Involvement In Behcet's Disease Research Articles

Efficacy of Infliximab in Patients with Refractory Uveitis Associated with Behcet Disease

Background: Ocular involvement in Behcet disease is responsible for severe morbidity by causing nongranulomatous panuveitis and retinal vasculitis. In some cases, recurrent attacks cannot be controlled by conventional immunosuppressives and corticosteroid or interferon. The aim of this study is to investigate the efficacy of infliximab treatment in patients with refractory uveitis associated with Behcet disease.Methods: Nine patients with Behcet disease related uveitis were included in the study. Uveitis had been refractory to treatment with either a combination of cyclosporine-A, azathioprine, and corticosteroid or interferon α-2a monotherapy in all patients. Patients received infliximab infusions (5 mg/kg) at weeks 0, 2, 6, and every 8 weeks thereafter. In patients receiving interferon α-2a at the time of initiation of infliximab therapy, interferon was ceased before starting infliximab. We evaluated visual acuity, ocular inflammatory attacks, and adverse effects.Results: The Patients consist of seven men and two women; their ages ranging from 28 to 49 years. The mean follow-up after initiating infliximab therapy was 14.4 months. Four of the patients had no ocular inflammatory attacks during follow-up. Two of the remaining five patients developed a mild anterior uveitis only once after the start of infliximab infusions. Three of the patients had a mild panuveitis attack 2-4 months after the initiation of infliximab therapy. Best corrected visual acuity (BCVA) improved at least 2 lines in 6 (33.3%) eyes, from no light perception to hand motion in one eye, and from light perception to hand motion in another eye. BCVA remained stable in 9 (50%) eyes. In one patient, infliximab treatment was terminated owing to HPV+genital warts at 8th month of therapy. No other adverse effects were noticed apart from temporary infusion reaction and urticaria-like rash.Conclusions: With a follow-up period ranging from seven to thirty three (7-33) months, infliximab was effective and well tolerated in refractory Behcet uveitis.

P.14.3 Dermatomyositis in a patient with Behcet’s disease

A case of dermatomyositis associated with Behcet's disease (BD) is described. A 51-year-old male with a 12-year history of BD, was currently on remission with colchicine therapy (0.5mg/day), presented with acute onset difficulty in walking and skin lesions. His neurological examination revealed proximal muscle weakness in both the upper and lower extremities. No myalgia was noted. He had cutaneous lesions typical for dermatomyositis on his face, neck, upper trunk and hands. Laboratory tests demonstrated normal findings except elevated ESR and serum CK level (639U/L). Muscle MRI of lower extremities revealed multiple patchy hyperintense lesions in pelvic and thigh muscles on STIR images. There was no muscle atrophy or fat replacement and it was commented as muscle inflammation. The clinical and laboratory findings were compatible with the diagnosis of dermatomyositis. He responded well to IV methylprednisolone within days. He continued on prednisolone therapy orally with gradual tapering. This case of Behcet's disease associated with dermatomyositis (DM) is a very rare one. Muscular involvement in BD is rarely described but DM in association with BD has been never reported. The underlying cause of BD and the precise pathogenic mechanisms responsible for DM are unknown. The association of BD and DM may provide insights into genetic predisposition and pathogenesis of these complex and multisystemic autoimmune disorders.

Assessment of QRS duration and presence of fragmented QRS in patients with Behçet’s disease

QRS prolongation and the presence of QRS fragmentation in 12-lead ECG are associated with increased mortality and sudden cardiac death in the long term. In this study we aimed to assess QRS duration and fragmentation in patients with Behçet's disease (BD). A total of 50 patients (mean age 42.7±12.0 years) previously diagnosed with BD were recruited. In addition, a control group consisting of 50 healthy people (mean age 39.4±12.5 years) was formed. The longest QRS duration was measured in surface 12-lead ECG and QRS complexes were evaluated in terms of fragmentation. Serum C-reactive protein levels were also obtained. QRS duration and corrected QT duration were significantly longer in patients with BD compared with controls (102.75±11.91 vs. 96.99±10.91 ms, P=0.007; 438.55±30.80 vs. 420.23±28.06 ms, P=0.003, respectively). Fragmented QRS (fQRS) pattern was more common in patients with BD than controls [n=27 (54%) vs. n=16 (32%), P=0.026]. Disease duration was longer in patients with BD with fQRS compared with those without (12.67±8.68 vs. 7.09±7.06 years, P=0.010). Furthermore, C-reactive protein level was higher in patients with BD with fQRS compared with those without (6.53±4.11 vs. 4.97±6.32 mg/dl, P=0.043). Correlation analysis revealed no association between disease duration and QRS duration (r=0.219, P=0.126). QRS duration is greater and fQRS complexes are more frequent in patients with BD. These findings may indicate subclinical cardiac involvement in BD. Given the prognostic significance of ECG parameters, it is reasonable to evaluate patients with BD with prolonged and fQRS complexes more in detail such as late potentials in signal averaged ECG in terms of cardiac involvement.

Major vascular involvement in Behçet’s disease: a retrospective study of 796 patients

Behçet's disease (BD) is a multi-systemic inflammatory disorder which can affect all types and sizes of blood vessels. This study aims to evaluate the prevalence and characteristics of vascular involvement in BD. Among 796 patients diagnosed with BD, 102 patients (81 male, 21 female) with vascular involvement were included, whose detailed clinical characteristics were recorded. The diagnosis of vascular lesions was made on clinical signs, by Doppler ultrasonography, and/or angiography using computed tomographic or magnetic resonance techniques. Vascular involvement occurred in 12.8 % of BD patients. Male to female ratio was 3.86:1. Mean age at onset of vascular involvement was 29.5 ± 11.3 years. Vascular lesion was the initial sign of BD in 28 patients, accounting for 27.5 %. Of 102 BD patients with vascular involvement, 72 had venous lesions (70.6 %) and 56 had arterial lesions (54.9 %), among which 26 (25.5 %) patients had both venous and arterial involvements. Female BD patients were more often involved with arterial lesions, whereas male BD patients developed venous lesions more often than females, P = 0.000. The most common type of vascular involvement was deep venous thrombosis in lower extremities (n = 49), other affected venous sites including inferior vena cava, superior vena cava, and cerebral venous. The prominent type of arterial lesions was dilatation (n = 25, including 24 cases of aneurysms); other types included eight cases of occlusion and 23 cases of stenosis. The main locations of arterial lesions were the aorta (n = 19), lower extremity arteries (n = 15), pulmonary arteries (n = 13), coronary arteries (n = 5), and subclavian arteries (n = 5). Compared with those without vascular lesions, ocular involvement, genital ulcers, and arthritis were significantly less frequent among patients with vasculo-BD (23.5 vs 35.2 %, P = 0.024; 54.9 vs 76.5 %, P = 0.000; 19.6 vs 30.5 %, P = 0.026), whereas a higher frequency of cardiac involvement was found in vasculo-BD patients (20.6 vs 3.6 %, P = 0.000). Vascular involvement is a complication in BD patients. This study illustrated that venous lesions are more frequently involved than arterial lesions. Vascular lesions correlated with a high frequency of cardiac involvement and a low incidence of ocular lesions, genital ulcers, and arthritis.

Interleukin‐2 gene polymorphism inTurkish patients withBehçet's disease and its association with ocular involvement

Behçet's disease (BD) is a chronic immune-mediated systemic disease, characterized by oral and genital lesions and ocular inflammation. Several cytokine genes may play crucial roles in host susceptibility to BD, because the cytokine production capacity varies among individuals and depends on the cytokine gene polymorphisms. The association of the interleukin (IL)-2 gene polymorphisms with the susceptibility to BD was investigated in this study. DNA samples were obtained from a Turkish population of 97 patients with BD and 76 healthy control subjects. Polymorphisms of IL-2 gene at position -330 and +166 were determined using the polymerase chain reaction with sequence-specific primers. In the patients with BD, there was a significantly increased frequency of IL-2 -330 GT genotype. Interestingly, we demonstrated that the frequencies of IL-2 -330 GT and IL-2 + 166 GG genotypes were increased in BD patients with ocular involvement, whilst IL-2 -330 TT genotype was significantly decreased. Also, analysis of allele frequency demonstrated that the presence of G allele at position +166 of IL-2 seems to be a risk factor for ocular involvement. These results reveal that IL-2 -330 GT genotype may be a susceptibility factor for BD, whereas IL-2 -330 TT genotype seems to display a protective association with BD. Additionally, IL-2 gene polymorphisms might be associated with ocular involvement in BD.

Muscular involvement of Behçet's disease: ultrasonography, computed tomography, and magnetic resonance imaging findings

Behçet's disease (BD) is a form of systemic vasculitis with the classic triad of recurrent oral and genital ulcers along with uveitis. In BD, muscular involvement is very rare. We report a case of muscular involvement in BD with characteristic findings using ultrasonography, computed tomography, and magnetic resonance imaging.

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.

Left atrial volume and function in patients with Behcet's disease assessed by real-time three-dimensional echocardiography

Behçet's disease (BD), a multisystemic inflammatory disorder, has been associated with a number of cardiovascular dysfunctions, including endomyocardial fibrosis of the right heart, atrial fibrillation, ventricular arrhythmias and sudden cardiac death. The incidence and nature of cardiac involvement in BD are not yet clearly documented. Our aim was to evaluate left atrial (LA) volume and functions using real-time three-dimensional echocardiography (RT3DE) in Behcet's patients without any cardiac symptom. The study included 40 BD (16 females, 24 males and mean age of 33±7 years) and 30 healthy (11 females, 19 males and mean age of 35±6 years) subjects. All the patients' demographic parameters such as age, gender, and duration of BD were recorded. All the individuals underwent comprehensive 2D echocardiography examination, and RT3DE was performed to assess LA volumes and mechanical functions. LA maximum volume (Vmax) and before atrial contraction volume (Vpre A), LA active stroke volume and total stroke volumes (TSV), total emptying and active emptying fractions and expansion index were significantly higher in Behcet's disease patients when compared with the controls (P<0.0001 for all). LA passive emptying fraction was significantly lower in the patients with BD than in the controls (41±7 vs. 44±5, P=0.039). There were positive correlations between TSV and high-sensitive C-reactive protein level (r=0.413, P=0.008), TSV, and disease duration (r=0.417, P<0.007). Our study has shown that LA mechanical functions and volumes are impaired in BD. These results may be an early form of subclinical cardiac involvement in patients with BD who have no clinical evidence for cardiovascular disease.

Simultaneous Involvement of Nervous and Gastrointestinal Systems in Behçet's Disease

Simultaneous Involvement of Nervous and Gastrointestinal Systems in Behçet's Disease

Pulmonary Artery Involvement and Associated Lung Disease in Behçet Disease

Pulmonary artery aneurysms (PAAs) are well known causes of mortality and morbidity in Behçet disease (BD). However, pulmonary artery involvement in BD is not limited to PAA; the other main type of pulmonary artery involvement is pulmonary artery thrombus (PAT), with or without associated PAA. In addition, other types of lung disease like nodules and cavities in the lung parenchyma are frequently associated with pulmonary artery involvement, and can be misinterpreted as being due to infection. We surveyed the clinical, radiologic, and laboratory characteristics and outcome of 47 BD patients with pulmonary artery involvement and the associated findings, all seen and followed at a single dedicated tertiary care center.We identified 47 (41 male, 6 female) patients in whom pulmonary artery involvement was diagnosed, who were registered in the multidisciplinary clinic at Cerrahpasa Medical Faculty between January 2000 and December 2007. Mean age at diagnosis was 29 ± 8 years, and mean disease duration to the onset of pulmonary artery involvement was 3.6 ± 4.8 years. Hemoptysis was the most common presenting symptom (79%) followed by cough, fever, dyspnea, and pleuritic chest pain. Thirty-four of 47 patients (72%) presented with PAA, including 8 with associated PAT. The remaining 13 patients (28%) had isolated PAT. Patients with isolated PAT in general have clinical features similar to patients with PAA. However, hemoptysis was less frequent and voluminous in patients with isolated PAT. Most (91%) of the patients had active disease outside the lungs when they presented with pulmonary artery involvement.Forty (85%) patients had nodules and 6 (13%) had cavities when first seen. Peripheral venous thrombosis was present in 36 of 47 (77%) patients, and intracardiac thrombi in 12 of the 36 (33%) patients. Nodules, cavities, and intracardiac thrombi were mainly present in the acute stages of pulmonary artery involvement.Pulmonary artery involvement is usually multiple, and involves mostly descending branches of the pulmonary artery. Pulmonary artery involvement may disappear, but arterial stenosis or occlusions usually develop at the same location. After a mean follow-up of 7 years, 12 of 47 (26%) patients were dead; patients with larger aneurysms were more likely to die. Sixteen of 47 (34%) patients were symptom free, and the remaining 40% had mild dyspnea (13/47) and/or small bouts of hemoptysis (8/47).Pulmonary artery pressure may be elevated, and may indicate a poor prognosis. Mediastinal lymphadenopathy and mild pleural and pericardial effusions may also be observed. Corticosteroids and immunosuppressive agents are the mainstays of treatment; however, refractory cases may require embolization, lobectomy, cavitectomy, and decortication.

Spectrum of Cardiac Lesions in Behçet Disease

Cardiac abnormalities in patients with Behçet disease (BD) include pericarditis, myocarditis, endocarditis with valvular regurgitation, intracardiac thrombosis, endomyocardial fibrosis, coronary arteritis with or without myocardial infarction, and aneurysms of the coronary arteries or sinus of Valsalva. Data regarding the clinical spectrum, prevalence, and outcome of cardiac lesions in BD are lacking. In this study, we report the main characteristics, treatment, and long-term outcomes of 52 patients with cardiac lesions from a cohort of 807 (6%) BD patients. Forty-five (86.5%) patients were male, with a mean (±SD) age at BD diagnosis of 29.3 ± 10.3 years.Cardiac involvement was the first feature of BD in 17 (32.7%) patients. Cardiac lesions included pericarditis (n = 20; 38.5%), endocarditis (mostly aortic insufficiency) (n = 14; 26.9%), intracardiac thrombosis (n = 10; 19.2%), myocardial infarction (n = 9; 17.3%), endomyocardial fibrosis (n = 4; 7.7%) and myocardial aneurysm (n = 1; 1.9%). Patients with cardiac involvement were more frequently male (86.5% vs. 64.9%; p < 0.01) and had more arterial (42.3% vs. 11.1%; p < 0.01) and venous lesions (59.6% vs. 35.8%; p < 0.01) compared to those without cardiac manifestations. Factors associated with complete remission of cardiac involvement were treatment regimens with oral anticoagulants, immunosuppressants, and colchicine. The 5-year survival rate was 83.6% and 95.8% (p = 0.03) in BD patients with and without cardiac involvement, respectively. After a median (Q1-Q3) follow-up of 3.0 (1.75-4.2) years, 8 patients had died, in 3 cases directly related to cardiac involvement.In conclusion, cardiac lesions affected 6% of our large cohort of BD patients. The prognosis of cardiac involvement in BD is poor and improves with oral anticoagulation, immunosuppressive therapy, and colchicine.

Correlation of DEFA1 Gene Copy Number Variation with Intestinal Involvement in Behcet's Disease

Copy number variation has been associated with various autoimmune diseases. We investigated the copy number (CN) of the DEFA1 gene encoding α-defensin-1 in samples from Korean individuals with Behcet's disease (BD) compared to healthy controls (HC). We recruited 55 BD patients and 35 HC. A duplex Taqman® real-time PCR assay was used to assess CN. Most samples (31.1%) had a CN of 5 with a mean CN of 5.4 ± 0.2. There was no significant difference in the CN of the DEFA1 gene between BD patients and HC. A high DEFA1 gene CN was significantly associated with intestinal involvement in BD patients. Variable DEFA1 gene CNs were observed in both BD patients and HC and a high DEFA1 gene CN may be associated with susceptibility to intestinal involvement in BD.

Behcet's Disease and Endocrine System

Behcet's disease (BD) is a chronic disease which is characterized by recurrent oral apthous ulcerations, recurrent genital ulcerations, skin eruptions, ocular involvements and other various systemic manifestations as well as systemic vasculitis. Endocrine involvement in BD regarding various systems can be seen. Hypophysis is one of the best and dense vascularized organs of the body, thus it is likely that it can be affected by BD. Not only anterior hypophysis functions, but posterior hypophysis functions as well can be affected. As BD is a disease of autoimmune process, it may be possible that adrenal insufficiency or alterations in the cortisol levels could be expected. Another concern is whether or not there is insulin resistance in patients with BD. The avaliable data suggests that there is an increased susceptibility to insulin resistance in patients with BD.

Pathologic Features of Behçet's Disease in the Tubuler Gut

Behçet's disease (BD) is a vasculitic disorder of relapsing acute inflammation characterized by recurrent oral ulcers, genital ulcers, uveitis, and skin lesions. The disease also affects other organs, including joints, the nervous system, blood vessels, and gastrointestinal (GI) system may also be involved and the lower GI tract is the mostly involved part, leading to severe morbidity. The frequency of GI involvement in BD varies among different ethnic groups. Although 50–60% of Japanese patients have GI disease, these manifestations are rare in patients from Mediterranean countries. The gastrointestinal manifestations of BD usually appear 4.5–6 years after the onset of the oral ulcers. The intestinal lesions are usually resistant to medical treatment and recur after surgery. The elementary lesion is apthous ulcer. Deep, round or oval ulcers with a punched-out appearance tend to perforate easily, so that many patients require urgent operation.

Surgical Treatment of Peripheral Aneurysms in Patients with Behcet’s Disease

Introduction Our aim was to report our experience with 23 patients presenting with 32 peripheral aneurysms secondary to Behcet’s disease (BD) and their outcome after vascular surgery. Methods The study was retrospective in nature. Except for those presenting with aneurysm rupture, patients underwent surgery after treatment of acute inflammatory lesions. All aneurysms appeared to be pseudo-aneurysms. Graft interposition with polytetrafluoroethylene or saphenous vein was most commonly employed. Postoperatively, all patients were put on immunosuppressive and antiplatelet therapy. Follow-up was done every 6–12 months, complications recorded and managed appropriately. Results All the patients were males. The mean age at diagnosis of a peripheral aneurysm was 41.0 ± 9 years. There were 17 (53%) femoral, 8 (25%) popliteal, two carotid, two external iliac, two brachial and one internal iliac aneurysms. Fourteen (61%) patients had a single peripheral aneurysm while nine had two. Surgery was performed for all initially presenting 23 aneurysms. Six patients with multiple peripheral aneurysms had surgery for their second asymptomatic aneurysm. The mean follow-up period was 84 ± 62 months. Of 29 aneurysms operated on, 7 (24%) anastomotic pseudo-aneurysms and 11 (38%) graft occlusions developed. Five (22%) patients underwent major lower extremity amputations. Six (26%) mortalities were recorded. Conclusion Surgery for peripheral aneurysms in BD is warranted in many instances. Results of operation can be improved by prolonged monitoring. However, despite all efforts, peripheral aneurysm involvement in BD worsens the prognosis.

Behçet’s Disease

Neurologic involvement in Behçet's disease (BD) is seen in about 5% to 10% of all BD patients. Clinical and imaging data suggest that neurologic involvement in BD presents in two major forms. The first, central nervous system (CNS) parenchymal involvement with a predilection to brainstem-diencephalic regions, is seen in the majority of patients with neuro-BD (NBD). The second form is cerebral venous sinus thrombosis (CVST), which is seen in up to 20% of cases. BD is very rare in children, but when it does occur, the patterns are reversed: most children with NBD present with CVST. Other syndromes such as spinal cord involvement, arterial CNS involvement, optic neuritis, aseptic meningitis, and peripheral neuropathies may be seen, but are rare. Venous sinus thrombosis in BD has a significantly better neurologic prognosis than parenchymal CNS involvement. There is no Class I evidence regarding treatment of parenchymal CNS involvement or CVST in BD. Current treatment applications are based largely on expert opinion; none are evidence-based. Acute parenchymal CNS involvement should be treated with high-dose intravenous methylprednisolone (IVMP), 1g per day, for 5 to 10days, followed by either a prolonged oral taper or intermittent IVMP pulses with a low oral dose between the pulses, over 6months. After treatment of the acute attack, long-term maintenance with immunosuppressive agents should be considered in patients with parenchymal CNS involvement, as this form may follow a relapsing or secondary progressive course and may result in significant physical and cognitive deficits leading to severe neurologic disability. A number of randomized controlled studies have tested treatments for systemic manifestations of BD. Colchicine was found to be effective for mucocutaneous symptoms, thalidomide was found to be effective in erythema nodosum-like skin lesions, azathioprine and cyclosporine were shown to be effective in BD uveitis, and cyclophosphamide was shown to be effective for major vascular involvement. More recently, interferon alfa and anti-TNF agents were also shown to be effective in BD uveitis. Although randomized controlled studies have not been performed in NBD, the most widely used long-term therapeutic agent is azathioprine. Recent observations suggest that the addition and long-term use of azathioprine in NBD could be associated with a more favorable course. A growing number of case reports in recent years suggest that anti-TNF agents may be an effective alternative in NBD, but current experience with these agents is limited. CVST in BD is also treated with steroids. The addition to glucocorticoids of anticoagulation, including short-term fractionated heparin, is controversial, as these patients have a higher probability of harboring pulmonary or other aneurysms, which may be associated with an increased risk of bleeding. Long-term oral anticoagulation is unnecessary. Interestingly, the prognosis of CVST due to BD seems to be much more favorable than the prognosis of CVST due to other causes, with much less tendency for venous infarcts and seizures. However, as recurrences may occur, long-term treatment with azathioprine is recommended.

Silent central nervous system involvement in Egyptian Behçet's disease patients: clinical, psychiatric, and neuroimaging evaluation

Behçet's disease (BD) is an idiopathic multisystem disorder. Involvement of CNS occurs in 4-48% of cases. This study was designed to evaluate the prevalence of subclinical neuropsychiatric affection in asymptomatic Egyptian BD patients using psychometric tests and brain imaging with single photon emission computed tomography (SPECT) and magnetic resonance imaging (MRI), also to assess possibly associated clinical predictive variables. Twenty-five BD patients without overt CNS involvement and ten healthy controls matched for age, education, and sex completed a comprehensive neuropsychological battery including Hamilton scales for anxiety and depression and Wechsler memory scale-revised. Disease activity was assessed using Behçet's Disease Current Activity Form (BDCAF). SPECT was done for all subjects, and 12 patients underwent brain MRI. Compared to controls, 23 (92%) and 24 (96%) patients had anxiety and depression scores respectively above normal range; also, BD patients had significantly lower memory quotient (MQ). SPECT revealed abnormalities in 16/25 (64%), while in 3/12 patients (25%), MRI was abnormal. Subjects with abnormal SPECT had significantly higher ages than those with normal SPECT (P = 0.02) and were more frequently males (P = 0.03). No statistically significant differences between cases with normal or abnormal SPECT were found regarding disease duration, frequency of headache, BDCAF, frequency of active eye disease, major vascular involvement, mean Hamilton anxiety and depression scores, and mean MQ. Early diagnosis of neurological involvement in BD is important in reducing or preventing complications. Neuropsychiatric evaluation and HMPAO brain SPECT were found to be useful for detecting subclinical neurological abnormalities in BD patients.

Neurologic Soft Signs in Behcet Disease

Patients with Behcet disease have an excess of minor neurologic abnormalities (neurologic soft signs). (a) To investigate the neurologic soft signs (NSS) in Behcet disease (BD) patients who had no neurologic symptoms, by using the neurologic evaluation scale (NES). (b) To evaluate the effect of silent infarction on NES scores in BD patients. Thirty six BD patients without neurologic symptoms and 36 healthy controls were included in the study. NSS were assessed with the NES. Cranial magnetic resonance imaging was conducted to determine the presence of silent cerebral infarcts. Patients with BD had significantly higher scores overall and on each subscale (except for subscale "others") of the NES than the control group's. Tandem walk, adventitious overflow, tremor, graphesthesia, fist edge palm test, Ozeretski test, finger thumb opposition, mirror movements, extinction, synkinesis, convergence, finger nose test, glabellar reflex, grasp reflex, and suck reflex were also significantly higher in patients with BD than in the healthy control group. There were no significant differences in the total NES scores, total subscale scores and each of the NES items between BD patients with silent infarction and those without infarction. Early diagnosis of neurologic involvement in BD is important and the NES is a useful instrument for detecting subclinical neurologic involvement in BD patients.

Laryngeal involvement in Behcet’s disease—a challenge for treatment

A patient with severe Behcet's disease (BD) was referred to our clinic because of severe pharyngolaryngeal involvement. This complication developed under treatment of prednisone and etanercept (Enbrel) 25 mg twice a week. A surgical approach was considered, but following three daily doses of Solu-Medrol (500 mg each) and 300 mg infliximab on the fourth day, the patient improved significantly. This is the first case reporting treatment with infliximab in laryngeal involvement of BD. Although some cases may still require surgical intervention, the therapeutic option of anti-TNF agents, specifically anti-TNF antibodies such as infliximab, may decrease the need for the surgical option.

Les manifestations neurologiques de la maladie de Behçet : étude de 67 patients

Neurological manifestations of Behçet disease (BD) are polymorphic and serious. The purpose of this study was to analyze the clinical patterns and outcome of neurological involvement in BD. The medical records of patients with neurological manifestations of BD were reviewed retrospectively. All patients fulfilled the International Study Group Criteria for the Diagnosis of BD. Patients with headache and normal findings (neurological examination, cerebrospinal fluid, computed tomography scan, magnetic resonance imaging) were excluded. Sixty-seven patients had clinical evidence of neurological involvement. There were 53 men and 14 women. A male/female sex ratio was 3.78. The average age of onset of neurological involvement was 31.5. The meningoparenchymal (MP) central nervous system involvement was found in 83.58%. The most common findings were pyramidal signs. Eleven patients (16.41%) without parenchymal central nervous system involvement were noted. In this group, there were six cases of intracranial thrombosis, one case of intracranial aneurysm and four cases of idiopathic intracranial hypertension. Other clinical features were reported: extrapyramidal signs and isolated spinal cord involvement. The course of disease was favorable in 70% of cases, and unfavorable in 30%. Clinical patterns of neurological involvement in BD are various and particularly serious in the MP group.