[A case of MELAS presenting complex partial status epilepticus].

We reported a 37-year-old man who presented complex partial status epilepticus as the initial symptom of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). He showed fluctuating consciousness disturbance, left homonymous hemianopsia, and paroxysmal conjugated eye deviation to the left. The lactic acid level was elevated in blood and CSF, and ragged-red fibers were observed in the biopsied muscle. MRI demonstrated T2-prolonged lesions in the right occipito-parieto-temporal lobes. Since a mutation of mitochondrial DNA (A3243G) was identified, he was diagnosed as having MELAS. On an ictal record, high amplitude, rhythmic sharp waves were observed at right parieto-temporo-occipital region. High amplitude slow waves were also observed on the right hemisphere, especially in the right frontal lobe. These ictal discharges gradually decreased at their amplitude and in frequency, and then ictal EEG turned to the interictal EEG. During an ictal period, conjugated eye deviation to the left side and consciousness loss were observed. These seizures were observed once every several minutes. During the interictal period, sharp waves and sharp-wave complexes were observed frequently at right parietal and posterior temporal lobes. The venous injection of diazepam (10 mg) normalized EEG quickly. When consciousness loss, especially fluctuating, was observed in the patients of MELAS, complex partial status epilepticus should be considered.

[Successful repair of distal type of aortopulmonary window by deep hypothermia with circulatory arrest].

A one year old boy with the distal type of aortopulmonary window was successfully repaired by the transaortic approach and discharged two weeks after operation. Exposure of the defect was enhanced by using deep hypothermia with circulatory arrest so that aortic cannula and cross-clamp could be removed. 2 months before the operation, he needed the respiratory support during a month because of respiratory syncytial viral infection, cardiac failure and equivalent pulmonary hypertension. Postoperative cardiac catheterization after 4 months revealed that PA pressure decreased to 25/5 mmHg without any medication. Aortogram showed the left single coronary artery.

[A new trial assessment of left ventricular function before and after aorto coronary bypass surgery--application of ejection vector (E.V.)].

In 34 patients underwent aorto coronary bypass, left ventriculography was analyzed and ejection indexes (E.F. 100, E.F. 50, E.V. = 2 X E.F. 50/E.F. 100) were calculated. There was not difference in ejection indexes of patients between who had been complicated with preoperative myocardial infarction and not. E.F. 100 was not changed postoperatively, otherwise, E.F. 50 and E.V. showed significant increase. In 15 patients whose quality of life remarkably was improved postoperative period, E.F. 100 was not changed but E.F. 50 and E.V. were significantly increased. Four patients could not perform exercise test to full dose because of intractable ischemic calf pain. In twelve patients, whose left ventricular function was improved at postoperative exercise test, their E.F. 100 was found to be unchanged, but in contrast, E.F. 50 and E.V. were increased significantly. In conclusion, we found that E.V. could become to be more sensitive indicator of left ventricular function compared with E.F. 100 before and after aorto coronary bypass surgery.