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Purpose: To underline the unusual but possible occurrence of epileptic spasms (ES) in children >1 year of age., Methods: Cases in whom onset of spasms occurred after 1 year of age were identified through a retrospective review of the records of all patients referred for ES to the Saint-Vincent de Paul Hôpital (Paris) and American Memorial Hospital (Reims) between 1974 and 1994., Results: Eighteen cases were identified among the 734 children referred for ES, 18 cases were identified where spasm onset time ranged from 12 to 38 months of age. In 1/3 of the cases, the diagnosis was suspected from the onset of clinical manifestations; in the remaining 2/3, diagnosis was delayed by a mean 6 months (range, 2-25 months). Neurobehavioral regression affected two-thirds of the patients. Modified hypsarrhythmia was present in 11 patients; all but one exhibited major and diffuse spike- and slow-wave activity. EEG abnormalities were detected in the frontal areas in 11 patients. Spasms were cryptogenic in 9 patients. Steroids were administered to 13 patients; these controlled the spasms in 6 patients. Outcome was favorable for both seizures; cognition favorable in only 2 of the 18 patients., Conclusions: Beginning after the first year of life, ES, or late-onset infantile spasms, are distinct from early Lennox-Gastaut syndrome, although etiology, prognosis and treatment are similar to that for the classical infantile spasms.

Transient movement disorders are quite common in pediatrc practice, occurring mainly in infants. They are underdiagnosed but represent about 20% of cases of movement disorders (tics excluded) seen in a neuropaediatric department. Dystonia, tremor and myoclonus are the most common. Transient idiopathic dystonia of infancy is quite common, and the diagnosis can be suspected on clinical examination. Knowledge of these disorders avoids not only unnecessary tests and treatment, but also familial anxiety.

The time relationship between DTP immunization and infantile spasms (IS) onset was examined using three models--association, temporal shift, and no-effect--and the case/control data from the National Childhood Encephalopathy Study (NCES). Infantile spasms cases classified as being previously abnormal (e.g., tuberous sclerosis complex patients) showed a no-effect relationship, whereas those classified as previously normal suggested a fit to the temporal shift model, i.e. no increase in number of cases but a shortening of time to onset of seizure. No data fit the association model. Analyses for vaccine complications should examine for temporal changes (i.e. temporal shift) in addition to increased risks.

Purpose: To determine the prevalence and descriptive epidemiology of Lennox-Gastaut Syndrome (LGS) among metropolitan Atlanta children., Methods: We conducted a population-based study of LGS as part of the Metropolitan Atlanta Developmental Disabilities Study (MADDS) using a multiple-source surveillance system for epilepsy and developmental disabilities. Children were defined as having LGS if they had onset of multiple seizure types before age 11 years, with at least one seizure type resulting in falls, and an EEG demonstrating slow spike-wave complexes (<2.5 Hz). Mental retardation (MR) was not used as a diagnostic criterion., Results: The lifetime prevalence of LGS at age 10 years was 0.26/1,000. Ninety-one percent of those with LGS had MR (IQ < or = 70), and 39% had a history of infantile spasms (IS). A comparison of children with LGS and those with multiple seizure types without slow spike-wave complexes demonstrated that those with LGS were more likely to have MR, history of IS, and multiple disabilities (MR, cerebral palsy, blindness, hearing impairment). Seventeen percent of all children in Atlanta with profound MR (IQ < 20) had LGS., Conclusions: LGS accounts for only 4% of all childhood epilepsy, yet is a significant contributor to childhood morbidity.

The incidence of epilepsy in 323 patients with cerebral palsy (CP) was 41.8%. Almost half of the patients with spastic tetraplegia and hemiplegia had epilepsy. The incidence was lower in patients with spastic diplegia. No sex differences were observed. Partial seizures were by far the most common form of epilepsy in spastic hemiplegia, while generalized tonic-clonic episodes predominated in all other forms of CP. A very high incidence of West syndrome was observed in patients with spastic tetraplegia. Most of the patients with spastic tetraplegia had their first seizure in the first year of life. In patients with spastic hemiplegia the onset of epilepsy was often delayed for several years. A high rate of polytherapy was recorded, but two-thirds of the patients remained seizure-free for long periods. In just over one-fifth of the patients successful withdrawal of medication was achieved.

Of the epileptic syndromes beginning in early infancy, we described the clinical and electrical characteristics of early myoclonic encephalopathy (EME) and Ohtahara syndrome (OS), and the differential diagnosis between the two syndromes, reviewing previous articles and our own study of 6 cases with EME and 15 cases with OS. With regard to the clinical seizure types, the main seizure type was tonic spasms in OS while myoclonias, especially erratic myoclonias, and frequent partial seizures were predominant in EME. The suppression-burst (S-B) pattern was a common feature of both syndromes, although their form, time of appearance, and duration of appearance differed considerably. The S-B pattern in OS was characterized by periodic and consistent appearances during both waking and sleeping states, whereas in EME, the S-B pattern was enhanced by sleep and often not manifested in the awake states. OS showed a specific pattern of evolution as age-dependent epileptic encephalopathy with concomitant EEG change while EME had no specific evolution with age. In OS, obvious brain lesions such as brain malformations were often seen. In contrast, frequent occurrence of familial cases suggested some kind of congenital metabolic disorder as the etiological factor in EME.

The health records of 76 infants with infantile spasms (IS) were reviewed retrospectively. The distribution of seizure onset was examined with respect to calendar month, annual changes in day length (photoperiod), and global solar radiation (GSR). IS onset was more frequent during the months with low GSR and short photoperiods. The greatest frequency of IS onset was in December and January (22 cases, 29%); the lowest was in April and May (8 cases, 10.5%). IS onset frequency (f) was more than twice as great in the months with a short photoperiod (10Light-14Dark) and a low GSR (f = 11) as compared with months with a long photoperiod (16Light-8Dark) and a high GSR (f = 5). IS onset frequency was 2.2-fold greater in months with a short photoperiod and a low GSR. This finding suggests that environmental photoperiodic factors (zeitgebers) may play a role in the onset of IS.

Purpose: To study the occurrence of partial seizures (PS) and elucidate the characteristics of patients with West syndrome (WS) with PS., Methods: We investigated the electroclinical and radiologic features in 92 children with WS who were older than 3 years at follow-up., Results: Thirty-six (39.1%) children had PS at some time during the course of the disease. They had a significantly high incidence of asymmetric spasms, hemiparesis, and asymmetric hypsarrhythmia. Their seizure prognosis was significantly less favorable. PS appeared only before spasms (group A, six children), concomitant with spasms (group B, 18 children), and only after spasms ceased (group C, 12 children). Five children in group B had PS after spasms stopped. PS in group A and during the period of active spasms in group B showed high seizure frequency and variability of both seizures and EEG manifestations. The PS and EEG epileptiform discharges were mainly in parietal-posterior temporal-occipital, and central regions. Late PSs in group B and PSs in group C were characterized by stereotyped manifestations and relatively low seizure frequency. These PSs originated mostly from frontal regions., Conclusions: In some children with WS, cortical lesions can induce both PS and spasms in series, whereas in others, both types of seizures occur with abnormally increased excitability throughout the cortex rather than as the result of a single cortical lesion. These findings suggest caution in interpreting focal discharges, which may fluctuate and are not necessarily indicative of a surgically accessible lesion. When the discharges are stable and correlate with an equally stable clinical pattern, the likelihood of an underlying cortical lesion is greater.

The combination of West syndrome (WS) and Down syndrome appears not to be coincidental. Fourteen patients free of cardiac malformation or history of perinatal hypoxia were referred and investigated before they had received any treatment and were followed to the mean age of 4.5 years (range 19 months to 14 years). Spasms had onset at the mean age of 8 months (range 4-18 months) in cluster and were symmetrical. Hypsarrhythmia was symmetrical and, after intravenous diazepam (4 patients, 0.5 mg/kg) it disappeared, without any remaining focus. Recorded spasms during a cluster were "independent," with recurrence of hypsarrhythmia between successive spasms, and thus had the ictal and interictal EEG characteristics of idiopathic WS. Seven patients exhibited other types of seizures after WS, consisting of myoclonic jerks, atonic, tonic-clonic or absence seizures, which proved quite easy to control with valproate and/or ethosuximide.

This population-based, retrospective cohort study of neonatal seizures included all 16,428 neonates born to residents of Fayette County, Kentucky, from 1985 to 1989. Eighty potential cases were ascertained by computer search of hospital-based medical record systems, birth certificate data files, and multiple-cause-of-death mortality data files. Medical records for potential cases were abstracted, and relevant portions were reviewed independently by three neurologists using prospectively determined criteria. Both unweighted and weighted kappa statistics were used to measure agreement between each pair of observers in the classification of potential cases as seizures, possible seizures, or not seizures, adjusting for the proportion of agreement expected by chance. Agreement in the classification of potential cases was excellent (kappa = 0.72-0.79, average = 0.76; weighted kappa = 0.85-0.88, average = 0.87). The kappa extension statistic of Kraemer was used to assess agreement in the classification of seizure types by a simplification of the classification scheme of Volpe. This documented excellent agreement between raters in the classification of seizure types (kappa e = 0.72). Experienced raters can reliably classify potential cases of neonatal seizures using seizure descriptions transcribed from medical records.

Purpose: The characteristics of seizures were analysed in a population-based study of active epilepsy in 6- to 13-year-old mentally retarded children., Methods: The search procedure included diagnostic registers, EEG registers, and registers of the Education of the Subnormal. Medical files were scrutinized, and clinical examinations and interviews with parents or caretakers or both were performed., Results: The median age of seizure onset was 1.3 years, 3.1 for children with mild retardation and 0.8 for children with severe retardation. Among the 98 children identified, current seizure groups were partial in 20, generalized in 59, and mixed in 19. The prevailing seizure types were tonic-clonic, myoclonic, atypical absences, and partial complex seizures, present in 42, 33, 23, and 23 children, respectively. A total of 46 children had more than one seizure type. Seizures every day/week occurred in 44 children. There was a constancy between seizure type at onset and later seizure type. Neonatal seizures (n = 25), infantile spasms (n = 12), and status epilepticus (n = 37) occurred independent of one another. Prognostic factors for poor neurologic outcome were early onset of epilepsy, infantile spasms as onset type, and prior neonatal seizures. Children with only partial seizures less frequently had severe mental retardation, cerebral palsy, and visual impairment than those with only generalized seizures., Conclusions: Epilepsies in children with mental retardation are characterized by severe seizure manifestations. The brain damage giving rise to mental retardation and epilepsy is probably the main factor in terms of seizure outcome.

To analyze the magnitude of the risk factors for infantile spasms, we evaluated the records of 80 children with infantile spasms, 474 children with other types of epilepsy, 2,196 children with febrile seizures, and 262 children with CNS infections. There was a family history of seizures in 13.8% of children with infaNtile spasms, 28.5% of children with other forms of epilepsy, 25.5% of children with febrile seizures, and 5.3% of children with CNS infections. Children with a family history of seizures were 2.82 times more likely to have infantile spasms, 7.05 time more likely to have other epilepsy, and 6.08 time more likely to have febrile seizures than controls (children with CNS infections). However, a family history of seizures increased the risk for infantile spasms only in the cryptogenic group. Children with infantile spasms were significantly more likely to have cerebral palsy, microcephaly, hydrocephaly, CNS malformations, neonatal hypoxia, or neonatal seizures than children with other types of epilepsy, febrile seizures, or CNS infections. There was a modest genetic predisposition to seizures in children with infantile spasms. However, our data suggest a much stronger association with underlying neurologic abnormalities, mainly neonatal seizures, neonatal hypoxia, and CNS malformations.

We performed a retrospective study of infantile spasms (IS) in 57 children (19 girls and 38 boys) from the east-central region of Sweden for the period 1987-1991. The incidence was 0.45/1,000 newborns a year, with a spread of 0.17-0.76 between the seven counties included. Forty-three cases (75%) were symptomatic and 14 (25%) were idiopathic. Cerebral malformations were diagnosed in 8 children, and chromosomal aberrations were diagnosed in 5. In addition, 2 children each were diagnosed as having tuberous sclerosis and neurofibromatosis. At follow-up, 57% in the idiopathic group had normal development, whereas 91% in the symptomatic group had died or developed a multihandicap. The value of modern neuroradiological techniques for etiological diagnosis is stressed.

The present study examined whether changes in the incidence of infantile spasms could be used to evaluate changes in the quality of pre- and perinatal care over time. 107 children in 1960 to 1976, and 102 in 1977 to 1991, developed infantile spasms in southern Finland. The number of small-for-gestational age (SGA) infants and infants with neonatal hypoglycaemia decreased significantly. Brain malformations, malformation syndromes and patients with tuberous sclerosis increased; this probably reflects the development of more refined neuroradiological screening methods. SGA infants are probably more apt to develop infantile spasms than preterm infants appropriate for gestational age. Early prenatal factors seem to play a major role in the genesis of infantile spasms. Little can be done to reduce the incidence of infantile spasms, but every effort should be made to reduce the number of SGA infants by good prenatal care. Treatment of hypoglycaemia is important because infantile spasms might be preventable in these infants.

We determined the average annual incidence of epilepsy characterized by infantile spasms (IS) in Iceland based on the 13 cases identified in a 10-year interval (1981-1990). The cumulative incidence was 3 in 10,000 live births. Males were more frequently affected than females. All patients responded completely (10) or partially (3) to ACTH or steroid therapy. At follow-up, all children with cryptogenic IS are seizure-free and have developed normally intellectually. One of the children with cryptogenic IS has mild spastic diplegia. All children in the symptomatic group are mentally retarded, and 5 of 7 continue to have unprovoked seizures. We detected no evidence for familial aggregation, and the incidence of seizures or epilepsy did not appear to be increased in first-degree relatives of cases. The proportion of cases with a favorable outcome was greater than that reported in most clinical series.

Seventeen infants (7 boys and 10 girls) with visual abnormalities (such as poor tracking and following and nystagmoid eye movement) and occipital slow activities with irregular polyspikes on EEG were studied. Two thirds of the children showed development of West syndrome with hypsarrhythmia on follow-up EEG. In 1 child, hypsarrhythmia was evident on the follow-up EEG, but no clinical seizures occurred. Because most of the children in our series developed West syndrome, patients with visual abnormalities associated with occipital EEG discharges in early infancy may be at high risk for West syndrome. Occipital abnormalities in early infancy may relate to the occurrence of West syndrome.

The authors retrospectively identified 17 children with Down syndrome who developed infantile spasms, and analysed their etiology, EEG findings, response to medication, development and prognosis. Compared with symptomatic infantile spasms in the general population, which have a poor prognosis, these patients had a relatively benign outcome with regard to seizure control: only three of 16 survivors currently have seizures and seven of the 16 currently receive anticonvulsants. Developmental outcome was poorest in those with a superimposed hypoxic insult and in those who regressed developmentally at the onset of the spasms and did not regain developmental milestones. The overall neurological prognosis for children with Down syndrome and infantile spasms appears to be better than for children with infantile spasms in the general population.

A surveillance network was established in Oregon and Washington state to identify cases of certain neurologic illnesses during a one-year period (August 1, 1987 to July 31, 1988) among children 1-24 months of age. Reported here are the observed case occurrence rates and findings from capture-recapture analyses used to predict rates that would have been observed had ascertainment been complete. The network consisted of a hospital component involving 98% of all eligible facilities, a provider component involving 93% of all eligible pediatricians and neurologists, and a research staff component of record abstractors. Of 3,876 potential cases reported, 626 met the study criteria. The observed rates for the primary diagnostic groups were: encephalopathies, 16 per 100,000 children; infantile spasms, 10 per 100,000 children; afebrile seizures, 159 per 100,000 children; and complex febrile seizures, 101 per 100,000 children. Of the 626 qualifying cases, 41% were reported by two or more surveillance components. Capture-recapture analyses with log-linear modeling to control for source dependence suggested 80% of all study cases were detected. Comparable percentages for the four illness groups were: encephalopathies, 82%; infantile spasms, 94%; afebrile seizures, 69%; and complex febrile seizures, 91%. The predicted rate for afebrile seizures, corrected for under-ascertainment, exceeded the upper 95% confidence interval bound around the observed rate. For all other conditions, predicted rates fell within the 95% confidence intervals around the observed rates. These findings suggest capture-recapture analyses should be applied to the full sample of cases and to relevant disease substrata.

A 3-year-old boy with West syndrome who was shown to have a cystic lesion on magnetic resonance imagings underwent surgical treatment. He had been initially managed with several antiepileptic drugs, but abnormalities on electroencephalography (EEG) persisted. Histological examination of the cystic wall obtained during surgery suggested porencephaly. Two years and 2 months later the child remained seizure-free and his EEG remarkably improved.

Fifteen patients with neurofibromatosis type one (NF1) and West syndrome (WS) were studied. The evolution of WS was clearly different from that seen in the other neurocutaneous syndromes, especially tuberous sclerosis. Steroids were efficacious and spasms disappeared. There was no relapse after discontinuation of steroids and antiepileptic drugs were successfully stopped several years later. Mental outcome was also very good. The association of both conditions does not seem to be a coincidence. WS with NF1 seems remarkably benign and resembles idiopathic WS more than symptomatic WS.

Clinical features of seizures occurring in a neurofibromatosis clinic population were examined in a retrospective study. Twenty-two individuals in a population of 359 persons with neurofibromatos 1 (NF1) had had one or more seizures. Age at first seizure ranged from 4 days to > 20 years. Patients were classified into five groups on the basis of medical history, seizure type, and EEG findings. Two patients had seizures in the first 3 months of life: 1 was born prematurely and had perinatal encephalopathy, and the other developed infantile spasms (IS) and had extensive growth of a plexiform neurofibroma. Two males had seizures in association with aqueductal stenosis. Six individuals had one or more generalized seizures with fever, and 3 had primary generalized epilepsy. Nine patients had complex partial seizures (CPS), which varied in clinical manifestations and response to antiepileptic drugs (AEDs). In none of the individuals with seizures in this study was a structural lesion in the brain visible by neuroimaging. Seizures appear to be relatively uncommon in individuals with NF1, and when they occur have a natural history similar to that of seizures in the general population.

To determine the recurrence risk of West syndrome (WS), we studied the familial antecedents of consecutively referred patients. Among siblings, there was an increased incidence of WS but not of febrile convulsions. Familial incidence of epilepsy was intermediate between the epileptic and nonepileptic control groups. When cases resulting from a genetically determined disease were excluded, incidence of epilepsy among siblings was similar to that in normal controls. Five of the 11 familial cases of WS were due to an identifiable cause: twin pregnancy, tuberous sclerosis, and recurrent maternal toxemia. In 4 of the remaining families, the clinical picture included spasms, erratic myoclonus, and postnatal microcephaly, suggestive of a previously unidentifiable progressive encephalopathy. Therefore, when identifiable familial diseases were excluded, the recurrence risk was < 1%.

Purpose: To investigate the radiologic characteristics of the clinical progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy (PEHO) symptom complex. This complex is nonspecific, but within this syndrome, a subgroup with a defined neuropathologic phenotype and apparently autosomal recessive inheritance exists., Methods: Brain CT or MR studies were performed on 21 patients with the clinical PEHO syndrome. Their previous neuroradiologic studies were re-evaluated., Results: Twelve patients (group A) showed uniform changes with early progressive brain atrophy accentuated infratentorially, and abnormal myelination. The gyral pattern was normal. Brain atrophy of nine patients (group B) differed by being less progressive, supra- rather than infratentorial, and often combined with abnormal gyral formation., Conclusions: Postmortem studies permitted correlation of radiographic and morphologic findings in three cases. Two autopsied group A patients were compatible with the true PEHO syndrome, while one group B patient was incompatible. Group A seems to correspond to the core group of the PEHO syndrome. During a patient's life, a suggestive diagnosis of the true PEHO syndrome is thus feasible, although neuropathologic studies are needed for a conclusive diagnosis.

A 12-year retrospective study to elucidate the incidence of "fifth day fits" including 127 infants with neonatal convulsions was carried out in the Randers area of Denmark. The incidence of neonatal convulsions was 8.3/1,000 live-born infants. The etiology in 74 cases of latent and 53 cases of convulsive seizures was asphyxia (38%), intracranial hemorrhage (17%), hypoglycaemia (9%), hypocalcaemia (4%), infections (6%) and developmental defects (4%). In 28 cases (22%) the etiology was unknown. In only two of these cases, the seizures started on day 4-6, and no peak incidence was found.

Few population-based studies of infantile spasms have been done, and most reports have not included comparison groups. In spite of these limitations, this review attempts to summarize what is known about the distribution, etiology, and natural history of infantile spasms in populations; discusses the limitations of current data; and includes suggestions for further population-based research. Most estimates of the incidence of infantile spasms are between 0.25 and 0.42 per 1000 live births per year. Among children less than 10 years of age, the annual prevalence of infantile spasms is 0.14 to 0.19 per 1000. The peak age at onset of spasms is 4 to 6 months, and there appears to be a slight excess of male cases. The etiology of infantile spasms is unknown for 40% to 50% of affected children. Selected syndromes (eg, Aicardi syndrome, Down syndrome, etc) and inherited disorders (eg, tuberous sclerosis) may account for as many as one third of cases. Other factors, such as intrauterine infection, "birth injury," and head trauma have not been systematically evaluated, and thus, their contribution to the etiology of infantile spasms is uncertain. Areas for future etiologic research include controlled studies of immunologic factors and in utero and postnatal infections and further exploration of the interrelationship between infantile spasms and Lennox-Gastaut syndrome.

The pattern of epilepsy and other convulsive disorders in 1,000 consecutive Saudi nationals is described. These disorders were common with a hospital frequency rate of 8 per 1,000. Men were more frequently affected than women and 60% of the patients were under 10 years old at the onset of their illness. The epilepsies were the commonest type (74%). Febrile convulsions (20%) presented mainly between the ages of one and five years. Isolated seizures (3%) and acute symptomatic convulsions (3%) were uncommon. In the epileptic group, generalised seizures (71%) were more frequent than partial (29%) and complex partial seizures occurred mainly in those above 21 years old. Absences (4%), infantile spasms (3%) and atonic seizures (3%) were uncommon. No specific etiology of the epilepsy was determined in the majority of the cases (63%). The identified major etiologic factors of the epilepsies were perinatal encephalopathy (21%), cerebral trauma (11%), sequelae of meningitis or encephalitis (2%), brain tumors (0.5%), and vascular lesions such as stroke and arteriovenous malformation. Perinatal encephalopathy accounted for 40% of the epilepsies in children less than 5 years old, and trauma for 20% of those above 20 years old. A family history of epilepsy in close relations was obtained in 23% of the cases, and the consanguinity rate among the parents was 53%. The high incidence of associated perinatal encephalopathy found in this study suggests that perinatal factors play a major role in the pathogenesis of epilepsy in Saudi Arabia. The high frequency of cerebral trauma was also striking. Although consanguinity of the parents appeared not to be a major factor in the genetics of convulsive disorders in this environment, it might have potentiated the tendency of familial aggregation of convulsive disorders in this community. Consanguinity may be an important factor in the production of some of these disorders but its precise role has not been determined.

Computerized tomographic scanning of the brain was performed in 26 infants with Infantile spasms. Majority of the patients, 18 (69%) had some abnormality. Changes noted were cerebral atrophy in 12, calcifications in 5 and dysgenesis of the corpus callosum in 3 patients. One infant each had porencephaly, hydrocephalus and cavum septum pellucidum. Five patients had more than one abnormality simultaneously. Three infants had progressively worsening atrophy on ACTH therapy. Patients with infantile spasms, without any physical or neurological abnormality, are unlikely to have any abnormalities on brain CT scanning (p = less than 0.005).

The first multi-institutional survey of the Aicardi syndrome in Japan was performed during 1985 and 1986. Among the 20 collected cases, 9 fulfilled the typical clinical triad; infantile spasms, agenesis of the corpus callosum and chorioretinal lacuna. Three cases had agenesis of corpus callosum and chorioretinal lacuna. Five cases had agenesis of the corpus callosum and infantile spasms. We classified 12 cases with both agenesis of the corpus callosum and a lacuna as typical cases, and the remaining 8 cases as atypical cases. No familial cases were found among either the typical or atypical cases. The presence of the vertebral anomalies or other anomalies in the atypical cases, which were frequently observed in the typical cases, suggested that unknown common processes were affected in both the typical and atypical groups.

113 cases of infantile spasms were diagnosed in the period 1 April 1970 to 31 March 1975 after Denmark changed her immunization programme. Previously whooping cough vaccine was given as the triple combination at 5, 6, and 15 months of age. From 1 April 1970 it was given as a monovalent vaccine at 5 and 9 weeks of age and at 10 months of age. A comparison with previous material of 86 cases of infantile spasms from 1957 to 1967 showed no change in the age at onset. Though there may be an occasional connection between immunization and infantile spasms, the most important factor is a time-coincidence between any immunization given at a certain age with the neurological disorders which are natural for that age.

This series of cases of infantile spasms comprised 107 children born between 1960 and 1976 in the county of Uusimaa, Finland. The incidence of the syndrome was 0.38 to 0.42/1000 live births and has remained almost unchanged during those years, although there have been changes in the size of different aetiological groups. The importance of prenatal and perinatal factors, and especially of neonatal hypoglycaemia, as causal factors has decreased, whereas the numbers of cases due to infections and malformations has increased. Compared with investigations in other countries, this Finnish series differs in the incidence of familial cases and congenital cytomegalovirus infection, and especially in the higher incidence of neonatal hypoglycaemia and the lower incidence of tuberous sclerosis.

The incidence of hypsa over a 40-year period in 667 pts was noted to rise to a peak in 1958 and then to fall so that relatively few cases appeared in the early 1970's, with hypsa seen only rarely in the past 10 years. This diminishing incidence was confirmed in a major children's hospital. The incidence curve of rubeola is seen to follow a similar course; the possibility is discussed that subclinical infectious disease, and rubeola in particular as a prototype, may have played a more prominent role in the etiology of hypsa than has been considered in the past.

Data on incidence, aetiology, therapy and prognosis of infantile spasms are reviewed. In a large proportion of cases the aetiological factors of infantile spasms can now be established with some certainty. Especially the new neuroradiological and virological investigative methods have enabled a more accurate diagnosis. There have been changes in the aetiologic pattern over the years. The optimal dosage and duration of ACTH or glucocorticoid therapy have not yet been established. In the Finnish study the large ACTH doses (120-160 IE) generally used in many Nordic countries did not carry a better prognosis than the smaller doses (20-40 IE). Side-effects of ACTH therapy are relatively common. They are more frequent with large doses than with smaller ones. Measures can be taken to prevent or treat part of them. The prognosis and related factors in infantile spasms as well as some recommendations to improve them are reviewed.

During the 1970s, there was an epidemic of neonatal convulsions occurring in apparently normal babies during the fourth and fifth days of life. This syndrome was noted in France as well as in Australia. A study was undertaken to review the King George V Hospital (KGV) experience with these 'fifth day fitters'. All cases of neonatal convulsions at KGV during the years 1972-85 were reviewed. A total of 94 cases of fifth day fits occurred during this period, accounting for 57% of all neonatal convulsions occurring during 1972-79. The fits occurred in healthy term infants after an uncomplicated pregnancy. They appeared on the fourth and fifth days of life. The seizures lasted an average of 24 h, were refractory to drug therapy and, despite extensive investigation, no cause was found. The infants were assessed as normal at the time of discharge from hospital. Follow-up of these infants was incomplete. However, from the data available, it cannot be assumed to be a benign entity. The 'fifth day fit' syndrome reached epidemic proportions at a number of maternity units during the 1970s. At KGV, no case has been observed since 1982.