Remodeling of cell surface glycoproteins by N-acetylglucosaminyltransferase III gene transfection: modulation of metastatic potentials and down regulation of hepatitis B virus replication

OBJECTIVE: To determine the long-term prognosis of neurologic involvement in Beh?et syndrome. DESIGN: Forty-six patients with Beh?et syndrome, who had been the subjects of a previous report with short-term follow-up, were reexamined 7 years later; 42 of them could be reexamined neurologically. Neuropsychological testing, magnetic resonance imaging, electromyography, and evoked potential studies were performed when available. RESULTS: Of the 27 patients who had had headaches without any neurologic symptoms or signs previously, 2 had developed an acute neurologic attack. In addition, 7 patients in this group showed minor abnormalities on neurologic examination and/or other laboratory investigations, without history of any attacks. Among the previous neuro-Beh?et group (n = 15), as defined by the presence of neurologic signs or symptoms, other than headache, 7 had a stationary course, while 8 had been progressive. Three of the latter group had died. Patients with progressive course had had abnormal cerebrospinal fluid findings at the time of the previous report, whereas patients with a stationary course had not. CONCLUSIONS: Silent neurologic involvement may occur in Beh?et syndrome. Patients should undergo periodic neurologic evaluation. The long-term prognosis in neuro-Beh?et syndrome does not seem to be as favorable as we observed in short-term follow-up. Cerebrospinal fluid findings may predict prognosis.