Safety of zonisamide therapy: prospective follow-up survey

AbstractZonisamide safety was evaluated based on a postmarketing surveillance study of patients treated for 1–3 years. Nine hundred twenty-eight children and 584 adults (ages 1 month to 79 years), including 372 newly-diagnosed patients, received zonisamide for partial and generalized epilepsies. Of the intractable patients, 1008 received zonisamide in combination with other antiepileptic drugs (AED), and 52 successfully transitioned to zonisamide monotherapy. A total of 1089 adverse events occurred in 476 (31.5%) of 1512 patients. Incidence of adverse effects was significantly lower among patients receiving zonisamide monotherapy than in those receiving polytherapy: 21% (18.9% of children, 29.4% of adults) versus 35.6% (30.4% of children, 41.7% of adults), respectively. The total incidence of adverse effects was lower for children (26.2%) than for adults (39.9%). Most common adverse events included mental/psychiatric symptoms (19.4%), gastrointestinal symptoms (8.7%), and neurological symptoms (5.8%). Effects that seemed unique to zonisamide were impairment of mental function, motivation or volition, and hypohidrosis. Urinary calculi were detected in only two patients (0.13%). Teratogenicity was evaluated in six patients. Two patients on zonisamide monotherapy and three on polytherapy delivered normal children. One of four patients on polytherapy conceived a fetus with a skull defect with cerebral and cerebellar dysgenesis

Vergleichungsstudien über die verschiedenen Trockenmittel

Um praktisch die Wirkung der Entfeuchtung bei geschlossenem Raum zu studieren, stellten wir in das dicht geschlossene Laboratoriumzimmer einen mit Papier überzogenen Glaskasten (1cm) ein, mit dem man eine bestimmte Temperatur und Feuchtigkeit erzeugen kann. Die relative Feuchtigkeit im Versuchskasten wird durch Einführung von Wasserdampf auf über 90% gesteigert, dann wird eine bestimmte Menge von verschiedenen Trockenmitteln (Calcium chloratum purum für Analyse von Isizu, Calcium chloratum siccum für Trocknung, Calcium chloratum siccum neutrale von Merck, I. D. Trockenmittel, Adsole, Schwefelsäure, und Wolle) in den Kasten eingeführt. Die Wirkung der Trockenmittel wird zeitlich durch verschiedene Psychrometer (Augustsche, Assmannsche, Fuessche, Lambrechtsche Psychrometer) untersucht. Dabei werden die Katawerte mit einem Wett-katathermometer bestimmt. Da die relative Feuchtigkeit von der Veränderung der Temperatur abhängt, wurden alle Untersuchungen immer in einer bestimmten Temperatur ausgeführt. Diese Versuche wurden im Sommer (bei 30 C) und im Winter (bei 10 C) angestellt, weil die Wirkung der Trockenmittel durch die Temperatur mehr oder minder beeinträchtigt wird. 1) Bei hoher Temperatur im Sommer, d. h. bei 30 C, zeigt sich Calcium chloratum in bezug auf Lufttrockung am stärksten, dann folgen I. D. Trockenmittel, Adsole, Schwefelsäure und Wolle. 2) Bei niedriger Temperatur im Winter, d. h. bei 10 C, abfeuchtet Calcium chloratum am stärksten, es folgend I. D. Trockenmittel und Adsole. Unter Calcium chloratum wirkt als Trockenmittel das Calcium chloratum purum für Analyse am stärksten, diesem folgt Calcium chloratum siccum neutrale von Merck, Calcium chloratum siccum steht hinter diesen ziemlich weit zurück. 3) Es ist bemerkenswert, dass die Absorptionskraft für die Luftfeuchtigkeit hauptsächlich von der Flächengrösse der Absorptionsmittel abhängig ist. Wenn man also geklümpftes Calcium chloratum siccum fein zerbricht, so steigt seine Absorptionskraft auf die des Calcium chloratum purum für Analyse. 4) In der gleichen Weise kann man durch verteilte Anwendung des Trockenmittels viel bessere Resultate erzielen als bei einmaligem Gebrauch desselben. 5) Die Trockenmittel zeigen ihre Absorptionskraft bei hoher Temperatur im Sommer deutlich stärker als bei niedriger Temperatur im Winter

Proton Magnetic Resonance Spectroscopy in Patients with Migration Disorders

Proton Magnetic Resonance Spectroscopy (1H-MRS) can be used to detect cerebral metabolites including N-acetylaspartate (NAA)，creatine (Cr) and choline (Ch). Hence，clinical applications of this method for neuropediatric diseases can be expected. However，regarding neuronal migration disorders，there have been only a few reported studies. We therefore examined the lH-MRS in six patients with migration disorders，ages ranged from 8months to 28years 10months with a mean of 10years 10months. Investigation was performed using Magnetom H15 (Siemens) with a repetition time of 1500 msec and an echo time of 270msec. The ratio of NAA/Cr，Ch/Cr were examined. The volume of interest with the size of 2 × 2 × 2 ～ 3 × 3 × 5cm3 was chosen in the area including lesions，and a contralateral area without lesions was also investigated. Results were as follows. 1) The ratio of NAA/Cr was low in the area with lesions in all 6cases; 1.41，1.95，2.27 and 1.71 in cases with heterotopic gray matter，0.99 in one case with polymicrogyria，an d 1.30 in one case with hemimegalencephaly，contrasted with a contralataral area without lesions: 1.89， 2.89，2.87，2.55，3.26，2.03，respectively. 2) The ratio of Ch/Cr showed no consistent difference between the area including lesions and contralataral area without lesions. Our findings of a decreased NAA/Cr ratio can be inferred to reflect the decreased numbers of neuronal cell population，or reduced metabolism in the lesions

Cerebral blood flow velocity in handicapped children

Using a transcranial Doppler blood flowmeter, the blood flow velocity (BFV) ratio of the middle cerebral artery (MCA) to the basilar artery (BA) was investigated in 12 patients with severe motor and intellectual disability syndrome. The BFV of the MCA was also investigated in 58 handicapped children, classified according to the severity of their motor and intellectual disability. The ratio of the MCA to the BA was lower by 2 SD from the mean of our previously reported standard value in 8 out of the 12 cases with severe motor and intellectual disability syndrome, suggesting a more profound decrease in the level of brain activity in the MCA area than that of the BA area. The BFV of the MCA mainly decreased in cases belonging to the category of the most severe motor disability (bed-ridden). Hence, it is suggested that motor disability is the main factor related to the decrease in the BFV of the MCA.</p

Anesthetic experience of a patient with Ohtahara syndrome -A case report-

Ohtahara syndrome (OS) is a rare epileptic encephalopathy that is characterized by an abnormal electroencephalogram (EEG) and intractable seizures in the neonatal and early infantile period. The patient of this reported case was delivered normally at 39 weeks of gestation without any complication. One week after birth, seizures that were refractory to anticonvulsants started with repetitive clustered tonic spasms. The child had no abnormal findings on the initial laboratory investigations. But he was diagnosed with OS according to the frequent tonic spasms, an abnormal EEG pattern of suppression-burst and magnetic resonance imaging of cortical dysplasia. He was planned to undergo an operation for brain lesion. This report describes our experience with the general anesthetic management when we performed craniotomy and right hemispherotomy for a patient with OS

Farber's disease (disseminated lipogranulomatosis): the first case reported in Japan.

We report the first case in Japan, i.e., the first case among oriental subject of Farber's disease. This is a rare disorder of lipid metabolism in infancy subsequent to a genetically-determined defect in ceramide degradation. Main features are characterized clinically by hoarseness, joint swelling, subcutaneous nodules and retarded psychomotor development. Lipid analysis and pathological investigation on the material obtained from a subcutaneous nodule confirmed clearly the presence of ceramide and intracytoplasmic inclusion bodies characteristic for Farber's disease. In this case, we experienced also corneal opacity and striking abnormalities in electroencephalogram, which have apparently not been noticed in the 17 cases hitherto reported.</p