Satoshi Yoshinari

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 (35.8%). Seizures ceased within 5 minutes of lidocaine administration in all 19 patients who were responsive to the drug. Regarding aetiology of status epilepticus and types of seizures, there was no statistical difference in effectiveness. Mild decrease of oxygen saturation, monitored by pulse oximetry, was observed in one patient, which improved by oxygenation using a mask. Lidocaine is a useful anticonvulsive agent; however, the response rate to lidocaine appears to be quite low, as less than half of the seizures were effectively controlled by lidocaine. Favourable properties of the drug include prompt responses, less alteration of consciousness, and fewer adverse effects, including less respiratory depression.

Regional cerebral blood flow and developmental outcome in cryptogenic west syndrome

Summary:  Purpose: To elucidate the relation between alterations of regional cerebral blood flow (rCBF) by adrenocorticotropic hormone (ACTH) therapy and developmental outcomes of cryptogenic West syndrome.

Interictal cerebral blood flow abnormality in cryptogenic West syndrome

Purpose:  To elucidate the abnormality of interictal regional cerebral blood flow (rCBF) of West syndrome at the onset.

Aggressive large cell medulloblastoma extending to the extracranial region in brain-dead state.

IntroductionThe authors describe the case of a 29-month-old boy who presented with acute non-communicating hydrocephalus caused by a small tumor in the fourth ventricle. He became brain-dead immediately and remained stable in that condition.Materials and methodsSix months later, despite being in a brain-dead state, a rapid direct tumor extension from the intracranial to extracranial region was observed, and chemoradiotherapy was performed following tumor biopsy. The histopathological diagnosis was large cell medulloblastoma. Although treatment was initially effective, the tumor again aggressively invaded the cervical muscles via the spinal canal. Comparative genomic hybridization (metaphase) analysis revealed a pattern of aberrations predictive of a poor prognosis (+1q, ?17p, +17q, and probable amplification of c-myc gene), and he eventually died 11 months after onset.ResultsDirect invasion of medulloblastoma from the intracranial to extracranial region is extremely rare, and, to our knowledge, this is the first report of medulloblastoma exhibiting rapid extension to the extracranial region in brain-dead state.ConclusionsFor patients with medulloblastomas, careful observation is needed even in brain-dead state. The etiology of this rare condition as well as the genetic characteristics responsible for aggressive tumor behavior are discussed.

Nonthalamic Generalization of Ictal Spikes in Atypical Absence Seizures

Reported here is the case of a boy who had a thalamic hemorrhage as a neonate and developed symptomatic focal epilepsy at 3 years of age. At the onset of focal epilepsy, the interictal spikes were localized in the occipital regions; over time, they gradually expanded, and atypical absences developed at the age of 6 years. When the patient was hospitalized at the age of 7 years 11 months, the spatiotemporal distribution of the synchronous spikes was assessed for each generalized spike-and-wave discharge observed on ictal electroencephalography. The occipital spikes were always the first to appear, and most spikes had posterior-to-anterior distribution. Occasionally, the frontopolar spikes appeared before the frontal spikes. These results indicate that the generalized spikes observed during atypical absences were formed by rapid generalization of the focally generated spikes from the occipital region through the cortex and the long association fibers, but not through the thalamus.