Background: BEHCET disease is a vasculitis with mucocutaneous, ocular, arthritic, vascular, and other manifestations. Its neurologic manifestations (neuro-BEHCET disease) are relatively rare, but they must be thoroughly investigated due to their grave prognosis.Review Summary: The frequency of neurologic manifestations, more common in tnale BEHCET patients, is between 5% and 30%. Both the central and peripheral nervous systems can be involved. Central nervous system manifestations can be divided into 2 main groups: (1) parenchymal involvement, which includes brainstem involvement, hemispheric manifestations, spinal cord lesions, and meningoencephalitic presentations; (2) nonparenchymal involvement, including dural sinus thrombosis, arterial occlusion, and/ aneurysms. Peripheral neuropathy and myopathy are relatively rare. Cerebrospinal fluid analysis reveals pleocytosis and elevated protein levels. Magnetic resonance imaging is the investigation of choice which often reveals iso-/hypointense lesions in T1-weighted images and hyper intense lesions in T2-weighted images, mostly in the mesodiencephalic junction, cerebellar peduncles, and other parts the brainstem. Corticosteroids and adjuvant immunosuppressive therapy are used for parenchymal manifestations, and corticosteroids and anticoagulants are used for treatment of dural sinus thrombosis.Conclusion: Neuro-BEHCET disease must be considered in the differential diagnosis of stroke in young adults, multiple sclerosis, movement disorders, intracranial hypertension, intracranial sinovenous occlusive diseases, and other neurologic syndromes.

BEHCET‘s syndrome, a major cause of posterior uveitis and visual loss in the Third World, has probably existed for more than 4000 years. It is a multisystem, inflammatory disorder whose principal manifestations are oral and genital aphthosis as well as inflammation of the eye, skin, and joints. The principal causes of death are from vascular and neurological involvement. No etiological agent has yet been identified for the disease; current evidence suggests that the normal flora of mucosal tracts induce immunological hyper-reactivity in genetically predisposed individuals.

BEHCET’ s disease is a systemic disease classified among vasculitides. Major manifestations are mucous membrane lesions (oral aphthosis and genital aphthosis), skin manifestations (pseudofolliculitis, erythema nodosum), ocular manifestations (uveitis, retinal vasculitis), joint manifestations, vascular lesions (small to large vessel thrombosis, aneurysm), gastrointestinal manifestations, orchiepididymitis, and some rare manifestations like cardiac, pulmonary, and renal impairment. Diagnosis is mainly clinical. The International Diagnosis Criteria for BEHCET’ s Disease may be of help. The gold standard of treatment for mucocutaneous lesions is colchicine. In refractory cases, levamisole, thalidomide, and dapsone may be of help. For major organ involvement like the eyes and the brain, immunosuppressive drugs and prednisolone are the gold standard. In refractory cases, biological agents are the last resort. For gastrointestinal manifestations, sulfasalazine and prednisolone are the first-line treatment. For vascular involvement, the first line treatment was anticoagulation, but recently it was shown that immunosuppressive drugs and prednisolone were confirmed to be the best. In all refractory cases and for all different organs, the last resort is biological agents.

BEHCET Disease (BD) is an autoimmune disorder with recurrent ocular, vascular, central nervous system, articular, mucocutaneous, and gastrointestinal manifestations with unclear etiology and pathogenesis. The further characterization of inflammatory features of BEHCET’s disease may eventually lead to development of better treatment options. Clinical and laboratory observations suggested an important role of IL-17, IL-21 and neutrophil-mediated process in the pathogenesis of BD. New therapeutic modalities target specific and nonspecific suppression of the immune system. The various non-specific immunosuppressive drugs, used either alone or in combinations, frequently fail to control inflammation or maintain remissions. Due to encouraging clinical results (i.e. Antigenic specification, prolonged survival with acceptable levels of toxicity); antibody-based drugs could be effective for the clinical management of BEHCET’s disease.

Background: The association of BEHCET’s disease (BD) and ankylosing pondylitis (AS) is still a matter of debate. Objective: As the presence of sacroiliac joint (SIJ) involvement is an essential criterion in diagnosis of AS, we decided to determine the revalence of SIJ involvement in BD and compare it with that of a control group. Patients & Methods: We randomly selected two groups of 199 BD patients and 168 non-BD cases (the controls). All cases were over 20 years of age. Standard anteroposterior radiographs of the SIJ were obtained and interpreted by two rheumatologists and a radiologist blinded to the diagnosis. To determine the severity of the condition, the following 5-point scale was employed: Normal (0), pseudo-widening (1), sclerosis (2), erosion (3), and bony fusion (4). To eliminate any doubts, only grades 3 and 4 were considered as sacroiliitis. Both groups were separately evaluated for age ( 30, and >30), and gender. Results were compared using Chi square test. Results: The groups were matched for age and sex: There were 98 (49.2%) females in BD vs. 91 (54.2%) in the control group (p=0.35). The mean?SD age was 35?8.3 years in BD and 35?10 in control group (p=1). The SIJ was involved in 9 (4.6%) patients in BD and 7 (4.2%) patients in control group (p=0.93). Comparisons between the results of the unisexual cohorts revealed no significance either (p=0.68 for males, and p=0.64 for females). The age subdivisions (under- and over-30) again showed no significant difference (p=0.96 and p=0.69 for under- and over-30 patients, respectively). Conclusion: The presence of radiographic signs of SIJ involvement is not mandatory for the diagnosis of AS.