[The Behcet syndrome (Gilbert's ophthalmia lenta)].
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Behcet syndrome, a multisystem disorder characterized by ocular, mucocutaneous, articular, vascular, gastrointestinal, and neurologic abnormalities, is described in six pediatric patients. The patients ranged in age from 2 months to 11 years at time of onset. Several years were usually required before additional manifestations of the disease occurred. Aphthous ulceration was present in all six patients, arthritis in three, erythema nodosum in four, sterile cellulitis in three, gastrointestinal manifestations in five, neurologic manifestations in two, and genital or perianal ulcerations in three; ocular involvement was present in only one. There were no diagnostic laboratory studies, and, as in the adult population, no cause for Behçet syndrome was found. Patients had a variable response to corticosteroid therapy. Two patients who had significant morbidity and who responded poorly to corticosteroid therapy also received chlorambucil therapy, which appeared to provide improved control of signs and symptoms. Based on the prolonged interval between onset and the appearance of complete manifestations, Behcet syndrome may be more common in children than previously reported.
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A thirty-six year old male with a sixteen month history of a progressive neurological disorder had Behcet's disease. His initial electroencephalogram showed periodic lateralizing epileptiform discharges (PLEDs). The electroencephalographic changes in this disorder have not been described in detail in the American literature. The review of world publications in this matter disclosed that there is no specific EEG pattern in this condition, and that electroencephalographic changes are mainly related to the side of the lesion and have no prognostic value.
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