be used as a reliable laboratory marker as the BD patients' serum and urinary neopterin levels do not increase in the active stage even though these levels increase when compared to healthy controls.

The Presentation of Headache in Neuro‐Behçet's Disease: A Case‐Series

Behçet's disease (BD) is a chronic, relapsing, multisystemic, inflammatory disorder with unknown etiology. Neurological involvement is observed in about 5% of the patients with BD and headache is a frequently reported symptom with or without neurological involvement. In this case-series, we aim to demonstrate the secondary headaches associated with neuro-Behçet's disease in consecutive BD patients who had been referred for neurologic evaluation. The case-series included 17 patients (11 males [65%]), mean age 41 +/- 11.4, who met the criteria for BD established by the International Study Group for BD and developed headache during the disease course. Seventeen BD patients were identified, the neurologic presentations included in parenchymal involvement 12 (70%), extraparenchymal involvement/venous thrombosis in 5 (30%), seizure disorder in 2 (12%), and psychiatric problems/depression or anxiety in 5 (30%) patients, respectively. The characteristics of the headache according to the patients' reports included subacute, moderate to severe headache with unilateral localization and throbbing quality accompanied by nausea, vomiting, and aggravation upon awakening. Because neurological involvement is one of the most devastating aspects of BD, the need to treat neurological involvement as early as possible, the possible role of headache onset as an early indicator for neurological involvement in this relatively uncommon disease should be kept in mind.