Sachie Nakashima

Cortical Hypometabolism and Delayed Myelination in West Syndrome

Summary: Purpose: We examined the relation between cortical hypometabolism and delayed myelination in patients with West syndrome (WS).

Frontal Lobe Epilepsy of Childhood Onset

Summary: We retrospectively studied long‐term clinical profiles of 38 patients with cryptogenic or symptomatic frontal lobe epilepsy (FLE) of childhood onset. More patients in our patient population had FLE than temporal lobe epilepsy (TLE). Etiology of symptomatic FLE included tuberous sclerosis, cortical dysplasia, intracranial hemorrhage, perinatal asphyxia, and hemiconvulsion‐hemiplegia‐epilepsy syndrome. In 6 patients, magnetic resonance imaging (MN) showed no abnormality, but positron emission tomography (PET) with [18F]fluorodeoxyglucose (FDG) showed glucose hypometabolism in the frontal lobe. The seizures were characterized by postural seizures, focal tonic or clonic seizures, complex gestural automatism, eye deviation, and adversive seizures. Seizure prognosis was poor. Only 13 patients (35%) became seizure‐free for 2 years. Poor prognostic factors for seizures included intelligence deficits, seizure clustering, and aura.

Widespread glucose hypometabolism in patients with hippocampal atrophy: Evaluation with 18F-fluorodeoxyglucose positron emission tomography

Positron emission tomography (PET) with 18 F-fluorodeoxyglucose was performed to measure the interictal local cerebral metabolic rate for glucose (ICMRGlc) in 18 patients observed to have hippocampal atrophy (HA) on magnetic resonance imaging (MRI). In all patients, the MRI showed unilateral atrophy of the hippocampus, and the side showing HA was ipsilateral to the EEG lateralization. PET scans were obtained in a semicoronal plane at an angle of −30 ° to the orbitomeatal line, approximately -60 ° to the long axis of the temporal lobe. The metabolic asymmetry was compared with the frequency of epileptic seizures, duration of illness, and intellectual deficit. The absolute values of ICMRGlc (mg/min/100g) in the hippocampus, extrahippocampal temporal lobe, and frontal lobe were significantly lower on the side ipsilateral to the epileptic focus than on the contralateral side. In patients with frequent seizures, there were greater asymmetry of the ICMRGlc in the extrahippocampal temporal lobe and less in the hippocampus, suggesting that the ipsilateral temporal lobe and contralateral hippocampus were affected by repeated seizures. In conclusion, in patients with presumed unilateral temporal lobe epilepsy and HA, PET studies demonstrated widespread glucose hypometabolism in extrahippocampal areas, and the semicoronal scan was useful in its evaluation.

CLINICO-EPIDEMIOLOGICAL FEATURES OF INFANTILE HYDROCEPHALUS IN JAPAN

In 1988, a nationwide survey was conducted in Japan, in order to examine the prevalence and clinico‐epidemiological features of hydrocephalus that developed before 1 year of age. The study was based on questionnaires that were sent to all hospitals in Japan with more than 200 beds. There were 1435 patients who were eligible for the analysis. They were born before 1987 and diagnosed as having infantile hydrocephalus. Approximately two‐thirds of the patients had primary hydrocephalus. Neural tube defects were the most frequent cause of the primary hydrocephalus and common among full‐term infants. On the other hand, secondary hydrocephalus occurred more often in preterm infants, mainly following intracranial hemorrhage. Intracranial hemorrhage as a cause of hydrocephalus increased over the year, while the incidence of hydrocephalus secondary to intracranial infection decreased during the same period.

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.

Widespread Hypometabolism and Hippocampal Atrophy in Temporal Lobe Epilepsy: Evaluation with 18F‐fluorodeoxyglucose Positron Emission Tomography and Volumetric Magnetic Resonance Imaging.

truck, and erratic extremity myoclonia. The EEG showed persistent 2to 3-Hz high-voltage slow wave (HVS) bursts associated with diffuse spikes and diffuse spike-waves seen predominantly in the bilateral posterior head area. The atonias and myoclonias did not synchronize with the EEG spikes. In the remaining case, 2to 3-Hz rhythmic jerks were frequently observed in the distal part of the extremities and eyelids without definite disturbance of consciousness. These jerks occurred mostly in the right upper extremity but sometimes appeared synchronously in more than two body areas. The analysis of these jerks by using simultaneous videoEEG polygraphic monitoring revealed that brief electromyogram (EMG) discharges evoked by the jerks appeared synchronously with each EEG spike component of persistent central-parietal spikewaves. These persistent central-parietal spikewaves were sometimes interrupted by diffuse spike-wave bursts, when the patient became inactive, resulting in disappearance of the EMG discharges. This peculiar state of rhythmic jerks interrupted by episodes of inactivity occurred daily for times varying from -30 min to a few hours. Thus we diagnosed this state as NSE. In analyzing the relation between interictal EEG findings and epileptic seizures during the clinical course, we found that appearance of diffuse spikewaves on the EEG and NSE were observed almost simultaneously. In the three cases followed up continued until after age 6 years, the EEG findings changed from diffuse spike-waves to multifocal spike-waves between ages 2 and 5 years, when clinical seizures and NSE were suppressed. On the other hand, another case showed symptoms of NSE, such as inactivity and myoclonias, persisting even at the end of the follow-up at age 6 years. These EEGs showed occipital-dominant diffuse spike-waves and HVS regardless of clinical symptoms. Although we examined the electroclinical correlation by using topographic power analysis, we could not find a definite correlation between clinical symptoms and EEG findings. We performed all-night EEGs in three cases. During non-random eye movement (REM) sleep, spike-waves slightly increased without myoclonias. During REM sleep, both seizure discharges and HVS decreased. %SREM showed normal values (20.6-23.5%). As NSE therapy, i.v. diazepam was administered in all cases, and two received i.v. clonazepam. In all cases, both clinical and EEG improvement were observed. However, the EEG effects varied somewhat from complete suppression of both epileptic discharges and HVS to suppression of epileptic discharges without decrease of HVS. High-dose valproate therapy, per 0s clobazam. and per 0s clonazepam also were effective. It was characteristic that all cases with Angelman syndrome showed peculiar NSE from late infancy to early childhood.

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.