Polysomnogram real-time processing system: Clinical use in childhood epilepsy

were retrospectively reviewed at Children's Hospital from January, 1981 to December, 1990. There were 38 boys and 16 girls with the male to female ratio of 2.2:1. The ages ranged from 3 months to 14 years (mean: 7.15 --- 3.48 years). The common symptoms and signs were as follows: nausea and vomiting (72.2%), headache (75.92%), fever (68.51%), and changing of consciousness (57.41%). The common underlying disease was congenital cyanotic heart disease especially tetralogy of Fallot. The diagnosis was made by CT in 46 patients and by cerebral angiography in 7 patients. The sites of brain abscesses were parietal (37%) and frontal (25.9%). Multiple abscesses were present in 28.3%. Microbiologic study of the pus taken from the brain abscess showed anarobe peptostreptococcus in 20%. The overall mortality rate was 24.07%.

296. FACTORS ASSOCIATED WITH PRECOCIOUS PUBERTY IN GIRLS WITH SPINA BIFIDA CYSTICA G. Ahlsten, M. Dahl, L. Proos, J. Gustafsson, and T. Tuvemo, Uppsala, Sweden The risk of developing precocious puberty (PP) has been reported to be increased in children with spina bifida cystica. In order to evaluate this further and to study possible factors associated with PP, the medical records of all girls with spina bifida cystica born since 1970 (n = 64) and coming for check-ups at our unit were reviewed. PP was defined as breast development or pubic hair Tanner stage 2 before the age of 9.2 years. In 28 of 64 patients data were sufficient for such evaluation. Eighteen girls had PP and 10 girls normal data for puberty. The medical histories of the 2 groups did not differ regarding length of pregnancy or birth weight. Girls with PP had a significantly higher frequency of increased intracranial pressure during the perinatal period and were treated with intraventricular shunts more often than those with normal data for puberty (P < .01 and P < .05, respectively). The group of girls with PP, in general, seems to have more severe problems. In conclusion, PP is frequent in girls with spina bifida cystica and seems to be associated with increased intracranial pressure in early life.

297. ENDOTRACHEAL SUCTIONING OF NEONATES VENTILATED WITH HIGH-FREQUENCY OSCILLATORY VENTILATION: NEAR INFRARED SPECTROSCOPY STUDY G. Bernert, C. Kohlhauser, M. Hermon, R. Seidl, and A. Pollak, Vienna, Austria Endotracheal suctioning is associated with changes in cerebral circulation as shown in studies using Doppler ultrasound technique, noninvasive intracranial pressure monitoring, and near infrared spectroscopy (NIRS). The effect of high-frequency oscillatory ventilation (HFOV) on cerebral hemodynamics has not been studied until now. In an open prospective design, we studied 13 suctioning periods in 10 infants on HFOV (Sensormedics 3100). Gestational ages ranged 24-34 weeks (mean: 29 wks) and birth weights 520-2920 gm (mean: 1,337 gm). The measurements were done on days 1-28 (mean: 6.2). NIRS (NIRO 500, Hamamatsu KK) was used to monitor changes in cerebral concentration of oxygenated (HbO2) and reduced hemoglobin

(Hb) and cytochrome aa3 (cytaa3). Heart rate (Hrt), mean arterial blood pressure (MAP), arterial oxygen saturation (SatO2), and carbon dioxide tension (TcPco2) were monitored continuously. Behavioral state (BS) observations were made by the investigator and documented as well. In all measurements, a significant decrease in HbO 2 and an increase in Hb were seen during endotracheal suctioning. The magnitude of these changes was quantiffed by comparing them to baseline values, established before the onset of suctioning. NIRS is useful for evaluating the effects of new ventilatory techniques, such as HFOV, on cerebral circulation.

298. E F F E C T OF B E H A V I O R A L S T A T E S ON CHANGES OF CEREBRAL BLOOD VOLUME AND OXYGENATION DURING ENDOTRACHEAL SUCTIONING: NEAR INFRARED SPECTROSCOPY STUDY IN VENTILATED PRETERM NEONATES G. Bernert, K.V. Siebenthal, R. Seidl, Ch. Vanhole, H. Devlieger, and P. Casaer, Vienna, Austria and Leuven, Belgium Controversial results have been reported concerning changes of oxygenated hemoglobin (HbO2), reduced hemoglobin (Hb), and total hemoglobin (Hbtot), during endotracheal suctioning. To explain these differences, some authors proposed a possible relationship to differences in the activity level of the infants. In an open prospective design, we studied 20 suctioning periods in 13 infants who had been admitted to the neonatal intensive care unit of the Department of Pediatrics at the University Hospital of Leuven. Gestational ages ranged 26-32 weeks (mean: 29 wks, S.D. 2) and birth weights 694-1,750 gm (mean: 1,329 gm, S.D. 277). For NIRS measurements, a commercial system (NIR 1000, Hamamatsu KK) was used. Heart rate (Hrt), mean arterial blood pressure (MAP), arterial oxygen saturation (SatO2), and carbon dioxide tension (TcPco2) were monitored continuously. Behavioral state (BS) observations were made and documented as well. Statistical analyses (Mann-Whitney-U) showed that in patients who were active, with crying periods during suctioning, changes of Hb, HbO 2, and SatO 2 were significantly greater than in patients with predominant behavioral states 1, 2, and 3 (P < .05). These results underline the potential risk of high airway pressure, coughing, and fighting the ventilator. NIRS proved to be a valuable tool in re-evaluating the possible harmful effects of different suctioning techniques.

299. POLYSOMNOGRAM REAL-TIME PROCESSING SYSTEM: CLINICAL USE IN CHILDHOOD EPILEPSY Komei Kumagai, Susumu Ohi, Mana Kurihara, Hideki Horita, and Kihei Maekawa, Atsugi, Kanagawa and Tokyo, Japan In childhood epilepsy, especially epileptic syndrome in childhood, it is recognized that EEG usually shows a marked contrast between the waking record, which typically displays focal paroxysms, and sleep tracings which demonstrate almost continuous diffuse, slow spike-and-wave activity. In the EEG investigation of epileptic syndromes, there has been a need for a simple-to-use analytic system giving consistent results. An all-night real-time polysomnogram (PSG) processing system equipped with a per-

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sonal computer was developed. This system recognizes different sleep stages and detects epileptogenic spikes on EEG. The accuracy of this system was tested by comparing the computerized scores of 11 PSG records with those of 5 independent experts; a high-degree of concordance was obtained. The PSGs of 36 children with epileptic syndromes were examined by this method. The investigation was repeated yearly. The longest follow-up was in a patient from age 6 to 15 years. To evaluate the new system, the results of analysis of data collected by an automated system were compared with those obtained with manually collected data. It has been found to furnish relatively satisfactory results concerning its accuracy, and possess high practical value for follow-up of seizure activity in childhood epilepsy.

300. EPILEPSIA PARTIALIS CONTINUA (RASMUSSEN SYNDROME) RESPONDING TO PROLONGED IV GAMMAGLOBULIN Sara Kivity, Avinoam Rachmel, Liora Kornreich, and Nathan Gadoth, Petah Tikva, Israel A 23-month-old right-handed boy presented with brief, simple, partial seizures 5-9/day, characterized by left-sided tonic convulsions of the face and upper limb, often with obvious somatosensory onset of "burning" pain in his left arm. Despite aggressive antiepileptic drug polytherapy, corticosteroids, and acyclovir, seizures increased over the next 3 months, up to 60/day, evolving to bilateral tonic-clonic convulsions of the face and upper limbs with almost continuous fine myoclonia of the left hand, denoting epilepsia partialis continua (EPC). Hemiparesis of the left arm evolved. Initial EEG revealed no epileptic activity but within 6 weeks bilateral centroparietal spike-and-wave discharges appeared, predominantly on the right. There was no serologic evidence of viral infection. Cranial CT revealed a welldefined hyperdense right parietal lesion, 3 × 2 cm, involving both gyri and centrum semiovale. Cranial MRI was normal. At this stage, intravenous immunoglobulin was given at a dose of 2 grn/kg over 2 consecutive days; then the dose changed to 200 mg/kg every 2 weeks for 3 months, then once monthly for 4 months, and finally at 6 week intervals for a total of 1 year. Seizures ceased 3 weeks after beginning intravenous immunoglobulin. Hemiparesis and S/W discharges resolved.

301. MULTIPLE SCLEROSIS: ONSET BEFORE AGE 10 YEARS Dusan N. Vranjesevic, Aleksandra Djukic, Vedrana M. Milic-Rasic, Nebojsa S. Jovic, and Vesna M. Brankovic, Belgrade, Yugoslavia Fifteen patients (F:M ratio 1.16:1) with definite multiple sclerosis (MS) [Poser et al., 1983] with the onset of the disease before the age of 10 years (range: 3-10 yrs) were analyzed. Clinical parameters (i.e., onset, duration clinical form and course, EDSS), electrophysiologic (VEP, SSEP, AEP, EEG), immunologic investigations (3 parameters as signs of It IgG synthesis: IgG index, IgG synthesis/day, OB) and imaging studies (CT, NMR) were evaluated. All parameters were compared with the groups of older patients (onset after age 10 and adult patients). Some clinical expressions specific to the child with MS were found. Progression index of the disease (EDSS/duration of the

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disease) was reported to be lower in patients with earlier onset. No significant differences in immunologic parameters between groups of children and adult MS patients were found.

302. LIMB DEFECTS WITH OR WITHOUT MOBIUS SEQUENCE AND MISOPROSTOL USE IN FIRST TRIMESTER OF PREGNANCY Maria J. Marques-Dias, Jos6 A. Paz, Jos6 L. Gherpelli, Chong A. Kim, Clea R. Leone, Fernando R. Vargas, Maria J. Maldonado, Gfzis Ramos, and Claudette H. Gonzalez, Silo Paulo, Rio de Janeiro, Campo Grande, Belem, Brazil Since 1991 congenital abnormalities have been reported in 12 Brazilian children in association with maternal misuse of misoprostol during pregnancy [1,2]. In the last 14 months, 19 new patients have been studied. Misoprostol, a synthetic analog of prostaglandin E~ has been largely used in Brazil as an abortifacient since 1988. This new series of patients, whose mothers used 200-1,800 Ixg of this drug between 30th-60th days of pregnancy, consisted of 14 boys and 5 girls. According to the anomalies detected, the patients were grouped as follows: limb deficiency (LD) + Mfbius sequence (MS), 13 patients; MS, 3 patients; LD + hydrocephaly, 2 patients; and LD + MS + hydrocephaly, 1 patient. Nine patients presented with several degrees of neuropsychomotor delay, 7 patients were normal, and 3 did not return for follow-up. We believe that misoprostol is the causal agent in the pathogenesis of these anomalies that must result from a vascular disruptive mechanism around the 6th week of embryologic development. References: [1] Fonseca W, et al. Misoprostol and congenital malformations. Lancet 1991 ;338:56. [2] Gonzalez CH, et al. Limb deficiency with or without Mfbius sequence in seven Brazilian children associated with misoprostol use in the first trimester of pregnancy. Am J Med Genet 1993; 47:59-64.

303. OSTEO-CHONDRO-MUSCULAR DYSTROPHY (SCHWARTZ-JAMPEL SYNDROME) Alice H. Masuko, Maria Isabel C. Vergani, Pedro Tannous, Mauro Muszkat, and Rosana S.C. Alves, Sho Paulo, Brazil The aim of this presentation is to describe the clinical and neurophysiologic features of osteo-chondro-muscular dystrophy, a rare syndrome. This 5-year-old black male child was born of consanguineous parents, after an eventful pregnancy and delivery. He appeared normal at birth but had a peculiar facial expression and difficulties with sucking and swallowing were noticed by his mother by the age of 6 months. Milestones of development were normal except for mild walking delay. On physical examination, at the age of 5, he was 92.8 cm in height and weighed 11 kg. His intelligence was unimpaired and he had a high-arched palate. The face was expressionless and immobile, and the palpebral fissures were narrow with blepharospasm and ptosis. The mobility of his mouth and jaw was poor and he had a high-pitched voice. He presented with a stiff gait, depressed tendon reflexes and global pseudohypertrophy of the proximal muscles. No percussion myotonia could be elicited. Laboratory data revealed a mildly elevated CK and normal alkaline phosphatase and serum electrolytes. Radiographic studies revealed shortening of tibial diaphysis and normal vertebral bodies. EMG