Hombre joven con déficit motor y lesiones inflamatorias intracerebrales: reporte de un caso clínico de Neuro-Behcet / Young man with motor deficit and intracerebral inflammatory lesions: a Neuro-Behcet case report

La enfermedad de Behçet (EB) es una patología inflamatoria vascular, multisistémica y recurrente. El fenómeno subyacente es una perivasculitis que afecta el territorio arterial y venoso. La mayor prevalencia de la enfermedad se encuentra en el Oriente y cuenca del Mediterráneo, en la llamada “Ruta de la Seda”. La prevalencia en Chile es desconocida. Las manifestaciones clínicas más frecuentes son úlceras orales y genitales recurrentes, uveítis, artritis, compromiso de sistema nervioso y vascular. El compromiso de SNC (Neuro-Behçet) varía entre 5 por ciento y 13 por ciento, y se divide en dos tipos: Intra-axial o parenquimatoso, más frecuente, más grave, con lesiones inflamatorias en tronco del encéfalo, en unión meso-diencefálica y región pontobulbar. Puede extenderse hacia diencéfalo, bulbo y médula espinal. El compromiso hemisférico es menos frecuente. Extra-axial, que se presenta como trombosis de senos venosos, y aneurismas, estenosis y disección arterial. El tratamiento del Neuro-Behçet intra-axial se basa en esteroides en dosis altas asociados a inmunosupresores, entre ellos, y dependiendo de la severidad, Metotrexato, Azatioprina, Ciclofosfamida, Micofenolato, y Anti-TNFα en casos refractarios o severos.

The highest prevalence of the disease is found in the East and the Mediterranean basin, in the so called “Silk Road”. The prevalence in Chile is unknown. The most frequent clinical manifestations are recurrent oral and genital ulcers, uveitis, arthritis, and CNS involvement (Neuro-Behçet). Neurological disease varies between 5 percent and 13 percent, and it can be divided into two types: Intra-axial or parenchymatous: more frequent, more severe, with inflammatory lesions involving the brainstem, midbrain, diencephalon and pontobulbar regions. It may extend into the spinal cord. Involvement of the brain hemispheres is less common. Extra-axial: also called “Neurovasculo Behçet”, less common and with better prognosis. It can manifests as venous sinus thrombosis, and aneurysm, stenosis, or arterial dissection of intracerebral or extracranial arteries. Treatment of Intra-axial Neuro-Behçet is based on high-dose steroids associated with immunosuppressive agents like: Methotrexate, Azathioprine, Cyclophosphamide, Mycophenolate, and anti α-TNF in refractory or severe disease.

Diagnosis of Intestinal Behcet's Disease / 대한소화기학회지

Due to similar manifestations of intestinal Beh?et's disease (BD) to those of other colitis such as Crohn's disease or intestinal tuberculosis, it is still challenging for gastroenterologist to accurately diagnose intestinal BD in patients with ileo-colonic ulcers. Moreover, no reliable diagnostic criteria for intestinal BD have been developed yet. Therefore, IBD Study Group of KASID was formulated to establish the guideline for the diagnosis of intestinal BD using a modified Delphi process. The novel diagnostic criteria for intestinal BD were developed based on two aspects; colonoscopic findings and extra-intestinal systemic manifestations, in which patients were categorized into 4 groups including definite, probable, suspected, and non-diagnostic for intestinal BD. Furthermore, Disease Activity Index for intestinal BD was developed through a Korean multicenter study. These diagnostic and disease activity guidelines will contribute to understand intestinal BD.

Síndrome de Behçet / Behçet syndrome

Behçet syndrome is a vasculitis of a unclear pathogenesis, having diverse clinical diagnosis and manifestations which compromise different organs. In this article we review some of the pathogenic and clinical elements, diagnostic criteria, activity levels and severity of the syndrome. Emphasis is placed on the angio-behçet and neuro-behçet manifestations and treatment.

Síndrome antifosfolipídico / Syndrome antiphospholipid

Entre 1983 y 1986 Hughes describió un síndrome clínico con trombosis arteriales y venosas diseminadas asociadas a la presencia de anticuerpos dirigidos contra fosfolípidos. Inicialmente lo denominó síndrome anticardiolipina. En la actualidad se lo denomina más apropiadamente síndrome antifosfolipídico

Survey and Validation of the Criteria for Behcet's Disease Recently Used in Korea: a Suggestion for Modification of the International Study Group Criteria

The criteria employed for Korean Behcet's disease (BD) from January 1990 to December 2000 have been investigated, and the diagnostic validity for those criteria was determined. For the generation of a modified set of preliminary criteria from the International Study Group (ISG) criteria, the diagnostic values for individual feature of BD were calculated. The criteria by the Behcet's Disease Research Committee of Japan appeared to be widely employed with the ISG criteria. However, because the ISG criteria revealed a relatively valid outcome in Korea, the application of this criteria will be needed for the universal unification until the criteria with better performance comes out. On the other hand, the modified set of preliminary criteria that consisted of the clinical items with better results seemed to improve some pitfalls of the ISG criteria. Although that criteria showed better performance than the preexisting criteria, it should be necessary to validate its effectiveness in other areas.

The Comparison Between Behcet's Disease and Spondyloarthritides: Does Behcet's Disease Belong to the Spondyloarthropathy Complex?

This study was to clarify whether Behcet's disease (BD) could be classified into the spondyloarthropathy (SpA) complex. It was undertaken on 58 patients with BD (BD group), 56 patients with SpA (SpA group), and 3 patients who concurrently satisfied the criteria for BD and SpA (BDSpA group). The clinical parameters and known susceptible HLA antigens were compared between BD group and SpA group. In addition, 3 patients in BDSpA group were reviewed. The prevalence of definitive sacroiliitis (SI) in BD group and SpA group was 46.4% and 5.2%, respectively. However, none had a definitive SI in healthy controls. Enthesitis was observed in 3.4% of BD group and in 50% of SpA group. The patterns of eye involvement were different between these two groups. HLA-B27 was negative in all 49 patients of BD group, whereas it was positive in 67.9% of SpA group. The prevalence of HLA-B51 was 51.7% in BD group, and that in SpA group was 21.4%. One patient in BDSpA group was considered to have concurrent BD and ankylosing spondylitis (AS). Another patient was closer to AS, and the third to BD. Conclusively, it seems that BD could not be classified into the SpA complex.

Behcet's disease in Bahrain, clinical and HLA findings

To study the clinical features and the HLA findings of Behcet's disease in Bahrain. Method: A retrospective study of nine patients with Behcet's disease from the Salmaniya Medical Complex who were treated over a 15-year period are analysed. The male to female ratio was 3.5:1. Oral ulcers were present in all patients, genital ulcers in 88.9%, skin lesions in 66.7%, ocular involvement in 44.4%, arthritis in 44.4%, epididymitis in 57% of the males, and DVT in 22.2%. HLA B5 was positive in 66.7% of the patients. 66.7% of the cases had the HLA B5 allele. Their clinical manifestations are similar to those reported in the other Gulf countries

Diagnostic criteria of Behcet's disease: problems and suggestions

There are no specific diagnostic laboratory tests for Behcet's disease. Diagnosis relies on proper history-taking and typical clinical manifestations. To provide more objectivity to the diagnosis, several diagnostic criteria have been introduced including one major set of guidelines by the International Study Group for Behcet's Disease (ISGBD) in 1990 which has made a significant contribution, although some disagreements exist in interpretation. Based on this criteria, recurrent oral ulceration is an obligatory manifestation for the diagnosis of Behcet's disease; however, some data indicates it is not a requirement for the diagnosis. In this article the author critically reviews different accepted diagnostic criteria or classifications of Behcet's disease and gives recommendations for a more thorough diagnosis.

Epidemiological and clinical features of Behcet's disease in Korea

Behcet's disease occurs with a high prevalence in the Far East including Korea. In this report we inspected 1,155 patients with Behcet's disease and collected information concerning the clinical and epidemiologic features of Behcet's disease in Korea. In summary, patients in their 30s were the most common, with the most common age of onset in the 20s; the sex ratio was 0.63:1 with female predominance; according to revised Shimizu's classification, the order of frequency of the different types was incomplete (38.2%), suspected (25.4%), possible (20.3%) and complete (16.1%); oral ulcers were the most frequent major symptom (97.5%) and the most common initial symptom (78.5%); erythema nodosum-like lesion (55.3%) was the most common type of skin lesion; a seasonal aggravation of the disease was observed in 21.3% of patients; and the most common type of childhood onset Behcet's disease was suspected type.

Doença de Behçet: revisäo baseada na 6a. Conferência Internacional - Paris, 1993 / Behçet's disease: review established on 6th International Conference - Pris, 1993

Breve revisäo sobre aspectos etiopatogênicos, epidemiológicos, dermatológicos, critérios de classificaçäo e terapêutica da doença de Behçet, abordados na 6a. Conferência Internacional da Doença de Behçet, em Paris, França, em 30 de junho e 01 de julho de 1993

Temporal changes in the clinical type or diagnosis of Behcet's colitis in patients with aphthoid or punched-out colonic ulcerations

The intestinal lesion of Behcet's colitis shows aphthoid or punched-out ulceration. However, the diagnosis of Behcet's colitis should be based on the presence of other stigmata of Behcet's syndrome, since these morphological characteristics are not pathognomonic by themselves. Furthermore, the stigmata of Behcet's syndrome could appear simultaneously or separately with intervals of several months to years. Besides, when a physician first meets patients with intestinal ulcerations of aphthoid or punched-out shape, if they do not have any stigma of Behcet's syndrome, the physician has some difficulty in making a diagnosis of Behcet's colitis. The purpose of this retrospective study was to investigate the followings: 1) The upgrade in clinical type of Behcet's colitis with the advance of time. 2) What portion of the patients with aphthoid or punched-out ulcerations, but without any other clinical feature of Behcet's syndrome, could be diagnosed as Behcet's colitis with the advance of time? During the mean follow-up period of 38.2 months, 4 (22.2%) out of 18 patients with Behcet's colitis upgraded their clinical types. In the nonspecific ileocolitis group, who had no major stigma of Behcet's syndrome on their initial visit, 3 (30%) out of 10 patients were subsequently diagnosed as Behcet's colitis during the mean follow-up period of 33.3 months. From these results, we could conclude that in possible or suspicious cases of Behcet's colitis, a more confident diagnosis could be made by close observations for new developments of major stigma of Behcet's syndrome. Even in cases of nonspecific ileocolitis, the diagnosis of Behcet's colitis could be made in a significant number of cases as time goes by.

Prognostic comparison of Behcet's disease with or without HLA-Bw 51 antigen

To know the prognosis of Behcet's disease with HLA-Bw 51 antigen, comparison studies of 24 cases of Behcet's disease were investigated. The sex distribution was 14 males (58%) and 10 females (42%). The patients were grouped into three clinical types: the complete type with 11 cases (45%), the incomplete type with eight cases (33%) and the suspect type with five cases (22%), according to the criteria established by the Behcet's Disease Research Committee in Japan (1982). They were also divided into three ocular types according to the location of the inflammation: the anterior segment type, the fundus type and the mixed type. They were divided into 10%, 10% and 60% in HLA-Bw 51 negative group and 14%, 22% and 64% in HLA-Bw 51 positive group, respectively. Skin lesions observed in 30% of the HLA-Bw 51 negative group and 70% of the HLA-Bw 51 positive group, which was statistically significant (p 0.1).

Behcet's Syndrome in Korea: A Look at the Clinical Picture

Behcet's syndrome is being detected increasingly in Korea. To collect and document information concern- ing the clinical picture as it is records of patients seen in our clinic in the period between November 1983 and March 1986 were used as source material. In summary, it was found that (1) the total number of patients who had two majors symptoms at least were 410, including 170 men and 240 women; (2) Patients in their thirties were the most common, the mean age being 34.6 years; (3) according to Lehner's classification, the order of frequency of the different types was mucocutaneous (52%), ocular (33), arthritic (14%) and neurologic (1%) and according to Shimizu's classification, it was incomplete (39%), suspected (37%), and complete (24%); (4) oral ulcers, which had observed in 407 patients were the most frequent major symptom, followed by genital ulcers in 338 patients.