[Meningomyeloencephalitis in the Behçet syndrome].
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Twelve observations of Behcet's syndrome are described, which comply with the diagnostic criteria of O'DUFFY. The clinic symptomatology is reviewed and compared with those found by other authors.
We studied the plasminogen activator levels in plasma samples of 37 patients with Behcet's syndrome. A significant decrease of plasminogen activator activity level was shown by the euglobulin lysis time method, the amidolytic activity level (Testzym method), and the fibrin plate method. Such a decrease may be associated with the severity of Behcet's syndrome.
BACKGROUND: Sweet syndrome (SS), an acute inflammatory disease, has clinical and laboratory features similar to those of Behçet disease (BD). Serum levels of granulocyte colony-stimulating factor (G-CSF) are elevated in patients with SS, and exogenous administration of G-CSF has repeatedly been implicated in the causation of SS. Granulocyte colony-stimulating factor is a hematopoietic growth factor that regulates the production and differentiation of neutrophils. OBJECTIVES: To clarify the role of elevated serum G-CSF levels in patients with active SS and active BD compared with those with inactive SS or BD and healthy controls. To then analyze neutrophil apoptosis in the active state of SS and BD; and to also investigate the influence of autologous serum on neutrophil apoptosis. METHODS: Serum G-CSF was examined in 5 patients with active SS, 7 with inactive SS, 7 with active BD, 9 with inactive BD, and 5 healthy controls by means of an enzyme immunoassay kit. We measured apoptotic cells in the neutrophil fraction of peripheral blood collections in patients with active diseases and controls by means of flow cytometry. RESULTS: Serum G-CSF level was significantly higher in patients with active SS than in those with inactive SS. The difference in serum G-CSF levels among patients with active and inactive BD was also significant. Serum G-CSF level was significantly higher in patients with active SS than in those with active BD. Neutrophil apoptosis was significantly higher in patients with active SS than healthy controls. This increased apoptosis rate was also seen in patients with active BD. The increased rate of neutrophil apoptosis was significantly suppressed when the neutrophils were cultured for 18 hours in the presence of autologous active SS serum. Similarly, neutrophil apoptosis was suppressed in the presence of autologous serum in patients with active BD, but not significantly so. CONCLUSIONS: These findings indicate that increased production of G-CSF in patients with SS and BD may play an important role in the manifestation of these disorders. Given the suppression of neutrophil apoptosis in the active state in the presence of the influence of autologous serum, which includes elevated G-CSF level, we propose that serum G-CSF plays a significant role in the suppression of neutrophil apoptosis. Furthermore, G-CSF-induced suppression of neutrophil apoptosis appears to be deeply involved in the pathogenesis of SS and BD.
Conventional imaging with computed tomography (CT) and magnetic resonance imaging (MRI) may show abnormalities in central nervous system Behcet's syndrome but is normal in some cases. Recently in two cases positron emission tomography has shown abnormalities in blood flow and glucose metabolism far more extensive than the abnormalities seen on CT and MRI scans in the same patients. We report a patient with neuro-Behcet's syndrome presenting with headache and personality change in whom CT and MRI brain imaging was normal, but regional cerebral blood flow imaging using single photon emission tomography with the tracer HMPAO showed extensive perfusion deficits which partially reversed after 3 months of prednisolone therapy. This technique may aid the diagnosis of cerebral involvement in Behcet's syndrome, although the cause and incidence of the perfusion deficits need further evaluation.
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The serial EEGs on 10 cases of neuro-Behcet's syndrome have been investigated and were compared with the clinical symptomatology and autopsy findings in 4 cases. In 8 cases, the EEGs showed a mild to moderate increase in slowed alpha and theta waves and in 5 cases diffuse alpha patterns. In 2 cases, large slow wave patterns were observed and in Case 10, large slow waves changed into low voltage fast dominant patterns following the status epilepticus. In all the cases, the EEG changes seemed to be well correlated with the clinical symptoms. Our present study suggested that the diffuse alpha patterns were only seen in cases with the least affected brain stem diffuse projection system. From the investigation of the sequential alterations of large slow waves in Case 10, it was suggested that a synchronization mechanism would lie in the medulla oblongata.
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HLA typing done in 10 Mexican mestizo patients with Behcet's syndrome revealed that 7 of them had HLA-B5 antigen as compared to a frequency of 31% in a Mexican mestizo control population. These findings are akin to those made in Japan and Turkey but differ from those made in Caucasian populations in the United States and Great Britain. Ethnic groups with a high frequency of HLA-B5 in Asia and America may belong to the same ancestral trunk. The occurrence of Behcet's syndrome in them may be more frequently HLA-B5 positive than in other ethnic groups.