In children with Moyamoya disease, transient ischemic attacks often occur during hyperventilation, and the frequency of attacks usually decreases with advancing age. To elucidate the mechanism of the attacks, the regional cerebral blood flow was measured during rest and/or hyperventilation in children aged 3-16 with Moyamoya disease. Regional cerebral blood flow during rest was significantly higher in younger children with Moyamoya disease, and it progressively decreased with advancing age through childhood. During hyperventilation, regional cerebral blood flow decreased In all the children, although blood flow in younger children was still higher than that in the older children. Nevertheless, transient ischemic attacks were more readily precipitated by hyperventilation in the younger than in the older children. Cerebral metabolic demand is much higher in the first decade of life than in later decades. Therefore, it seems likely that even a moderate reduction in cerebral blood flow can cause metabolic impairment in young children. The most common symptom in children with MMD is transient ischemic attack (TIA), while in adults intracranial hemorrhage occurs more frequently. The incidence of TIAs is highest in the first decade of life and decreases rapidly thereafter.2 The question thus arises as to why TIAs occur more frequently in younger children with MMD and why the frequency of TIAs decreases rapidly with advancing age. In an attempt to resolve these problems, the regional cerebral blood flow (rCBF) was measured in children with MMD, and the relations between their ages, rCBF, and clinical manifestations were examined. Subjects and MethodsTwenty children with MMD were studied, 9 boys and 11 girls aged 3-16 years. In accordance with the occlusive and intermediate stages of Kudo and Fukuda,3 these children were divided into 2 groups: a younger group, aged < 10 years, and an older group, aged > 10 years. These children had suffered only recurrent TIAs since ages 2-10 and had no permanent neurologic deficit at the time of study. The frequency

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A 3 year, 11 month-old Japanese male with congenital hypomyelination is described. Clinical features are delay of motor development, generalized muscle hypotonia and weakness, absent tendon reflexes due to peripheral neuropathy, and normal mental development. Electrophysiologically, nerve conduction velocities could not be measured. Histological examination of the right sural nerve revealed total of absence myelin of most of the myelinated fibers. Electronmicroscopically, there was a concentric network of lamellae formed by double-layered sheets of basement membranes with fragments of Schwann cell cytoplasm around the myelinated fibers, so called "onion-bulbs". These peculiar features were similar to those in the cases reported by Lyon, (1969); Kennedy et al., (1977); Karch et al., (1975); and Anderson et al. (1973).

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In this study, initial hepatic metabolic function was evaluated by determining the arterial ketone body ratio (AKBR) and plasma amino acid concentrations in an experimental orthotopic combined hepatopancreatic transplantation (OHPT), and comparing the same values in orthotopic liver transplantation (OLT). In OHPT, AKBR decreased in the anhepatic phase and recovered to the preoperative value just 1 h after reperfusion. On the other hand, in OLT, the recovery of AKBR took 3 h after reperfusion with a significant difference compared to OHPT (P less than 0.05). Plasma amino acid levels, especially alanine and total free plasma amino acids increased in the anhepatic phase and recovered within 1 h of reperfusion in OHPT. However, they did not recover until 3 h after reperfusion in OLT. This rapid recovery of hepatic metabolic function in OHPT should be attributed to the order of reperfusion in which the reconstruction of arterial blood flow precedes that of portal blood flow. This model is useful for assessing the best way by which the grafted liver can for assessing the best way by which the grafted liver can control the timing, order, rate, and volume of blood that should be released.