Approximately 25-84% of adolescents have complaints of sleepiness. The major cause of their sleepiness is insufficient sleep, and their perceived amount of required sleep is less in the first half of the school week while it increases in the second half. Adolescents might accumulate sleep debt across weekdays and reduce it during the weekend. The difference in sleep duration between the weekend (holidays and non-school days) and weekdays (school days) is termed weekend catch-up sleep (WeCUS) or weekend oversleep (WeOS), which reflects sleep debt. This review article describes the current status and future problems associated with WeCUS/WeOS among adolescents. The difference in mean sleep duration between the weekend and weekdays among young Japanese (10-19 years old) group increased recently and reached 1.3 hours in 2020. According to the published literature, WeCUS/WeOS is longer in females, increases with age, and is longer in those with an early school start time. However, results associating WeCUS/WeOS with the chronotype, depression, risky behaviors, suicidal ideation, sleep problems, body weight, academic performance, and sleepiness remain inconclusive. Non-school day sleep duration among junior and high school students in Japan is less than the assumed optimal sleep duration of 8.5 hours, therefore, the current WeCUS/WeOS among adolescents in Japan might be insufficient to improve their potential sleep debt. Since it has now been recognized that improving potential sleep debt is an important issue, developing effective solutions to help adolescents with chronic sleep debt, especially in Japan, is urgently needed.

  Objective: More than 5 years have passed since the commencement of short-stay services at SUKOYAKA-Residential Care Institute for Children with Disabilities. We investigated the status of short-stay services and the reasons for patients discontinuing to use the services due to worsening of their physical condition. We also assessed the safety of medical management, our roles, and problems in our institution. Materials and Methods: We investigated 99 individuals with severe motor and intellectual disabilities (SMIDs) who have registered for our short-stay services in March 2022 about age, main disease, presence of the mechanical ventilation, tracheotomy, tube feeding and epilepsy. We aggregated faculty utilization rate from the commencement to March 2022. In addition, we retrospectively explored the reason for early exit using patient medical records. Results: In this study, 67% of the patients were under 20 years old. Regarding their etiology, 77% of them were either congenital or caused by episodes in the perinatal period. Complication rate of tracheotomy was 44%, and eventually 41% of the patients had been on a mechanical ventilator. That of tube feeding was 73% and epilepsy was 68%, respectively. Our institute’s reservation rate was 90%, and the utilization rate was over 80% before the COVID-19 pandemic. Moreover, the percentage of medically dependent (MD) patients with SMIDs and sub-MD-SMIDs among all facility user has increased. About five patients per year left the faculty early due to illnesses (including infection and epileptic seizures). Most of these patients were younger patients or those who required advanced medical care. Conclusion: We predict that infants with unstable symptoms and SMIDs who are receiving advanced medical care will use the short-stay services more in the future. In order to safely conduct short stays with advanced medical care for patients with SMIDs, it is important to carefully collect information from parents and care for the patients in the same way as at their home. Furthermore, we must stabilize their primary disease. Hence, cooperation with other hospitals and information sharing with the attending physician will be future issues. In addition, we should consider the needs of short-stay services for walkable patients with advanced medical care requirements.

  Objective: We investigated the efficacy of intravenous immunoglobulin (IVIG) therapy in acute phase of myasthenia gravis (MG) in children and the characteristics of patients for whom IVIG was effective. Methods: The study included 17 patients (6 boys) (8 children with ocular and 9 with generalized MG) aged ＜15 years who were diagnosed with MG, and received more than 1g/kg of IVIG therapy in acute phase between April 1, 2010 and March 31, 2022. Children in whom the MG activities of daily living score improved by ≥1 within 14 days after IVIG therapy commencement were considered to show a successful response to therapy Those in whom IVIG therapy was effective underwent post-treatment follow-up. Results: Median age at disease onset was 6.0 (0.9-13.9) years, and IVIG therapy in acute phase was administered at median age 6.9 (1.1-14.1) years. IVIG therapy was effective in 8 patients, with significantly more generalized MG in the effective cases (p=0.02). Among patients in whom IVIG therapy was effective, one patient achieved complete remission without medication for ＞2 years. Complete remission without medication for ＞2 years after treatment completion was achieved in four patients who responded to IVIG therapy in acute phase and received maintenance IVIG therapy. Conclusions: The efficacy of IVIG therapy in acute phase was higher in children with generalized MG than in those with ocular MG. When IVIG therapy in acute phase is effective, the use of steroids may be avoided or reduced by shifting to maintenance IVIG therapy.

  Rasmussen encephalitis (RE), a rare immune-mediated disorder affecting one hemisphere, typically develops with refractory focal seizures followed by progressive unilateral cerebral atrophy and dysfunction. Although atypical cases with or without delayed-onset seizures have also been described, little information is available regarding treatment decisions for these extremely rare cases. A three-year-old girl presented with a one-month history of progressive right hemiparesis and dystonia. Brain magnetic resonance imaging revealed left cerebral atrophy and unilateral lesions in the left putamen, globus pallidus, and caudate nucleus. A few days after the first visit, the patient experienced a right unilateral clonic seizure. A biopsy of the caudate nucleus was performed 20 days after the first visit, and the patient was diagnosed with RE. Although hemiparesis and dystonia improved with intravenous immunoglobulin therapy, epilepsy gradually worsened. Her family members observed seizures several times a week, despite treatment with multiple anti-seizure drugs. Long-term video electroencephalography (LT-VEEG) captured frequent seizures of various types during sleep that were too subtle for family members to notice. Functional hemispherectomy was performed 13 months after the onset of epilepsy, and she was seizure-free for 18 months after surgery. She was seizure-free for 18 months after surgery. LT-VEEG is an essential test for evaluating the condition of RE, even if family members are unaware of frequent seizures.

  We report a case of lissencephaly with a new variant in TUBA1A (p.(Ala281Val)) complicated with paroxysmal sympathetic hyperactivity (PSH) characterized by recurrent paroxysmal hypertonia, tachycardia, and elevated body temperature. Using Baguley’s excitatory : inhibitory ratio model, we considered the PSH had occurred due to impairment of the central autonomic network, as the cerebral cortex was thin and the deep white matter was almost absent. PSH is associated with extensive brain damage such as head trauma and hypoxic encephalopathy ; however, the present case expands causal variability to brain malformations. Administration of gabapentin (GBP), a GABA agonist, was effective to control PSH. We considered the PSH was suppressed by GBP which reduced non-nociceptive influx stimuli in spinal cord, affected as a GABA agonist to enhance inhibitory input in brain and spinal cord, and eventually weakened efferent input to sympathetic and motor nerves. In order to elucidate the pathogenesis of PSH associated with cerebral dysplasia and to establish therapeutic strategies, it is necessary to accumulate the same cases.

  Acute cerebellitis, a disease with a good prognosis, sometimes presents as fulminant cerebellitis, which causes cerebral herniation. We experienced fulminant cerebellitis positive for anti-glutamate receptor (GluR) antibody in the cerebrospinal fluid that resulted in an imminent cerebral herniation owing to hemicerebellitis. The case was a 5-year-6-month-old girl who presented with fever and vomiting ; on the second day of illness, eye deviation and impaired consciousness occurred. A head MRI revealed swelling of the right cerebellum and brainstem compression, and she was transferred to our hospital. Her symptoms temporarily improved with steroid pulse therapy and hyperosmolar therapy, but upon completion of hyperosmolar therapy, her impaired consciousness returned, and MRI showed an imminent cerebral hernia. As such, the patient was treated under sedation and mechanical ventilation while considering external decompression. After extubation, she exhibited right-sided ataxia and difficulty maintaining a seated position, but through rehabilitation, she was able to learn to walk with assistance and was discharged on day 41. Although MRI conducted seven months after onset showed atrophy of the right cerebellar hemisphere, ataxia symptoms were extremely mild, and she had no difficulties with her daily activities. In this case, no causative microorganism was found, and although anti-GluR antibody was detected in the cerebrospinal fluid (CSF) specimen, it is unclear how this was involved in the present pathology. Fulminant cerebellitis is a medical emergency in the field of pediatric neurology that should be proactively treated with hyperosmolar therapy and immunosuppressant therapy alongside considering indications for surgical treatment.

  Long term residents of institutions for children with severe motor and intellectual disabilities have poor opportunities for booster effect, and are unlikely to maintain antibodies even after vaccination. Changes in enzyme immunoassay for immunoglobulin G against varicella, mumps, measles, and rubella viruses over three years were studied in our institution in children under the age of 18. In the group that did not receive vaccination during the study period, antibody titers decreased, with a particularly marked 47% decrease in mean antibody titers for varicella. In addition to ensuring that vaccinations are administered as many times as necessary, attention should be paid to subsequent infection control measures.

  ADNP (activity-dependent neuroprotective protein) syndrome is characterized by autism spectrum disorder, intellectual disability, characteristic facial features, and early eruption of primary teeth. A 13-year-old boy presented with clinical symptoms characteristic of ADNP syndrome. Whole-exome analysis indicated that the patient had the NM_001282531.2 (ADNP) : c.2496_2499del gene variant. His intellectual disability was more severe than previously reported. As cases accumulate in the future, it will be increasingly possible to clarify the genotype-phenotype correlation.

  A questionnaire survey was conducted among members of the Japanese Society of Pediatric Neurology regarding adverse events with oral solution of midazolam (buccal MDL) used as prehospital treatment for status epilepticus. A total of 431 respondents were surveyed (12.0% responded). Of 580 patients treated with buccal MDL, 14 (2.4%) had adverse events (respiratory depression, falls and trauma due to dizziness, sedation for more than 1 hour). 7 of the 14 cases were due to respiratory depression. Respiratory depression should be considered as an adverse event when buccal MDL is administered for status epilepticus.