Slow prepubertal linear growth but early pubertal growth spurt in patients with shunted hydrocephalus.

OBJECTIVE: To evaluate growth and to compare anthropometric measures and the degree of physical maturation in children with shunted hydrocephalus with those in healthy children. METHODS: One hundred fourteen patients (62 male) and 73 healthy subjects (38 male) 5 to 20 years of age were analyzed for growth data and current auxology, stage of puberty, and bone age. RESULTS: Boys with hydrocephalus were shorter than control boys during their first 8 years of age, and no catch-up growth was observed until puberty. Girls with hydrocephalus were of the same size at birth as the control girls, but their linear growth retarded during the first years of life, leading to reduced relative height between the age of 5 to 8 years. The pubertal growth spurt occurred earlier in boys with hydrocephalus (age at midgrowth spurt, 12.1 vs 13.3 years), and a similar trend was seen in girls (10.0 vs 10.7 years). The final height was again reduced, especially in boys. Patients with hydrocephalus were more obese than control subjects, girls more often than boys. Relative bone age was retarded in prepubertal (-0.42 vs 0.32 SD) and accelerated in pubertal patients (0.54 vs -0.19 SD). CONCLUSIONS: Children with hydrocephalus experience slow linear growth in prepuberty, but they have an earlier adolescent growth spurt. Together these factors result in a reduced final height. An increase in relative weight emerges in the preadolescent period, and this phenomenon is accentuated after puberty, leading to an increased prevalence of obesity.

Failure of strychnine treatment during the neonatal period in three Finnish children with nonketotic hyperglycinemia.

Three Finnish infants with a severe neonatal-onset-type of nonketotic hyperglycinemia were treated with strychnine nitrate in a daily dosage of 0.2 to 0.9 mg/kg, given orally in four doses. In order to lower the plasma and CSF-glycine concentrations concomitant exchange transfusions (200 to 300 ml/kg of heparinized blood) were carried out in two of these infants. Although the strychnine therapy was started at ages 15, 40, and 62 hours, the strychnine produced no clinical effect, and the exchange transfusion caused only a transient decrease in the plasma glycine level. Despite treatment, the clinical course was the same as in the majority of children with the severe form of the disease--all died within the first ten days of life. Impressive effects of strychnine treatment initiated in two infants at ages 5 and 6 1/2 months, and given in addition to sodium benzoate and anticonvulsants, have been reported. These cases, however, probably represent a less severe type of nonketotic hyperglycinemia. Nevertheless, the therapeutic failure in the present cases probably indicates that strychnine treatment does not solve the therapeutic problems of severe forms of NKH.

Pituitary function in children with hydrocephalus before and after the first shunting operation.

OBJECTIVE: Children with shunted hydrocephalus experience slow linear growth in prepuberty, accelerated pubertal maturation and a reduced final height. A substantial proportion of these patients have a poor growth hormone (GH) response to stimulation and reduced pituitary volume. The basic mechanisms behind these phenomena are still unknown, but one can hypothesize that an unphysiological intracranial pressure (ICP) may be involved. This study was undertaken to investigate the effect of increased ICP on pituitary function. DESIGN: Twenty-one children (nine males) aged 4 months to 15 years were evaluated for pituitary function before and after their first shunting operation. METHODS: A clinical examination was performed, bone age was determined and a combined pituitary stimulation test was performed to evaluate GH, luteinizing hormone, follicle-stimulating hormone, cortisol, thyrotropin and prolactin secretion. RESULTS: GH concentrations were significantly higher 10 and 15 min before the operation (P=0.04 and P=0.03 respectively) than after it. The basal levels of insulin-like growth factor-I (IGF-I) tended to be higher before the operation than afterwards and those of its binding protein-3 (IGFBP-3) were significantly so (P<0.01). CONCLUSIONS: The higher GH response to GH releasing hormone and circulating IGFBP-3 levels in children with hydrocephalus before compared with after their first shunting operation raise the possibility that the reduced GH secretion and retarded linear growth observed in children with shunted hydrocephalus may be a consequence of decreased ICP and/or the lack of physiological pressure variations.

Ultrasonic evaluation of the Dandy-Walker syndrome.

Antenatal and postnatal ultrasonic findings of a case of cystic dilatation of the fourth cerebral ventricle (the Dandy-Walker syndrome) are presented. The triangular enlargement of the ventricle visualized ultrasonically was associated with mild widening of the lateral ventricles, and the finding was established postnatally by computer-assisted x-ray tomography.

Electroencephalographic findings in hydrocephalic children prior to initial shunting.

The EEG recordings of 105 hydrocephalic children with proven ventriculomegaly and increased intracranial pressure were studied prior to initial shunt treatment. Only 2 patients had a normal EEG and 7 had only abnormal dominant activity. Paroxysmal slow-wave activity, generalized or posterior, was present in 37 (35% of the patients) recordings and focal slow waves in 28 patients (27%), these mostly posterior. All types of slow-wave activities increased with age. Focal attenuation was very common under 1 year of age (in 41% of patients of this age group), but after this age only 4 cases (10%) were recorded. Spike- or sharp-wave activity was recorded focally or generally in 45 (43%) of the children. The prevalence of spikes and sharp waves correlated negatively with increasing age: only generalized spikes were usual after 7 years of age. No significant differences were found in the appearance of focal findings between hemispheres. According to the present study, the EEG is abnormal in more than 98% of children with proven hydrocephalus before shunting. The observed abnormalities are described in detail in order to serve as reference material in the follow-up of hydrocephalic children.

Initial electroencephalographic findings in children with acute lymphoblastic leukaemia.

Electroencephalography (EEG) was performed on 66 children with acute lymphoblastic leukaemia, 45 before treatment and 21 during the first 5 days of chemotherapy. The patients, aged 7 months to 16 years, 33 boys and 33 girls, had been admitted to the Department of Paediatrics, University of Oulu, between March 1976 and January 1987. The EEG findings were compared with those in 66 age and sex-matched control children chosen at random from the local population. The patients had significantly more frequent and more severe disturbances in background activity (p less than 0.001) than the controls and increased slow waves in the occipital (p less than 0.001) and temporal regions (p less than 0.01). The patients who had received chemotherapy before the EEG recording had EEG disturbances significantly more frequently than the other patients (p less than 0.01), but the latter still had EEG abnormalities significantly more frequently than their matched controls, although they did not have severe changes (grade 3). The results suggest that chemotherapy increases EEG changes during the early days of induction therapy and possibly induces long-term disturbances in brain function. The associations between EEG changes and clinical findings were also analysed and the results show that a long duration of leukaemic symptoms or an aggressive disease may lead to EEG abnormalities.

Long-term prognosis for children with shunted hydrocephalus.

We reviewed the previous medical history and the social status of all patients of Oulu University Central Hospital who had had in the age range 16-26 years shunted hydrocephalus (HC) during childhood. Of 42 patients selected 7 had died and another 5 had been institutionalized for severe mental handicap. Shunts had been changed a total of 103 times in 29 patients still living. The most common reason for a reoperation was blockage. Half of the patients re-examined showed neurological abnormalities or epilepsy. Both the verbal and the nonverbal IQ of the patients remained weak to average. Even though the patients' medical prognosis was fair, their social maturation did not keep up with their physical abilities. One-third were receiving or had received vocational training, but only a few were working. Up to one-quarter of the patients with shunted HC were at home without any meaningful work activities.

Central nervous system involvement in severe arterial hypertension of childhood.

The case histories of 125 children with hypertension and no apparent primary CNS disease were analyzed for neurological symptoms or complications. Eleven children had neurological symptoms of high blood pressure. In only one of these patients was the diagnosis of arterial hypertension made before the observation of the neurological findings. The symptoms were severe headache in eight children, convulsions and coma in four, hemiplegia in two, and impaired vision and apraxia in one child. Symptomatology was rapidly reversed by antihypertensive treatment in four children, while six had long-term stigmata and one child died in hypertensive crisis. Because elevated arterial pressure can cause severe neurological disease, routine blood pressure measurement in children--especially those with neurological symptomatology--is stressed.

Valvography in the assessment of hydrocephalus shunt function in children.

Assessment of hydrocephalus shunt dysfunction, especially when partial, causes severe differential diagnostic problems. Ordinary computer assisted tomography gives only indirect information about shunt dynamics and the estimation of intraventricular pressure is vague. In a series of 50 valvographies, this examination proved to be especially valuable in the diagnosis of partial obstruction of the distal catheter. Valvography is also superior to other forms of examination in the localization of x-ray negative catheter types. In the slit ventricle syndrome valvography will reveal the position and function of ventricular catheters, which by other means would be impossible preoperatively.

Electroencephalographic findings and epilepsy in the slit ventricle syndrome of shunt-treated hydrocephalic children.

Overdrainage of the cerebrospinal fluid (CSF) and collapse of the ventricles, slit ventricles (SLV), can cause clinical symptoms and result in the slit ventricle syndrome (SLVS). The EEG changes and the frequency and type of epilepsy in patients with SLV was analysed from a material of 113 shunt-treated hydrocephalic children. During the follow-up time (mean 8.9 years), 63 patients (56%) had developed SLV. The age at initial shunting was significantly lower (1.2 years) in patients who developed SLV than for those who did not (2.7 years). After initial shunting generalized spike and sharp wave activity (SWA) developed more frequently in patients who developed SLV (81%) than in those who did not (54%). Severe generalized SWA developed almost entirely in patients in the SLV group. This severe generalized SWA disappeared from the EEG in patients after treatment of the SLVS. Epileptic seizures appeared after initial shunting in 44% of patients in the SLV group but in only 6% of the non-SLV group. Treatment of the SLVS decreased the frequency of epilepsy to a level corresponding with the non-SLV group. Repeated EEG evaluation of shunt-treated hydrocephalic children is a valuable aid in follow-up. If EEG abnormality appears after initial shunting, especially SWA, shunt malfunction and overdrainage of the CSF should be suspected.

Do neurological disorders in childhood pose a risk for mental health in young adulthood?

To assess whether juvenile-onset epilepsy or motor disability is complicated by an increased number of mental health disorders or experience of psychosomatic symptoms in young adulthood, we studied 81 subjects with epilepsy and 52 with motor disabilities at the age of 19 to 25 years and compared them with 211 controls. The main diagnostic tool, the Present State Examination, was administered to those attending the interviews in person who were of normal intelligence; there were 62, 38, and 123 subjects in the three categories, respectively. Compared with the controls, the subjects with epilepsy showed an equal prevalence of psychiatric disorders whereas those with motor disabilities had a significantly higher prevalence, particularly of depressive disorders. The reported prevalence of psychosomatic symptoms confirmed this main result. Psychological illness affected everyday life of two out of five subjects with motor disabilities, but only half of those in the other two groups. It is concluded that motor disability since childhood, but not epilepsy, could be a factor that increases susceptibility to psychiatric morbidity, especially depression, and causes a large number of psychosomatic symptoms. The results challenge staff of clinics working with such adolescents to find individual approaches in preventing the negative influence of psychological disorders on social life.

Efficacy of five days' barbiturate anesthesia in the treatment of intractable epilepsies in children.

PURPOSE: To analyze the efficacy of barbiturate anesthesia in the treatment of intractable epilepsies in childhood. METHODS: Anesthesia for 4-5 days with thiopentone sodium was used to treat children with intractable epilepsy in the Department of Pediatrics, Oulu, Finland, from November 1980 through December 1995. The number of epileptic seizures, the number and dosage of antiepileptic drugs (AEDs), and psychomotor development before and after anesthesia were compared. RESULTS: Fifty-four children with intractable epilepsy were treated with barbiturate anesthesia. Twenty-four children had infantile spasms; 22, Lennox-Gastaut syndrome; seven, complex partial epilepsy; and one, myoclonic epilepsy. Twenty-four (44.4%) children had complications during the anesthesia. The seizures recurred in 53 of the 54 patients in a median time of 12 days after the anesthesia. In 42 (78%) children, the seizure frequency returned to a level equal to or higher than that before the anesthesia in a median time of 211 days. The number of AEDs was significantly greater after than before the anesthesia (6.33 vs. 4.8; p < 0.001). Seventeen (32.5%) children were treated surgically after the anesthesia. CONCLUSIONS: Although the seizures are eliminated or the seizure frequency decreases for a short period after the barbiturate anesthesia, the anesthesia does not change the long-term outcome and is therefore inefficient in the treatment of childhood intractable epilepsies.

Epilepsy in hydrocephalic children.

Epilepsy and epileptogenic activity in EEGs were studied in 168 shunt treated hydrocephalic (HC) children, the mean age at first operation 1.62 years (SD 1.87). 80 patients (47.6%) suffered from epileptic seizures during the follow-up period (mean 8.9 years). The epileptic seizures appeared before the initial shunting, and after the first shunt implantation in 43 (25.6%). There was no correlation between epilepsy and the aetiology of HC, number of shunt revisions, or shunt infections. Sixteen patients suffered from seizures during the neonatal period. Generalized spike and wave activity (SWA) was seen in EEG in eight out of these, and only one was seizure-free at the end of the follow-up period. All eight patients with epileptic seizures during the neonatal period without generalized SWA in EEG were seizure-free, however. SWA was seen in the first EEG prior to shunting in 75/168 patients (44.6%). All those patients who did not receive prophylactic medication, developed epileptic seizures, whereas 68.1% of those who received prophylactic anticonvulsive medication remained free of seizures. Partial epilepsy after shunting manifested itself in 15 patients, but this did not correlate with the side of the shunt or with the side of the SWA in the EEG. Slit ventricles (SLV) developed in 75 patients during the follow-up period, while the ventricles remained normal or dilated in 66 cases (27 patients had no CT follow-up). Epilepsy manifested itself in 8 out of these 141 patients (2 SLV, 6 non-SLV) during the first postoperative year, and in 29 patients in the SLV group and one in the non-SLV group at some time after the first postoperative year.

Social outcome of handicapped children as adults.

The social status of 52 disabled young adults (29 men, 23 women) was reviewed at the age of 19 to 25 years, and compared with that of 209 age-matched controls. 40 had successfully completed elementary school and 10 had completed high-school. 28 had gone on to basic further education and 11 had progressed to more advanced levels. 35 per cent of the study group and 11 per cent of the controls had had no further education. Half of the study group were receiving disability benefit and 12 had no occupation outside the home. There was little difference in present employment status between groups, but the study group had experienced twice as much unemployment as controls. Leaving the parents' home and sexual experience were delayed among the study group. The results indicate the need for more practical support for these adolescents and their parents.

Reduced levels of growth hormone, insulin-like growth factor-I and binding protein-3 in patients with shunted hydrocephalus.

OBJECTIVE: Children with hydrocephalus are characterised by slow linear growth in prepuberty, accelerated physical maturation during puberty, and reduced final height. We aimed to study the possible roles of growth hormone, insulin-like growth factor-I (IGF-I), and IGF binding protein-3 (IGFBP-3) in this growth pattern. STUDY DESIGN: One hundred and fourteen patients with shunted hydrocephalus (62 males) aged 5 to 20 years, of whom 17 had spina bifida (six males), and 73 healthy controls (38 males) were studied. Anthropometric measures, body mass index, and body fat mass were assessed and the stage of puberty was determined. Serum growth hormone and plasma IGF-I and IGFBP-3 concentrations were measured. RESULTS: The patients comprised 44 (26 males) who were prepubertal and 70 (36 males) pubertal or postpubertal, while 32 of the controls (19 males) were prepubertal and 41 (19 males) pubertal or postpubertal. The prepubertal children with hydrocephalus had lower IGF-I (p = 0.002) and IGFBP-3 concentrations (p < 0.001) than the controls, and the pubertal children had four times lower basal growth hormone concentrations (p < 0.001). There was a correlation between height SD score and IGF-I levels in the total patient population (r = 0.23; p = 0.01). Peripheral IGF-I concentrations peaked at pubertal stages 2-3 in the female patients and at stage 4 in the controls. The prepubertal patients on antiepileptic treatment, carbamazepine in most cases (73%), had higher IGF-I (p = 0.01) and IGFBP-3 concentrations (p = 0.03) than those who had never been treated with antiepileptic drugs, but still lower IGFBP-3 levels than the controls (p = 0.01). CONCLUSION: Based on these findings, it can be concluded that reduced growth hormone secretion may contribute to the pattern of slow linear growth and reduced final height observed in these patients.

Antipyretic effect and plasma concentrations of rectal acetaminophen and diazepam in children.

Plasma levels of acetaminophen (paracetamol) and diazepam were measured in 9 children by gas chromatography after administering these drugs simultaneously in separate suppositories. The antipyretic effects of oral and rectal acetaminophen-diazepam combinations were also studied and compared with that of oral or rectal acetaminophen alone. Diazepam at a dose of 0.2 mg/kg did not increase the antipyretic action of acetaminophen. Acetaminophen and diazepam seemed to be well absorbed from the rectal suppositories, the maximal plasma concentration of diazepam after a rectal dose of 0.5 mg/kg just reaching the assumed anticonvulsant level in about 2 hr. In light of this study, an acetaminophen-diazepam combination in separate suppositories may be suitable for the prevention of recurrent febrile convulsions in susceptible children, but its practical value and efficacy require evaluation in clinical experiments.

Accelerated pubertal development in patients with shunted hydrocephalus.

OBJECTIVE: To evaluate pubertal development and peripheral concentrations of gonadotrophins and sex hormones in children with shunted hydrocephalus compared with healthy controls. STUDY DESIGN: 114 patients (52 females, 62 males) and 73 healthy controls (35 females, 38 males) aged 5 to 20 years were analysed for stage of puberty, age at menarche, testicular volume, basal serum follicle stimulating hormone (FSH), luteinising hormone (LH), sex hormone binding globulin (SHBG), testosterone and oestradiol concentrations, and free androgen index. RESULTS: Male gonadal and male and female pubic hair development occurred significantly earlier in the patients than in the controls. The mean age at menarche was significantly lower in the female patients than in their controls (11.7 v 13.2 years; p < 0.001), and lower than it had been for their mothers (v 13.1 years; p < 0.001). Relative testicular volume was higher in the male patients than in their controls (1.2 standard deviation score (SDS) v 0.2 SDS; p < 0.001). The prepubertal patients had higher basal LH (0.13 U/l v 0.08 U/l; p < 0.001) and SHBG (132.3 nmol/l v 109.1 nmol/l; p < 0.01) than the controls. Both the prepubertal and pubertal females had significantly higher basal FSH than their controls (1.57 U/l v 1.03 U/l; p < 0.05, and 4.0 U/l v 2.9 U/l; p < 0.01, respectively). CONCLUSIONS: Hydrocephalic children experience accelerated pubertal maturation, reflected in a younger age at menarche in females and an increased testicular volume in males. This may be because of enhanced gonadotrophin secretion, possibly resulting from unphysiological variations in intracranial pressure.

Psychosocial outcome of young adults with epilepsy in childhood.

OBJECTIVE: To evaluate social maturation and psychiatric morbidity in young adults treated for epilepsy during their childhood. METHODS: Eighty one young adults (43 women, 38 men, mean age 22.3 years) with epilepsy during their childhood were interviewed about their social development and psychiatric symptoms. The results were compared with those from 211 randomly selected controls (106 women, 105 men, mean age 23.2 years). RESULTS: Compared with the controls the patients had more often not succeeded in passing the normal comprehensive school (20% v 2%) or had left school at the secondary level (53% v 46%) and remained without any vocational education (27% v 11%). There was no significant difference in the employment status of those with work between the patients and the controls. The patients were significantly more often labelled with poor social maturation and dependent lifestyle factors such as living with their parents. However, risk analysis showed that neither the disease itself nor antiepileptic medication were significant predisposing factors for poor social adjustment but low or borderline mental capacity or learning disabilities relating to epilepsy were. Psychiatric morbidity was similar in both groups. CONCLUSIONS: The social handicap found in a certain group of young adults with epilepsy during childhood is largely associated with neurological and cognitive impairments other than epilepsy itself. With the present mode of treatment epilepsy itself does not seem to disturb adolescent social and psychological development.