Electroencephalographic findings in urea-cycle disorders |
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Authors: | N P Verma Z H Hart K A Kooi |
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Affiliation: | 1. School of Mechanical Engineering, Pusan National University, Busan 609-735, Republic of Korea;2. LG Electronics, Changwon 614-711, Republic of Korea |
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Abstract: | Eleven electroencephalograms in 4 infants with urea-cycle disorders were reviewed. All infants had one or more abnormal EEGs. The abnormalities consisted mainly of multiareal spikes, spike-waves, or sharp-and-slow-wave activity. In addition, one patient, a term infant, exhibited exaggerated spindle-delta bursts. This infant, and also one other at a similar age, had monorhythmic paroxysmal theta activity. Clinically, all patients had seizures shortly preceding abnormal EEGs. EEG alterations were encountered over a wide range of elevated serum ammonia levels. Normal EEGs occurred in the face of slightly elevated levels. It is concluded that epileptiform EEG alterations may be a characteristic manifestation of urea-cycle disorders. |
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