Epidemiology of absence epilepsy. III. Clinical aspects.

Absence epilepsy was studied in a Swedish population, aged 0-15 years, in 1978-1982. Cases were selected by electroencephalographic criteria. In the 134 children with 3 Hz spike-and-wave discharges, 97 (72.4%) had absences alone or in combination with generalized tonic-clonic seizures (grand mal): 56 had absences alone, 31 absences followed by grand mal, and 10 started with initial grand mal. Two distinct groups could be discerned: 1) childhood absence epilepsy: onset before the age of 12, with a quick response to therapy, little or no risk of grand mal, and a high remission rate; 2) juvenile absence epilepsy: onset at the age of 12 or later, a very high risk of grand mal, and usually a good response to therapy, but a high risk of relapses at withdrawal. This classification of absence epilepsy into subgroups may be useful for prognostic guidelines.     

Epidemiology of Absence Epilepsy: III. Clinical Aspects

ABSTRACT. Absence epilepsy was studied in a Swedish population, aged 0–15 years, in 1978–1982. Cases were selected by electroencephalographic criteria. In the 134 children with 3 Hz spike-and-wave discharges, 97 (72.4%) had absences alone or in combination with generalized tonic-clonic seizures (grand mal): 56 had absences alone, 31 absences followed by grand mal, and 10 started with initial grand mal. Two distinct groups could be discerned: 1) childhood absence epilepsy: onset before the age of 12, with a quick response to therapy, little or no risk of grand mal, and a high remission rate; 2) juvenile absence epilepsy: onset at the age of 12 or later, a very high risk of grand mal, and usually a good response to therapy, but a high risk of relapses at withdrawal. This classification of absence epilepsy into subgroups may be useful for prognostic guidelines.     

Nonfebrile seizures after febrile convulsions: possible role of chronic cytomegalovirus infection

Twelve hundred children with convulsions when feverish were studied during a period of five years. Among them 52 subjects (4.33%) developed nonfebrile seizures after a period of eight months to five years from the first febrile convulsion (group A). Twenty-three children had neither afebrile seizures nor EEG abnormalities during the period of observation (group B). The two groups were comparable for age of the first febrile convulsion onset, sex, and socioeconomic status. None had risk factors for subsequent epilepsy or clinical signs of congenital cytomegalovirus infection. The isolation rate of CMV from urine was 53.84% in patients of group A, 26.09% in children of group B, and 26.83% in healthy control children. Twelve CMV-positive children from group A were followed for one to more than three years. In five of seven children with persisting EEG abnormalities, cytomegaloviruria was still present 13 to 41 months after the first isolation, whereas none of five patients with normal electroencephalograms had viruria after a comparable period. We found that CMV-positive children generally lacked cell-mediated immunity to the virus, whereas CMV-negative patients had positive reactions. Our data suggest a correlation between persistence of neurologic abnormalities and CMV excretion in children with nonfebrile seizures and CMV infection.     

Serum prolactin after cerebral and psychogenic seizures in childhood and adolescence--an additional useful method for differentiating the two forms of seizure]

Prolactin levels were measured immediately after the seizure in some, and 15 to 20 minutes later in all of 67 children aged between 6 months and 17 years. Values were determined after grand mal, complex partial and petit mal seizures and psychogen seizures. A more than 2 to 3 fold prolactin increase over the baseline value occurred almost always after grand mal and regularly after complex partial seizures. No hyperprolactinaemia was observed after petit mal seizures. Also after psychogenic seizures a rise in serum prolactin failed. The neurophysiological basis underlying this phenomenon is a decrease of gaba- und dopaminergic systems associated with the seizure. The described method is useful in the differential diagnosis of epileptogenic versus psychogenic seizures.     

Prolactin--a diagnostic aid in cerebral seizures

Prolactin concentration was measured 20 minutes after each seizure in 8 patients with grand mal, 2 patients with complex partial seizures and 5 patients with petit mal seizures. In the group of grand mal and complex partial seizures serum prolactin showed markedly increased levels. After petit mal seizures there was no change in serum prolactin concentration. The possible causes for changes in serum prolactin after a seizure in children are discussed.     

Epilepsy and mental retardation following febrile seizures in childhood

In an unselected group of children who were seen following an initial febrile convulsion, the frequency of subsequent afebrile seizures was 3.5% and of mental retardation 1%. The most common afebrile seizure type was generalized major (86%). About 3/4 of the children who developed afebrile seizures did so by three years and all by five years following the initial febrile seizure. The children with afebrile seizures differed from those without afebrile seizures in the frequency of neonatal abnormality, family history of mental retardation, focal initial febrile convulsions, and delay in psychomotor milestones before the initial febrile seizure. Only about 1/3 of the children who developed afebrile seizures ever had a recurrent febrile convulsion and none had complex recurrent febrile seizures. Half the children with mental retardation had histories of delay in psychomotor milestones prior to the initial febrile seizure, and no child with mental retardation had any seizure longer than five minutes. The administration of daily phenobarbital did not reduce the frequency of epilepsy, in spite of a significant reduction in the incidence of recurrent febrile seizures. There remains no evidence that the prevention of recurrent febrile convulsions significantly decreases the frequency of afebrile seizures or mental retardation.     

Epilepsy and Mental Retardation Following Febrile Seizures In Childhood

ABSTRACT. In an unselected group of children who were seen following an initial febrile convulsion, the frequency of subsequent afebrile seizures was 3.5% and of mental retardation 1%. The most common afebrile seizure type was generalized major (86%). About 3/4 of the children who developed afebrile seizures did so by three years and all by five years following the initial febrile seizure. The children with afebrile seizures differed from those without afebrile seizures in the frequency of neonatal abnormality, family history of mental retardation, focal initial febrile convulsions, and delay in psychomotor milestones before the initial febrile seizure. Only about 1/3 of the children who developed afebrile seizures ever had a recurrent febrile convulsion and none had complex recurrent febrile seizures. Half the children with mental retardation had histories of delay in psychomotor milestones prior to the initial febrile seizure, and no child with mental retardation had any seizure longer than five minutes. The administration of daily phenobarbital did not reduce the frequency of epilepsy, in spite of a significant reduction in the incidence of recurrent febrile seizures. There remains no evidence that the prevention of recurrent febrile convulsions significantly decreases the frequency of afebrile seizures or mental retardation.     

Lower degree of fever at the initial febrile convulsion is associated with increased risk of subsequent convulsions.

We studied 132 children admitted consecutively with their first febrile convulsion to assess whether the degree of fever at the onset of the convulsion can predict the risk of subsequent convulsions. The children studied were reviewed at least 2 years after the initial febrile convulsion to determine the number of children who had recurrences of febrile convulsions and/or afebrile convulsions. Children with body temperatures below 39 degrees C at the onset of their initial febrile convulsion (Group 1) were two and half times more likely to experience multiple convulsions within the same illness than those with body temperatures above 39 degrees C (Group 2). This occurred when the body temperature rose above that which had triggered the initial febrile convulsion. Children in Group 1 were also over three times more likely to experience recurrent febrile convulsion in subsequent illnesses than those in Group 2. As for subsequent development of afebrile convulsion or epilepsy, although the risk was low, it only occurred in Group 1. It is suggested that the known association between multiple convulsions, recurrent febrile convulsions and epilepsy may be due to the single predisposing factor of a low degree of fever at the onset of febrile convulsion. Each child with febrile convulsion may have his own threshold for eliciting a convulsion with fever; the lower this threshold is, the more likely are subsequent convulsions.     

Association between diabetes,severe hypoglycaemia,and electroencephalographic abnormalities.

Serial electroencephalographic recordings were made in 70 diabetic children and findings were related to age at electroencephalography and at diagnosis, duration of diabetes, daily insulin dose, long term metabolic control assessed by glycated haemoglobin A1 (HbA1) concentrations, and severe hypoglycaemic episodes. Abnormalities were found in 18 (26%) of diabetic children, and in only five (7%) of control subjects. There were no associations between electroencephalographic abnormalities and duration of diabetes, daily insulin dose, or HbA1 concentration. Diabetic children with electroencephalographic abnormalities were younger, had an earlier onset of diabetes and 21/34 (62%) of them had previously severe attacks of hypoglycaemia, whereas abnormalities were found in only 13/43 (30%) of diabetic children who had not had severe hypoglycaemia. All diabetic children with hypoglycaemic convulsions had permanent electroencephalographic abnormalities. The degree of metabolic control had no effect on the electroencephalographic findings during the early years of diabetes, but previous severe hypoglycaemia, young age, and early onset seem to be important risk factors for electroencephalographic abnormalities.     

Dental defects associated with neonatal symptomatic hypocalcaemia

12 children who had had hypocalcaemic convulsions between the 5th and 10th day of life were followed for 2 to 5 years. All 12 children had serious abnormalities of enamel of the canine and molar teeth resulting in progressive destruction of these teeth.     

Severe envenomation with convulsion following multiple bites by a common brown snake, Pseudonaja textilis

A case report of envenomation by a common brown snake, Pseudonaja textilis, in a 3.3 year old boy is presented. He suffered a brief grand mal convulsion 10 min after the bite which was shortly after removal of a compression bandage. A severe coagulopathy of the defibrination type required administration of five ampoules of brown snake monovalent antivenom (CSL). The association of envenomation by snakes and convulsions is discussed, as is the management of severe defibrination due to envenomation.     

Severe envenomation with convulsion following multiple bites by a common brown snake, Pseudonaja textilis

Abstract A case report of envenomation by a common brown snake, Pseudonaja textilis , in a 3.3 year old boy is presented. He suffered a brief grand mal convulsion 10 min after the bite which was shortly after removal of a compression bandage. A severe coagulopathy of the defibrination type required administration of five ampoules of brown snake monovalent antivenom (CSL). The association of envenomation by snakes and convulsions is discussed, as is the management of severe defibrination due to envenomation.     

The prognosis of convulsions during childhood shigellosis

We examined the long-term outcome in 111 children who had convulsions during shigellosis and were followed for 3–18 years after the incident. No deaths or persistent motor deficits occurred as sequellae. Poor coordination of fine hand movements were noted in 3.3% of the 92 children who had no pre-existing neurological abnormality. Only 1 child developed epilepsy by the age of 8 years. Of the children 15.7% had recurrent febrile seizures. The only risk factor identified for febrile seizures following convulsions in shigellosis was a previous history (P<0.01). These observations suggest that convulsions in shigellosis have a favourable prognosis, and do not necessitate long-term follow up.     

Late Electroencephalographic Findings after Recovery from Tuberculous Meningitis

Eighty five children and young persons cured of tuberculous meningitis were reexamined by EEG 6—12 1/2 years after the onset of the disease.
Moderate or severe EEG changes were seen in 50 cases, i.e. 59 per cent. The most frequent abnormality found was general dysrhythmia (29 cases) combined with focal abnormalities in 9 cases. Focal abnormalities were seen in the records of altogether 14 former patients. Of these, 9 had convulsions. Repeated EEG examinations were made in 15 cases. Seven of these showed improvement and 1 progression of the disturbances. Abnormal EEGs were seen most frequently among the youngest patients (0—3 years), among patients with severe symptoms on admission and among patients treated with cortisone.
Correlation was also found between EEG changes and other sequelae: convulsions, neurological disorders, mental sub-normality, behaviour disturbances and intracranial calcifications. There were no correlations between EEG abnormalities and late ophthalmological and otological sequelae.     

Prognosis of perinatal brain damage: a prospective study of a one year birth cohort of 12,000 children

The prognosis of perinatal brain damage was studied prospectively in a one year birth cohort of 12,000 children born in Northern Finland in 1966. Children were included in the study if they had an Apgar score of 0 at 1 min or less than 5 at 15 min, convulsions during the neonatal period, or a diagnosis of asphyxia, brain injury or intraventricular haemorrhage, but did not have CNS malformation, chromosomal aberrations or hereditary CNS degeneration. There were 233 children, 19.3 per thousand, of which 134, 58.0% were boys. Eighty-four, 36.4% died during the first 28 days and 7 children died before the age of 14 years, 6 of the latter group being handicapped. There were 44 children, 29.9% who had mental retardation, IQ less than 71, epilepsy or cerebral palsy. With regard to these children 13 had normal school performance, but there were 12 other children in the perinatal brain damage group who needed special education, two of them because of deafness. Perinatal brain damage accounted for 57.5% of all neonatal deaths, 30% of admissions to a special nursery and 12.5% of mental retardation (IQ less than 71), epilepsy and cerebral palsy at the age of 14.     

Gastroschisis and omphalocele: treatments and long-term outcomes

Between February 1994 and April 2004, we treated 40 children with gastroschisis and 26 children with omphalocele. We recorded the course of pregnancy, pre- and post-natal complications, delivery, operation, post-operative therapy, and long-term outcomes. Additionally, we conducted follow-up examinations of 37 of these 66 children (56%). We analysed their abdominal musculature, development, cosmetic result and quality of life. The median duration of follow-up was 6.3 years (range 1–10). In 35/40 children (88%) with gastroschisis and in 18/26 children (69%) with omphalocele, there had been prenatal diagnosis. The average maternal age of 23.9 years in the gastroschisis group was lower than in the omphalocele group (29.9 years). Delivery was by caesarean section in 93% of the gastroschisis group and 65% of the omphalocele group. Outcomes following vaginal delivery were no worse than those after caesarean section. Further, congenital abnormalities were shown in 28% of gastroschisis cases, and were limited to the gastrointestinal tract. Of the omphalocele cases 81% showed further abnormalities. Direct closure of the abdominal wall defect was possible in 31/40 (78%) of the gastroschisis cases and 15/26 (58%) of the omphalocele cases. Mortality in gastroschisis was nil; two children with omphalocele died (8%). Outcomes were better after primary closure than in stepwise reconstruction. Follow-up showed good results in all categories. Developmental delays were rapidly made up after treatment, and 75% of the children had no gastrointestinal problems, or suffered from these rarely. Almost all the children were of normal weight and height, and physical and intellectual development were delayed in only one third of the children. The surgical scar was rated as good or very good in about 80% of the cases. Except for those with severe defects, the children had good ratings for quality of life. Improvements in short-term results of gastroschisis and omphalocele treatment can be attributed to recent developments in prenatal diagnosis and the advancements of centralised perinatal care. Our long-term results clearly demonstrate that initial gastrointestinal problems and developmental delays were made up during the first two years of life. Prenatal counselling can now be more optimistic.     

Optic gliomas in children with neurofibromatosis type 1

To determine the frequency and natural history of tumors of the optic nerves and chiasm in patients with neurofibromatosis type 1, we obtained computed tomographic scans of 65 children who had no known visual or ocular abnormalities before their initial evaluation. Optic gliomas were detected in 10 children (15%). The median age of children with gliomas was 4.3 years (mean 5.8 years, range 9 months to 21 years). Three children (30%) had isolated, unilateral tumors, three (30%) had bilateral tumors, and four (40%) had involvement of the optic chiasm and of one or both nerves. Definite abnormalities of vision were found in only two children (20%). Five additional children were referred to the clinic after evaluation of ophthalmologic complaints led to the diagnosis of neurofibromatosis type 1: three had unilateral exophthalmos and two had plexiform neurofibromas of the eyelid with associated glaucoma. Ipsilateral optic gliomas were found in all five children; one child also had a contralateral tumor. Optic gliomas are commonly identified in young children with neurofibromatosis type 1 who have no ocular or visual abnormalities. Optic nerve gliomas may be associated with plexiform neurofibromas of the eyelid and glaucoma.     

A prospective incidence study of febrile convulsions

A prospective epidemiological study of febrile convulsions (FC) was performed during a 20-month period. 128 children with FCs were identified. The annual first attendance rate and annual incidence rate were 500/100,000 and 460/100,000 respectively, in the age group 0-4 years. The cumulative incidence was 4.1%. FCs were more common among boys, with a male to female ratio of 1.72 to 1. The median and mean age for first FC was for boys 16 and 19.9 months respectively, and for girls 18 and 19.7 months respectively. During an average follow-up period of 20 months, 18% had another FC and 10% had two or more FCs. 8.6% had an initial complicated FC. Four children had FCs within two weeks after being vaccinated by combined vaccine against morbilli, rubella and parotitis. Three of these had earlier had an FC.     

Ciliary abnormalities in respiratory disease.

One hundred and sixty seven children, ranging in age from 5 weeks to 16 years, with chronic upper or lower respiratory tract problems, or both, were investigated for ciliary dyskinesia. Abnormal ciliary function was found in 18 cases all of whom had chronic lower respiratory disease and most of whom also had upper respiratory problems. Fifteen of the 18 cases had reduced ciliary beat frequencies (less than 10 Hz) associated with dyskinesia and the other three showed apparent absence of ciliated cells. Of the 15 cases with reduced ciliary beat frequencies, ciliary ultrastructure was normal in seven cases but abnormal with missing dynein arms and occasional abnormalities of microtubular arrangement in eight. Respiratory symptoms in the perinatal period were more common in children with abnormal ciliary function and present in all those with ultrastructural abnormalities or absence of ciliated cells compared with 34 (26%) of 132 children, in whom symptoms were recorded, with normal ciliary function. This study would suggest that all children with unexplained chronic respiratory disease, in particular those with symptoms starting in the perinatal period, should be investigated for ciliary dyskinesia.     

A Prospective Incidence Study of Febrile Convulsions

ABSTRACT. A prospective epidemiological study of febrile convulsions (FC) was performed during a 20-month period. 128 children with FCs were identified. The annual first attendance rate and annual incidence rate were 500/100 000 and 460/100 000 respectively, in the age group 0–4 years. The cumulative incidence was 4.1%. FCs were more common among boys, with a male to female ratio of 1.72 to 1. The median and mean age for first FC was for boys 16 and 19.9 months respectively, and for girls 18 and 19.7 months respectively. During an average follow-up period of 20 months, 18% had another FC and 10% had two or more FCs. 8.6% had an initial complicated FC. Four children had FCs within two weeks after being vaccinated by combined vaccine against morbilli, rubella and parotitis. Three of these had earlier had an FC.