Search

A series of imidazolinylindole derivatives were discovered as novel kallikrein 7 (KLK7, stratum corneum chymotryptic enzyme) inhibitors. Structure-activity relationship (SAR) studies led to the identification of potent human KLK7 inhibitors. By further modification of the benzenesulfonyl moiety to overcome species differences in inhibitory activity, potent inhibitors against both human and mouse KLK7 were identified. Furthermore, the complex structure of 25 with mouse KLK7 could explain the SAR and the cause of the species differences in inhibitory activity.

A facile preparation of 3-acyl-substituted isoxazolines, benzisoxazoles, and isoxazoles from the corresponding 3-carboxylate esters is described. The process, involving reaction of the ester derivative of 3-carboxylic acid substituted heterocycles with Grignard or alkynyl lithium reagents, leads to direct generation of the corresponding 3-acyl heterocycle. The presence of α-imino ester moieties in the heterocyclic substrates for the reactions is thought to be a key feature governing the nature of these transformations. The synthetic utility of the new methodology is demonstrated by its application in a two-step route for the preparation of novel linked bis-heterocycles.

The subjects were 106 SMIDS with epilepsy. They were classified into four epileptic syndromes: (1) SE-MISF (34.0%), (2) SGE (25.5%), (3) SLRE (20.7%), and (4) epileptic discharge-free patients (EDFP) (19.8%). Clinical electroencephalographic studies elucidated the following result: (1) The seizure disappearance rate was the highest in SLRE (54.5%), and it decreased in the order of EDFP (47.6%), SE-MISF (36.1%), and SGE (11.1%). (2) Status epilepticus was most frequently seen in SGE (62.4%), but it was not so often seen in EDFP (14.3%) or SLRE (22.7%). (3) The age at seizures onset was the lowest in SE-MISF (0.84 years), and it increased in the order of SLRE (1.3), SGE (2.3), and EDFP (6.7). (4) The rate of Ohshima's classification 1 was highest in SE-MISF (61.1%) and lowest in SGE (40.7%). In conclusion, epileptic syndrome and EEG findings are good indicators for predicting the seizure prognosis and some of the clinical features, and the majority of epileptic syndromes could be classified by the very first EEG findings. Since epilepsy in SMIDS is so frequent (70.3%) and intractable (seizure disappearance rate more than 3 years, 36.2%), more attention should be paid to electroencephalography and epileptic seizures in SMIDS.

The static stability of COW (Coal-Oil-Water Mixture), which is made by the disintegration of leashed coal agglomerates, is investigated. The static stability is evaluated by two methods, that is, the direct measurement of coal volume fraction in COW and the rod penetration test. When the static stability of COW is high, the difference of coal volume fraction between the upper section and the lower section of the storage column is small, and the 50% diameters of coal particles in both sections are almost equal in size. On the other hand, when the static stability of COW is low, the coal volume fraction becomes large in the lower section, and in contrast the water volume fraction becomes large in the upper section. It is found that the rod penetration test only shows whether the hardpack layer of coal particles is formed on the column bottom. An empirical equation, which represents the degree of the static stability of COW, is obtained.

The flow characteristics of a coal-oil-water mixture (COW) were investigated. COW was prepared by disintegrating de-ashed coal agglomerates that had been obtained by oil agglomeration of coal. When the volume fraction of dispersed waterdrops in COW was smaller than 0.2, the dispersed waterdrops had a similar effect to that of the dispersed coal particles on the flow characteristics of COW. COW behaved as a Bingham fluid. Both the yield value and Bingham viscosity increased with increases in the total disperse-phase volume fraction in COW and with decreases in temperature. The relative viscosity of COW was well-correlated by an equation derived from Mooney's equation.

The flow characteristics of coal-oil-water mixture (COW) was investigated. COW was prepared by a disintegration of deashed coal agglomerates, which were obtained by the oil agglomeration of coal. When the volume fraction of dispersed waterdrops in COW was smaller than 0.2, the dispersed waterdrops influenced the flow characteristics of COW to the same extent as the dispersed coal particles. COW behaved as Bingham fluid. Both the yield value and Bingham viscosity increased with increasing total disperse phase volume fraction in COW, and with decreasing temperature. The relative viscosity of COW was well correlated by the equation derived with reference to Mooney's equation.

The reaction of methyl esters (I) and (VIII) derived from the title heterocycles with Grignard reagents or alkyl and alkyne lithium compounds is investigated.

Summary: We report eight sporadic cases of typical Angelman syndrome (AS) associated with chromosome 15q12 deletion. Age at first visit was 3–35 months (average 18 months), and follow-up period was 4-20 years (average 14.1 years). The characteristic features of epilepsy in AS are (a) seizure onset in early childhood (8 of 8); (b) evolution of seizure type with age (8 of 8); (c) EEG abnormality changes from high-voltage slow bursts (HVS) in infancy to diffuse spike and waves in middle childhood (4 of 5); (d) atypical absence seizures (8 of 8), often occurring as atypical absence status (4 of 8); and (e) diminution of seizure discharges and clinical seizures after puberty (7 of 7). We believe that AS may frequently exist in the intractable epilepsies of childhood with severe mental retardation. We stress the importance of AS as one of the main etiologic background diseases of the intractable epilepsies with infantile onset such as West syndrome, Lennox-Gastaut syndrome, and others.

Summary Purpose The purpose of this study is to examine prognostic factors for seizures in 106 epileptic patients with SMIDS. Subjects and methods One-hundred-six epileptic patients with SMIDS were the subjects of this study. The study group consisted of 60 male and 46 female patients. The ages ranged from 4 to 61 years. They were all followed up for more than 4 years in our residential facility hospital “Kobato Gakuen”. Fourteen possible prognostic factors were investigated statistically, and the validity is studied by factor analysis (principal component method). Results Statistically significant poor prognostic factors for epileptic seizures in SMIDS were (1) status epilepticus; (2) multifocal spikes (MFS) or Diffuse spike and waves (DSW) on final EEG; (3) symptomatic generalized epilepsy; (4) MFS or DSW on first EEG; (5) multi-antiepileptic drugs; (6) postnatal etiology; and (7) short duration of institutional hospitalization. As a result of factor analysis, the following five factors are elucidated: (1) Age/Time Passage; (2) Status epilepticus/Etiology; (3) Epileptic syndrome/EEG; (4) intensive medical care; and (5) Severity of Disabilities/Gender. Conclusion Our findings indicate that intractability of epilepsy may be identified early in the course of the disease, even in SMIDS, and EEG and epileptic syndrome are the very important factors for predicting the seizure prognosis.

We discuss here the indication and complications of tracheostomy performed in 57 home-care pateints with severe motor and intellectual disabilities (SMID) during the past 13 years at our hospital. Thirty-five cases underwent tracheostomy following emergency endotracheal intubation for acute respiratory failure. Recently, the number of cases without preceding endotracheal intubation have increased. Many patients underwent tracheostomy at the age of 1 to 4 years and 10 to 14 years. The quality of life (QOL) of almost all the patients without preceding intubation markedly improved, as well as that of their families, and they were able to return to home. The most decisive reason for tracheostomy was secretions and recurrent aspiration pneumonia in 8 patients, gastroesophageal reflux in 4 and upper airway obstructions in 3. Several complications of tracheostomy were observed: tracheal granulations in 9 patients, tracheal malacia in 8, and tracheoinnominate artery fistula in 5. Among 8 patients with tracheal malacia, bleeding from the tracheoinnominate artery fistula occurred in 3. In 7 patients, self-made long tracheostomy tubes were necessary for the initial management of the tracheal malacia or tracheal granulations. Subsequently, made-to-order long tracheostomy tubes were used in three of these patients. In 12 patients, improved endotracheal T-tube with the tip sealed on the vocal cord side was used to prevent aspiration. Home-care SMID patients with respiratory disturbance require tracheostomy timely performed, followed by careful observation to prevent postoperative complications.

Mutations in a gene on the X-chromosome encoding methyl-CpG-binding protein 2 (MECP2) cause Rett syndrome. We examined clinical symptoms of 27 patients with Rett syndrome (aged 2 to 37 years), diagnosed by the criteria of the Rett Syndrome Diagnostic Criteria Work Group. having MECP2 gene mutations. Two novel MECP2 mutations, 119 del AG resulting in amino acid frame-shift 40fs43X and C to G transversion resulting in amino acid change of F157L, were found. All patients had the most important symptoms of this syndrome, including loss of acquired purposeful hand skills followed by stereotyped hand movements. Two patients had mild perinatal abnormalities. Nine showed psychomotor delay or hypotonia before 6 months. Five patients over 4 years old did not have microcephaly. Speech was preserved in five patients. According to the criteria, 18 cases were diagnosed as Rett syndrome variants. Sixteen out of 26 patients over 3 years old were able to walk (61.5%), and 22 had epilepsy (84.6%). Mutations of the 5 patients without microcephaly were R133C, P225R, R255X, R306C and 376fs386X, whereas those of the 5 variants with preserved speech were 34fs123X, R133C, R255X and R270. Common T158M mutation was detected in 4 patients, R255X in 7 and R270X in 4. Patients with the same mutations showed different phenotypes. Patients with R133C and R306C presented a mild phenotype without microcephaly. Of the proposed diagnostic criteria, the following three may not be essential: apparently normal prenatal and perinatal period, apparently normal psychomotor development through the first 6 months, and deceleration of head growth between 5 months and 4 years.

This study aimed to assess the visuospatial abilities of five children with Williams syndrome (four males aged 9 years 3 months, 7 years 11 months, 8 years 1 month, and 10 years 8 months respectively, and one female aged 6 years 3 months). First, the children's visuospatial abilities were examined by asking them to copy a figure. Second, their cognitive processing abilities were assessed using the Japanese Kaufman Assessment Battery for Children. This test was used because it is an objective one, standardized in Japan, and is a measure of fluid ability including spatial localization. Participants scored significantly low on the spatial memory subtest indicating that there was a deficit in spatial localization. Children's performance in line copying tasks improved when the dots were in colour. Results suggest a deficit in the dorsal stream of visual cognition, with a relatively preserved ventral stream.

A GaAs surface chemically treated with hydrochloric acid (HCl) or hydrofluoric acid (HF) has been characterized by measurements of the contact angle between the surface and a water droplet on it in air. The contact angle measured from the GaAs samples dipped into water shows the formation of a thin surface layer on the GaAs surface and the subsequent removal of the layer upon chemical treatment with HCl or HF solution. Annealing in nitrogen atmosphere is shown to be effective in stabilizing the surface against water.

The etiological factors, clinical aspects and long term prognosis were studied in 200 patients with infantile spasms. Forty-eight (24.0%) died and the rest were aged 6 years or more at the time of final follow-up. In 73 (36.5%) the etiology was prenatal, in 44 (22.0%) perinatal, and in 17 (8.5%) postnatal: 18 cases (9.0%) were cryptogenic. The remaining 48 (24.0%) patients were doubtful cases. The mortality of the pre-and perinatal cases at 35.6% and 34.1% respectively was significantly higher than that in the other etiologic groups (P

The EEG, visual and auditory evoked potentials (VEP, AEP) were evaluated in 16 full-term newborn infants who had intracranial hemorrhage documented by computerized tomography (CT). Three of them had supratentorial, three, supra- and infratentorial hemorrhage, while the other ten had infra- or peri-tentorial bleeding. Three died during the neonatal period. Eight of the 13 surviving infants were neurologically normal and five were abnormal at the time of the follow-up. Those who had normal or mildly abnormal background EEGs all developed normally, while those whose neonatal EEG was severely abnormal subsequently developed neurological sequelae irrespective of the extent of intracranial hemorrhage. The EEG, VEP or AEP is of little value in the diagnosis of intracranial bleeding but the EEG is valuable in assessing the degree of associated parenchymatous damage and is of great prognostic significance.

We investigated the prognostic factors for mental and physical development and seizure control by dividing the subjects into various etiologic groups in 200 patients with infantile spasms, all of whom (except 48 who died) were aged six years or older. The results were as follows: 1) Intermediate (4-12 mos) onset was found to be a favorable prognostic factor for seizure control in cryptogenic cases, although there was no relation between the age of onset and prognosis in other etiologic groups. 2) There was a significant correlation between the treatment lag and long-term prognosis for mental and physical development only in cryptogenic cases. A short treatment lag (0-2 mos) was associated with a good prognosis. All cryptogenic patients who had no relapse after ACTH therapy developed normally, although in symptomatic cases, there was no correlation between the relapse and the outcome.

We investigated the effects and side effects of thyrotropin-releasing hormone (TRH) on severely epileptic children to evaluate the clinical usefulness of TRH in the treatment of epilepsy and compared them with the results of ACTH therapy. The subjects were 64 patients admitted consecutively between 1980 and 1986. Their seizures were frequent, more than one a day or more than one a week. The subjects were divided into two groups; 33 patients treated with ACTH and 31 treated with TRH. The mean follow-up periods in TRH and ACTH therapy were 8 months and 3.0 years, respectively. The daily dose of TRH-t 0.5-1 mg was administered intravenously (i.v.) or intramuscularly (i.m.) for 1-4 weeks. The follow-up periods were 3-12 months (mean 6 months). In the TRH group, complete control of seizures was achieved in 7 of 13 (53.7%) of those with infantile spasms, and marked improvement of EEGs were observed in 8 of 13 (61.5%) of them. In the ACTH group, seizure cessation was observed in 75% of infantile spasms. Of the patients who received ACTH, 66.7% had various side effects, including pneumonia, huge subcutaneous abscess, hypokalemia, cataracts, and brain shrinkage as shown on computed tomography (CT), whereas only 16.7% of the patients treated with TRH had transient reduction of urine volume without other laboratory and physical abnormalities. The results of the study indicated that some patients who received TRH had cessation of infantile spasms and improved EEG findings with no serious side effect. Because of the untoward side effects of ACTH therapy, TRH is considered a possible new treatment for children with infantile spasms.

複雑部分発作重積症を呈した9歳男児例の臨床症状および脳波の経時的変化を報告した。発作時脳波は右後側頭部から連続性の棘波が出現し次第に全汎性徐波となるもので, 臨床発作像は口部自動症を伴う複雑部分発作 (CPS) であった。発作-脳波同時記録で確認できた35分間に計7回の2-4分間持続するCPSが反復し, 発作間にも意識回復を認めなかった。DZP静注により臨床発作, 脳波所見とも直ちに消失した。本症例はCPS初発後5日目よりCPS頻発および意識障害が出現し, 意識レベルの変動を認めながらも計7日間の意織障害が存在し, その後完全に回復した。回復後7日目に幻聴・幻視が一過性に出現し3日後に消失したが, これは脳波からfbrccd normalizationが考えられた。

The electroencephalographic features and their evolutional changes with age were described in three cases of lissencephaly syndrome diagnosed by CT scan. The case with more severe lissencephaly displayed very similar EEG findings. In early or middle infancy when infantile spasms began, EEG showed very high amplitude (more than 400 microV) slow waves mixed with sharp theta-waves. In their clinical course, they showed extreme spindles and in late infancy, the EEG revealed a tendency towards bilaterally synchronous discharges of high amplitude sharp and slow waves. On the other hand, milder forms of lissencephaly showed hypsarrhythmia in early infancy. In the late infancy the EEG showed bisynchronous sharp and slow waves of more than 200 microV. The anomaly ranging from agyria to pachygyria seems to be closely associated with varying EEG abnormalities from extremely high voltage hypsarrhythmia to focal spikes. The very high voltage of hypsarrhythmic patterns and the very low frequency of sharp wave discharges seem to be typical in the most severe lissencephaly or agyria

Sleep and body movements were studied in 2 hydranencephalic neonates using visual analysis and polygraphic recording. They manifested periodic alternation of active and quiet sleep with peculiar movements of bilateral synchronized patterns. Generalized phasic movements were significantly increased and localized tonic movements were reduced. In view of the complete absence of cerebral hemisphere in this disorder, the movement of bilaterally synchronized pattern or increase of generalized phasic movement are considered to be generated from the basal ganglia and/or brain stem without cerebral hemisphere, whereas, localized tonic movements have more cortical correlation in its organization. The observation of body movements may be useful in detecting hydranencephaly in early infancy.

162例のWest症候群患児について, 5～7歳時, 8～10歳時の発作型および脳波像について, さらに, それらと身体知能予後との関連について検討した。発作消失例は約1/3であり, 発作存続例の約80%は全汎発作を, うち80%以上が強直発作を持っていた。脳波が正常化したのは約10%で, 焦点性発作波, 全汎性発作波がほぼ同数認められたが, 焦点性発作波がわずかに多かった。全汎発作の症例の約30%は焦点性発作波を示し, 局所性焦点性発作波を示すものもあった。発作もなく, 脳波も正常化した症例の2/3は身体知能予後も正常であったが, 全汎発作の症例では, 身体知能障害が70%以上に認められた。

The evolutionary changes of evoked potentials (EPs) were studied from the neonatal period up to 1 year of age in 41 infants with various perinatal disorders. Abnormal EPs in the first week of life recovered quickly. In infants with normal outcome, abnormal EPs became normal within a month. In infants with cerebral palsy (CP) or mental retardation (MR), EPs recovered within 2–3 months of age. Infants with more severe neurological damage showed abnormal EPs even beyond 6 months of age. Abnormal EPs beyond 2 weeks of age indicated poor prognosis. As for the wave form of EPs, absent responses or abnormal wave form reflected more severe brain dysfunction. AEPs tended to show more profound abnormalities than VEP. However, some infants with absent AEP in the first week of life had a favorable prognosis. AEPs seemed to be more easily affected by brain dysfunction.

The classical Lesch-Nyhan syndrome has the deficiency of hypoxanthine-guanine phosphoribosyltransferase (HGPRT) activity as the result of mutation in the structural gene for the enzyme located on the X chromosome and is believed to be of X-linked recessive or sex-linked mode of inheritance. This is the first report of a girl who showed typical clinical features and biochemical characteristics of the classical Lesch-Nyhan syndrome. Her mother was not a heterozygote for a deficiency of HGPRT. Possible genetic mechanisms responsible for this case were discussed.

EEGs and visual and auditory evoked potentials (VEP and AEP) were studied in 27 newborns with congenital hydrocephalus. Asynchronous sleep patterns were the main EEG abnormality in the newborn as well as in the older infants and children. Asynchronous tracé alternant patterns, the most common finding, were more frequently encountered in infants with more severe hydrocephalus. Asynchrony of bifrontal humps was another finding observed in a few infants. Intermittent semirhythmic theta or delta waves were noted in some infants. Depression of the EEG background activity was an infrequent finding observed in more severe hydrocephalus. VEP showed prolonged latency in 60% of cases, but its wave form was normal in most cases, even sometimes in patients with highly abnormal EEGs. Therefore, it can serve as a tool to assess the maturity of such infants. In contrast, AEP was normal in 76% of the cases. The neonatal EEG findings were significantly correlated with the clinical outcome and can be an additional prognostic indicator in congenital hydrocephalus.

Mutations in ZFHX1B, encoding Smad-interacting protein 1 (SIP1), have been recently reported to cause a form of Hirschsprung disease (HSCR). Patients with ZFHX1B deficiency typically show mental retardation, delayed motor development, epilepsy, microcephaly, distinct facial features, and/or congenital heart disease, in addition to the cardinal form of HSCR. To investigate the breadth of clinical variation, we studied DNA samples from six patients with clinical profiles quite similar to those described elsewhere for ZFHX1B deficiency, except that they did not have HSCR. The results showed the previously reported R695X mutation to be present in three cases, with three novel mutations—a 2-bp insertion (760insCA resulting in 254fs262X), a single-base deletion (270delG resulting in 91fs107X), and a 2-bp deletion (2178delTT resulting in 727fs754X)—newly identified in the other three. All mutations occurred in one allele and were de novo events. These results demonstrate that ZFHX1B deficiency is an autosomal dominant complex developmental disorder and that individuals with functional null mutations present with mental retardation, delayed motor development, epilepsy, and a wide spectrum of clinically heterogeneous features suggestive of neurocristopathies at the cephalic, cardiac, and vagal levels.

Body movements during sleep were observed in 14 full-term sick newborn infants such as perinatal asphyxia, purulent meningitis, meconium aspiration syndrome, melena, porencephaly, and hydranencephaly. Five newborn infants who had recovered from transient vomiting were examined as control. Infants with minimally depressed background EEG showed an increase of generalized movement (GM) and localized tonic movement (LTM) in quiet sleep. Hydranencephalic infants revealed an increase of generalized phasic movement (GPM) in active and quiet sleep. On the other hand, a decrease or an absence of body movements was observed in infants with severe EEG abnormalities such as moderate, marked and maximal depression. Hydranencephalic and porencephalic neonates showed a decrease of LTM in active sleep. One infant with purulent meningitis, showing markedly depressed EEG, revealed GPM-like movements of much longer duration. The follow-up study revealed that the absence of body movements or the increase of GPM might indicate a very poor outcome, whereas the presence of LTM, even in a small amount, suggested the preservation of cortical functions. The increase of body movements was also considered to correlate with sleep disturbance and served to indicate a brain dysfunction.

Body movements during sleep were examined in eight non-eventful preterm babies and two full-term babies using the polygraphic methods including superficial EMG. Typical movements in quiet sleep were startle (St) or generalized phasic movement (GPM) and increased tonic activity in chin muscles. In active sleep, instead of total simultaneous patterns observed in quiet sleep, more un-coordinated and localized movements such as generalized movement (GM), localized tonic movement (LTM), localized phasic movement (LPM) and clonic movement (CM) occurred. Longitudinal study of movements revealed a decrease of St or GPM, GM and LPM. LTM did not decrease with increasing conceptional age. The differences of decreasing tendency among these movements may indicate the differences of maturational changes in the different part of CNS.

• Electroclinical features of convulsive apnea and its relation to the behavioral state were described on the basis of polygraphic recordings from 21 newborns with various underlying disorders, including perinatal anoxia, purulent meningitis, and intracranial bleeding. The most frequent ictal discharges were rhythmic alpha waves, but other types of discharges, such as repeated sharp waves, rhythmic theta waves, delta waves, and repeated paroxysmal wave complexes, were also frequently seen. The area where the ictal discharges initially occurred or were most prominent was the temporal area, suggesting the limbic origin of apneic seizures. In more than half of the cases, the sleep cycle was abolished. In those cases where the sleep cycle was preserved, the seizures occurred most frequently in active sleep, but never in quiet sleep. ( Am J Dis Child 1982;136:980-984)

A case of Marinesco-Sjogren syndrome manifesting cataract, short stature, psychomotor retardation, hypogonadism, and progressive ataxia was described. The result of electrophysiological study reflected the presence of peripheral nerve involvement, which was clearly confirmed by sural nerve biopsy. The conspicuous abnormality observed in the peripheral nerve was the presence of segmental demyelination. The process of axonal degeneration was not remarkable. Results confirmed that Marinesco-Sjogren syndrome is a disorder which may involve both central and peripheral nervous system.

Various factors possibly influencing responsiveness to thyrotropin-releasing hormone (TRH) therapy were studied in 38 children (20 M, 18 F) with severe epilepsy. Mean age at treatment was 4.7 years (range; 0-18 years). Seizure type was infantile spasms (IS) in 16, generalized tonic seizures in 8, secondarily generalized partial seizures in 4, generalized tonic-clonic seizures in 2, atypical absence in 5, myoclonic seizures in 1, and atonic seizures in 2 cases. All seizure types were classified by ictal EEGs documented by simultaneous EEG-VTR according to the International Classification of seizures, except for two with atonic seizures and one with IS. Factors analyzed were sex, age, etiology, neurologic abnormality, seizure types, seizure frequency, EEGs, duration of TRH therapy, and serum hormone [human growth hormone (HGH), prolactin (PRL), thyroid-stimulating hormone (TSH)] levels before and after TRH therapy. We showed that: (a) serum PRL level was significantly correlated to effectiveness of TRH therapy (the higher the PRL, the greater the response rate); (b) serum basal PRL decreased significantly, especially in good responders; and (c) serum basal PRL was elevated especially in patients with IS, which responded more to TRH therapy. These interesting findings seen in severe epilepsy of childhood deserve further neuroendocrinologic study.

Neonatal findings and evolutionary courses of epileptic seizures were described in newborns who had seizures during the neonatal period and later developed epilepsy. Neonates with CNS dysgenesis ran the highest risk (80%) for subsequent epilepsy. About 30% of those who suffered from perinatal hypoxia and/or intracranial birth injury and 30% of those who had meningitis developed subsequent epilepsy. In perinatal brain injury, those who developed epilepsy showed more abnormal neurological and electro encephalographic findings than those who did not. The more abnormal the neonatal background EEG, the more frequently epileptic fits developed later. There was no such relationship in CNS dysgenesis. Myoclonic seizures were associated with the most severe brain damage. Newborns who later displayed West syndrome had also very abnormal neonatal EEG whereas those who manifested other types of fits more often had less abnormal neonatal EEGs. In West syndrome, hypsarrhythmia was preceded by focal or multifocal spikes, and closely temporally related with the onset of spasms, but there was a variety of intervals between the onset of clinical fits and EEG spikes in generalized or focal motor seizures.

SUMMARY Electroclinical features of neonatal seizures were studied in 215 neonates who developed seizures during the first three weeks of life. In 62 of them, clinical fits occurred during the actual polygraphic recording. The electro-clinical correlations were very variable, although there were some statistically significant associations between certain types of seizures and electrical discharges. The relation between seizures and sleep states was investigated in 40 infants who had more than three fits during the recording. The state in which more than 70% of seizures occurred was active sleep in 28% of the cases, quiet sleep in 13%, indeterminate sleep in 8%, and wakefulness in 8%. In 50%, seizures did not tend to occur in a given state. The authors thank Professor Suzuki for his continued interest and encouragement.