Search

We report two female infants with early myoclonic encephalopathy (EME) whose intractable focal seizures were suppressed with lidocaine and carbamazepine (CBZ). Although EME is a form of early-onset epileptic encephalopathy characterised by myoclonus and focal seizures that are highly resistant to treatment, lidocaine and CBZ may prove effective in treating this disorder. Future studies should be performed in order to determine whether there are common specific mechanisms of seizure generation related to the sodium channel in these patients.

Fibroblast growth factor 23 (FGF23) is a potent regulator of Pi and 1,25-(OH)(2)D homeostasis. Early postpartum infants show intriguing changes in serum levels of Ca, Pi, PTH and 1,25-(OH)(2)D. However, the role of FGF23 in the early neonatal mineral metabolism has not been clarified. In order to evaluate the significance of FGF23 during the early postpartum period, we examined the circulating FGF23 levels using an intact FGF23 ELISA and a C-terminal FGF23 ELISA either in 22 umbilical cord blood samples (the cord blood) or in 22 term infants at 5 days of life (the 5-day-old infant). We also compared these ranges with those of 11 healthy adults. Data were expressed as mean+/-SD, and analyzed by two-way ANOVA, followed by the Tukey's test. C-terminal FGF23 in the cord blood, the 5-day-old infants and the healthy adults were 73.3+/-22.4, 81.0+/-28.2 and 39.0+/-7.8 RU/ml, respectively. Intact FGF23 in the cord blood, the 5-day-old infants and the healthy adults were 3.9+/-1.6, 21.8+/-17.6, and 27.6+/-7.3 pg/ml, respectively. Immunoprecipitation assays using anti-FGF23 antibodies demonstrated that the intact 32 kDa FGF23 was low and the fragmented FGF23 of 18 kDa was abundant in the cord blood compared with those in the healthy adults. In conclusion, our observations indicated that the intact FGF23/C-terminal FGF23 ratio was very low due to the fragmentation of FGF23 during the early postpartum period and might have a considerable contribution to the Pi homeostasis in the healthy term infants.

116 immunizations were given to 61 children with febrile convulsion or epilepsy who had not had a seizure for 1 year since the last attack. In 92 of the 116 immunizations the electroencephalogram (EEG) was examined before and after immunization. No adverse effects on the EEG were observed in 19 immunizations with Japanese encephalitis, measles, mumps or rubella vaccines. Epileptic spikes reappeared after 10 immunizations and epileptic spikes increased after 10 immunizations among 73 given for diphtheria, acellular pertussis and tetanus (DPT), diphtheria and tetanus (DT), or Bacillus Calmette-Guerin (BCG). A convulsion was observed once in one child 7 days after immunization with BCG. A follow-up EEG examination is necessary after children with convulsive disorders are immunized.

Antibody-dependent cellular cytotoxicity (ADCC) and natural killer (NK) activity were examined using MT-2 cells persistently infected by HTLV-1 as target cells, and mononuclear cells as effector cells, from healthy one-week-old newborn babies, infants, children and adults. More than 10% of ADCC was observed in 17 newborn babies out of 22 (77.3%) and in all 67 healthy one-month-old babies to adults, by adding serum from anti-HTLV-1 positive carriers. When anti-HTLV-1 negative serum was added, less than 10% of ADCC was observed. If infants without anti-HTLV-1 antibodies were breast-fed they had the possibility of HTLV-1 vertical transmission. There was no significant decrease in NK activity between 90 healthy newborn babies, infants, children, or adults. These results suggest that ADCC and NK activity protect against the transmission of HTLV-1 from mother to child.

Intravenous acyclovir and vidarabine were compared in the treatment of varicella-zoster virus (VZV) infection in 25 immunocompromised children--13 with acute lymphocytic leukemia, three with other types of cancer, two with immunodeficiency and in seven undergoing prednisolone treatment. Thirteen had varicella and 12 had herpes zoster. Acyclovir was given intravenously to five patients with varicella and to four with herpes zoster at a dose of 5-10 mg/kg every eight hours. Vidarabine was given intravenously to eight patients with varicella and to eight with herpes zoster at a dose of 10 mg/kg/day. In varicella, vidarabine significantly shortened the time from the start of treatment to cessation of new lesion formation compared with acyclovir. However, there was no significant difference in time to complete crusting between the two treatments. In herpes zoster, acyclovir significantly shortened the time from the onset of the skin lesions to complete crusting. A slight raise of GOT in two cases was reported. While acyclovir and vidarabine were equally effective for VZV infection, in herpes zoster acyclovir was more effective.