Head injuries in pediatric patients are a common presentation in an acute care setting, and clinicians must decide whether the pediatric patient should undergo cranial computed tomography (CT). There are many CT scanners available in Japan, rendering immediate CT examinations possible in the emergency departments. However, the risk of radiation-induced cancer has been reported to be higher in Japan compared with that in Western countries. To date, three clinical decisions rules (PECARN, CATCH, and CHALICE) have been reported to determine the need for CT in children with head injuries. These clinical decision rules have been developed to identify children at a high risk of intracranial injuries, aiming to assist clinicians in minimizing the number of CT scans. If however, when the family of a pediatric patient expects a head CT examination to determine the extent of the injury, the clinicians should recommend CT. Furthermore, it is important to assess the suitability of the head CT scanner used on the basis of the clinical decisions rules and to explain care post head injuries.

  Objective: Seizure prophylaxis is indicated for various conditions. Although fosphenytoin (FOS) administered intravenously is used for seizure prophylaxis in children, it is associated with significant side effects. On the contrary, levetiracetam (LEV) administered intravenously may be also effective as a prophylaxis agent for seizures, and fewer adverse effects have been reported with its use. In this study, we compared the efficacy and safety of LEV and FOS for seizure prophylaxis in Japanese pediatric patients with seizures. Methods: This retrospective review included 35 patients with seizures aged 4-15 years, who were injected with LEV or FOS for seizure prophylaxis after seizures. Twelve and 23 patients who received LEV and FOS, respectively, were selected. Demographic data and outcome assessments were compared. Results: Subjects had a median age of 9.8 years and included 20 male and 15 female patients. Baseline characteristics of the treatment groups were similar. No significant difference was found in the probability of seizure recurrence (within 12 h of seizures following intravenous administration) between LEV and FOS groups. There were no side effects that required treatment in both groups. Conclusions: The efficacy and incidence of adverse events of intravenous LEV after seizures were equivalent to those of intravenous FOS. LEV is a reasonable alternative to FOS for prophylaxis of seizure recurrence.

  An 11 month-old boy and his father both with congenital hemiplegia were given a diagnosis of intrauterine periventricular venous infarction by their MRI. An additional cerebellar hemorrhagic lesion was defected in the boy. Whole exome sequence revealed a mutation in COL4A1 (NM_001845.5:c.4875C>A [p.Tyr1625*]), which should be examined in familiar patients with cerebral hemorrhagic lesions or when there are multiple hemorrhagic lesions.

  Tuberous sclerosis complex (TSC) is pathologically characterized by the presence of tumor-like lesions in multiple organ systems and central nervous system manifestations, such as epilepsy as well as intellectual and cognitive-behavioral disorders. Epilepsy in TSC appears in childhood and is often intractable. Here, we report the use of everolimus for an 8-year-old boy with TSC accompanied by subependymal giant cell astrocytoma (SEGA) who had intractable epilepsy. His epilepsy started when he was 4 years old, and he was diagnosed with TSC. Although he received antiepileptic drugs, his epilepsy continued. Furthermore, when SEGA progression was observed at an age of 8 years, everolimus treatment was administered. After the administration of everolimus, he became seizure-free. On electroencephalograms (EEGs), diffuse spike-and-waves observed before the initiation of everolimus improved to focal small spikes. His intelligence quotient (IQ), which was 77 before the initiation of everolimus, had improved to 104 after 1 year. His ADHD-RS score had improved from 15 to 3 after 3 years. Everolimus is expected to ameliorate epilepsy and improve intellectual and cognitive-behavioral manifestations. The treatment of TSC has changed by the introduction of mTOR inhibitors. For each case, symptoms including cognitive behavior manifestations should be assessed over time, and the most suitable treatment should be searched for.

  We report a 1-year-old boy with infantile type dentatorubral-pallidoluysian atrophy (DRPLA). He developed normally until the age of 5 months. He had no epileptic seizures, and there was no family history of epilepsy. He visited our hospital because of development delay and hypertonia. Spinocerebellar degeneration was suggested on MRI, and he was diagnosed with DRPLA based on 93 repeats of CAG in ATN1. DRPLA patients with infant onset exhibit atypical symptoms such as abnormal muscle tone and developmental retardation. However, it may be difficult to diagnose DRPLA because these clinical findings are similar with those for cerebral palsy. Atrophy of the cerebellum and tegmentum of the pons is an important MRI finding for the early diagnosis of DRPLA. We also mention that we should build a medical team including multiple profession to inform genetic results and provide psychosocial support for the family members with DRPLA.