Incidence and risk factors of acute encephalopathy with biphasic seizures in febrile status epilepticus.

Objective: To clarify the incidence and risk factors of acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) in pediatric patients with febrile status epilepticus (FSE).

Methods: We retrospectively surveyed patients with FSE (≥20 min and ≥40 min) who were younger than 6 years by mailing a questionnaire to 1123 hospitals in Japan. The survey period was 2 years.

MeCP2_e2 partially compensates for lack of MeCP2_e1: A male case of Rett syndrome.

Background: Rett syndrome (RTT) is a neurodevelopmental disorder that predominantly affects girls. Its causative gene is the X-linked MECP2 encoding the methyl-CpG-binding protein 2 (MeCP2). The gene comprises four exons and generates two isoforms, namely MECP2_e1 and MECP2_e2.

Regional volumetric assessment of the brain in moderately preterm infants (30-35 gestational weeks) scanned at term-equivalent age on magnetic resonance imaging.

Background: Early volume analyses of the infantile brain may help predict neurodevelopmental outcome. However, brain volumes are not well understood in moderately preterm infants at term-equivalent age (TEA).

Aim: This study retrospectively investigated the relationship between regional brain volumes and infant gestational age (GA) at birth in moderately preterm infants (30-35weeks' GA) on magnetic resonance imaging (MRI) at TEA.

[A child case of surfer's myelopathy].

We report a case of so-called "surfer's myelopathy" involving a 15-year-old girl. Preceding episodes of vaccination and infection were absent. She experienced back pain during a surfing class for beginners, followed by weakness in both legs, which progressed to paraperesis.

[A case of rapidly progressive glomerulonephritis with anti-glomerular basement membrane antibody in the course of MPO-ANCA positive interstitial pneumonia].

A 78-year-old man developed rapidly progressive glomerulonephritis (RPGN) in the course of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-positive UIP that had been found four years previously. When UIP was diagnosed, the MPO-ANCA titer was low and urine was negative for proteinuria and hematuria. On admission, his serum creatinine increased to 16.

A prognostic value of neuron-specific enolase in cerebrospinal fluid of acute cerebellar ataxia.

We examined 16 patients with acute cerebellar ataxia (ACA) to determine whether clinical manifestations or laboratory findings could predict the prognosis of ACA. We divided the patients into two groups: a benign group of patients whose cerebellar symptoms completely disappeared within 21 days (9 patients), and a prolonged group of patients whose cerebellar symptoms persisted for more than 22 days (7 patients). The two groups were compared on the basis of demographic and clinical characteristics, and laboratory variables.

Consistently high levels of exhaled nitric oxide in children with asthma.

Background: Exhaled nitric oxide (eNO) levels in children are unstable because they are regulated by many potent factors. The purpose of the current study was to evaluate the reliability of eNO levels between a long interval and other lung functions in normal and asthmatic children.

Methods: Eighty-three elementary school children (aged 11-12 years; male : female, 39 : 44) participated in this study.

[Recurrent idiopathic cerebral infarction in a 5-year-old boy, with emphasis on the importance of platelet aggregation analysis for appropriate selection of anti-platelet drugs].

We present a 5-year-old boy with recurrent idiopathic cerebral infarction in which analysis of platelet hyperaggregability was useful in choosing appropriate anti-platelet drugs. The patient presented with gait disturbance at the age of 5 years and 1 month. Brain MRI demonstrated multiple infarctions in the right thalamus and left cerebellum.

[Feasibility of helical CT in the diagnosis and management of tracheo-innominate artery fistula and tracheo-innominate artery allovascularity after tracheostomy in patients with severe neurological disorders].

Tracheo-innominate artery fistula (TIF) is one of the most important complications of tracheostomy in that it is often life threatening. We experienced two cases with TIF and tracheo-innominate artery allovascularity, in which three-dimensional helical CT (3DCT) was useful in making the diagnosis of TIF and tracheo-innominate artery allovascularity. In one case, acute hemorrhage occurred during the procedure of routine tube exchange, and hemostasis was achieved by inflating the cuff of the tracheostomy tube.

Intravenous lidocaine for status epilepticus during childhood.

The clinical efficacy of lidocaine for convulsive status epilepticus in 53 convulsive episodes was examined in 37 children (17 males, 20 females). Mean age of patients receiving lidocaine was 3 years 7 months (SD 3y 5mo). Lidocaine administration achieved control of status epilepticus in 19 of 53 convulsive episodes (35.

[Choice and administration sequence of antiepileptic agents for status epilepticus and frequent seizures in children].

We investigated the sequence of the administration, the efficacy and the safety of antiepileptic drugs (AED) given intravenously for the treatment of status epilepticus and frequent seizures in children. Our institute has a recommended sequence of AED administration for treatment of status epilepticus: the first-line agent is diazepam (0.3 - 0.

Autoantibodies and cell-mediated autoimmunity to NMDA-type GluRepsilon2 in patients with Rasmussen's encephalitis and chronic progressive epilepsia partialis continua.

Purpose: To evaluate antibody-mediated and cytotoxic T cell-mediated pathogenicity that has been implicated as the autoimmune pathophysiological mechanism in Rasmussen's encephalitis.

Methods: We examined autoantibodies against the N-methyl-d-aspartate glutamate receptor (NMDA-type GluR) epsilon2 subunit and its epitopes in serum and CSF samples from 20 patients [five histologically proven (definitive) Rasmussen's encephalitis with epilepsia partialis continua (EPC), four definitive Rasmussen's encephalitis without EPC, and 11 clinical Rasmussen's encephalitis with EPC]. We examined 3H-thymidine uptake into lymphocytes after stimulation by GluRs.

[Bell's palsy in children: analysis of clinical findings and course].

To evaluate treatment of Bell's palsy (acute idiopathic peripheral facial nerve paralysis) of children, the authors analyzed 38 cases (18 females, 20 males) of Bell's palsy in children aged below 16 years old. The mean age of all cases was 6.8 +/- 6.

[Efficacy of lidocaine on seizures by intravenous and intravenous-drip infusion].

We retrospectively studied the efficacy of intravenous and intravenous-drip infusion administration of lidocaine on seizures treated in Saitama Children's Medical Center during the period of 1997-2002. Thirty eight seizures of 29 children (12 boys and 17 girls, average age 41.4 months) were treated with lidocaine.

[A case of chronic cerebellitis with anti-glutamate receptor delta 2 antibody].

A 1-year-8-month-old patient developed cerebellar ataxia following a prodromal infection. Despite initial diagnosis of acute cerebellar ataxia, his symptoms lasted for more than 30 days. High-dose intravenous immunoglobulin and steroid pulse therapy failed to ameliorate his cerebellar symptoms, which fluctuated in association with infections.

Long-term follow-up study of West syndrome: Differences of outcome among symptomatic etiologies.

Objectives: To evaluate the outcome of West syndrome and to elucidate the differences in the outcome related to the timing of brain injury.

Study Design: Medical records of 60 patients who were followed regularly for more than seven years were reviewed. The following clinical features were assessed: onset, seizure evolution, electroencephalography and intelligence.

[High-dose intravenous immunoglobulin therapy in a child case of Bickerstaff's brainstem encephalitis].

We report an 11-year-old boy with Bickerstaff's brainstem encephalitis (BBE). He had gait disturbance, disturbed consciousness, and diplopia after upper respiratory tract infection. On admission, he showed multiple cranial nerve palsy, muscle weakness of arms, cerebeller ataxia and generalized areflexia.

[Midazolam treatment for status epilepticus of children].

We investigated the efficacy and safety of midazolam given intravenously for the treatment of status epilepticus in children. Patients received one to three bolus injections of midazolam (0.15 to 0.

[MRI findings and effectiveness of cyproheptadine in two patients with ophthalmoplegic migraine].

We reported the MRI findings and clinical course of two patients with ophthalmoplegic migraine. Both patients presented with unilateral oculomotor nerve palsy. Contrast enhanced MR imaging revealed unilateral enhancement and thickening of the oculomotor nerve in one patient.

[Paroxysmal tonic upgaze of childhood].

We described a child who developed paroxysmal abnormal eye movement. At the age of 12 months, she had a high fever and a febrile seizure. On the next day she showed tonic upward deviation of the eyes for 1 to 2 seconds, and downward saccades on attempted downward gaze.