Toshiko Kubota

Absent Cyclicity on aEEG within the First 24 h is Associated with Brain Damage in Preterm Infants

OBJECTIVE Our aim was to clarify the relationship between amplitude-integrated electroencephalographic (aEEG) findings before 24 h of age in preterm infants and neurodevelopmental outcome. DESIGN 12 infants born between 27 and 32 weeks of gestation were eligible. The recordings of aEEG and conventional EEG were started within 12 h after birth. The background aEEG findings were evaluated and classified. Additionally, we evaluated the absence or presence of changes on the lower border of the aEEG. RESULTS All infants had discontinuous normal voltage background on aEEG, corresponding to decreased or normal continuity on conventional EEG. Cyclicity on aEEG was seen in 8 of 12 infants within 24 h of age, and all of these infants had favourable outcomes. Cyclicity on aEEG was not recognized in 4 infants. 3 of the 4 infants with absent cyclicity had abnormal neurodevelopmental outcomes at 12 months. One of these infants had intraventricular haemorrhage (grade 2) with delayed development, and 2 had cystic periventricular leukomalacia followed by spastic diplegia. CONCLUSION Absent cyclicity on aEEG within 24 h of age was associated with poor outcome in preterm infants.

Differences of Clinical Manifestations According to the Patterns of Brain Lesions in Acute Encephalopathy with Reduced Diffusion in the Bilateral Hemispheres

BACKGROUND AND PURPOSE: The precise clinical characteristics of acute encephalopathy with bilateral reduced diffusion are not fully understood. We compared clinical, laboratory, and neuroimaging findings according to the patterns of brain lesions among children with reduced diffusion in the bilateral hemispheres. MATERIALS AND METHODS: Nine patients were analyzed. The patterns of brain lesions were divided into diffuse lesions and central-sparing lesions. Diffuse lesions were defined as reduced diffusion in the whole cortex and/or subcortical white matter. Central-sparing lesions were defined as the lack of reduced diffusion in the areas around the bilateral Sylvian fissures. Clinical, laboratory, and neuroimaging findings were compared between groups. RESULTS: Five patients showed diffuse lesions and 4 showed central-sparing lesions. Coma was significantly more common in patients with diffuse lesions, whereas a biphasic clinical course was more common in those with central-sparing lesions. Outcome was worse in patients with diffuse lesions. Maximal aspartate aminotransferase, alanine aminotransferase, and kinase levels were also significantly higher in patients with diffuse lesions. In 2 patients with diffuse lesions, diffusion-weighted images during the acute phase revealed reduced diffusion in the bilateral frontal and occipital areas, followed by diffuse lesions. No patient with central-sparing lesions showed MR imaging abnormalities during the acute phase. CONCLUSIONS: Clinical manifestations in patients with diffuse lesions were severe, whereas those in patients with central-sparing lesions were relatively mild.

Epileptic Spasms Preceded by Partial Seizures with a Close Temporal Association

Summary: Purpose: To investigate the distinctive features of patients with West syndrome who had partial seizures followed by epileptic spasms (PS‐ES).

Evolutional Changes and Outcome of West Syndrome: Correlation with Magnetic Resonance Imaging Findings

Summary: The prognosis and evolutional changes of 77 patients with West syndrome (WS) were studied after patients were classified into four groups on the basis of their magnetic resonance imaging (MRI) findings: anomaly, perinatal injury, normal, and the other groups. The average age at onset of spasms was earliest in the patients with anomalies and latest in patients with normal MRI findings. Patients with normal MRI findings had the shortest duration of spasms, and patients with anomalies had the longest duration of spasms. Antecedent seizures were observed in 6 patients (3 patients with anomalies, 1 patient with normal MRI findings, and 2 patients with other abnormalities). Thirty‐five patients had subsequent seizures. Patients with anomalies often had partial seizures and patients with perinatal injuries often had generalized seizures. Seizures were infrequent in patients with normal MRI findings. Developmental outcome was best in the patients with normal MRI findings and worst in patients with perinatal injuries. Various types of epileptic syndromes occurred subsequent to WS in patients with anomalies, although nonspecific symptomatic generalized epilepsy was common in patients with perinatal injuries. These results suggest that seizure prognosis, evolutional changes in seizures, and developmental outcome are different among the types of brain lesions.

Diffusion-weighted magnetic resonance imaging in infants with periventricular leukomalacia.

OBJECTIVE We examined diffusion-weighted magnetic resonance imaging (DWI) findings in periventricular leukomalacia (PVL) and then evaluated the relationship between the mean apparent diffusion coefficient (ADC) values and the region and severity of PVL. METHODS Between April 2006 and July 2007, 98 infants at gestational ages between 27 and 33 weeks were enrolled in the study. DWI was performed during the first week of life when electroencephalography indicated PVL. The mean ADC values were evaluated using regions of interest drawn manually in the periventricular white matter, posterior limbs of internal capsules (PLICs), and various regions of the brain. RESULTS DWI was performed in three of four infants with PVL indicated on electroencephalography. All had decreased diffusivity in the anterior to posterior white matter despite a predominance in the posterior white matter. DWI abnormalities were also observed in the corpus callosum and PLICs and were more broadly distributed in the brain than those detected by later conventional MRI. In the PLICs, the changes in the ADC values were correlated with the severity of the PVL. CONCLUSION The DWI findings provided additional information regarding PVL. Among the findings, the association of the presence of decreased diffusivity in the corticospinal tract with later motor impairment was the most interesting.

Electroencephalogram and flash visual evoked potentials for detecting periventricular leukomalacia.

OBJECTIVE The aim of this study was to evaluate the usefulness of a combination of electroencephalogram (EEG) and flash visual evoked potentials (FVEPs) for predicting periventricular leukomalacia (PVL) in the early days of life. STUDY DESIGN Eighty-six of 108 infants admitted to Anjo Kosei Hospital during 1998 through 2000 were enrolled in this study. All subjects underwent EEG and FVEP during the early neonatal period and were followed-up until 18 months of corrected age. EEG was performed once within 72 h after birth, every 1-2 weeks during the first month and every 2-4 weeks during the second month. FVEPs were recorded at least twice, at the first and the second week of life. RESULTS Of the 86 infants, 13 were diagnosed as having PVL. Among them, EEG abnormalities were observed in 11 infants and FVEP abnormalities in 10. The sensitivity and specificity of EEG were 0.85 and 0.95, respectively. The sensitivity and specificity of FVEPs were 0.77 and 0.96, respectively. All except one (92%) infant with PVL had EEG and/or FVEP abnormalities. CONCLUSIONS The combination of EEG and FVEPs can increase the sensitivity, but reduces the specificity to identify infants with PVL. The combination can makes up for the shortcomings of each method.

Successful polymerase chain reaction-based diagnosis of fungal meningitis in a patient with chronic granulomatous disease.

Abstract Meningitis is not a common complication of chronic granulomatous disease (CGD). Here, we present details of a 3‐year‐old boy with X‐linked CGD, who suffered from fungal meningitis. While 19 samplings using conventional cerebrospinal fluid (CSF) cultures failed to detect any organisms, fungal DNA was identified in the CSF by a new polymerase chain reaction (PCR)‐based method. The patient recovered without any sequelae after treatment with a combination of antifungal agents, interferon‐φ and granulocyte infusions. This case report demonstrates that fungal meningitis must be included in the differential diagnosis of infections in CGD patients and that the PCR‐based detection of fungal DNA is a powerful tool for diagnosis.

Correlations Between the Distribution of Cortical Hypometabolism and Clinical Manifestations in Patients with West Syndrome

Purpose: To examine the relation between the distribution of cortical hypometabolism and clinical manifestations in patients with West syndrome (WS).

A Comparison Between Seizure Manifestations and Outcome in Frontal Lobe Epilepsy of Childhood Onset

Purpose: The seizure manifestations of frontal lobe epilepsy (FLE) vary considerably, and the correlation between the ictal symptoms and seizure prognosis has not yet been fully elucidated. We retrospectively compared seizure manifestations with outcomes of the seizures in FLE of childhood onset.