Postnatal cerebral infection leading to hemiplegic cerebral palsy: functional limitations and disability of 13 children in Sweden.

PURPOSE: To describe the motor function and disability of children with postnatal cerebral infection leading to hemiplegia, and to determine the severity of their motor disability. METHOD: The disablement process was used to describe these children. Participants included 13 children (7 girls and 6 boys, mean age 9.8 years). Traditional neurological assessment methods, together with the Movement Assessment Battery for Children and Paediatric Evaluation of Disability Inventory, were used. The children's non-hemiplegic sides were also tested. To determine severity of motor disability, new definitions were created that reflected the child's balance and fine-motor ability in relation to expected performance at his or her age. RESULTS: The children had involvement of the non-hemiplegic leg and arm in all but one case. Balance and hand function was impaired in all. Hypotonicity was present in 10 children, weakness of hip muscles in 9. There was great variation in grip function. Motor difficulties dominated during pre-school years, whereas the dominant problem of all school-age children was social and communication skills. Behavioural, communicative, and chewing and swallowing problems were common. CONCLUSIONS: A postnatal infectious aetiology influences function on both the hemiplegic and non-hemiplegic side. In school-age children, social skills and communication difficulties are more pronounced than motor problems. The results of this study encourage the use of methods standardized for age where force dynamics and quality of movement are assessed.

Postnatal cerebral infection leading to hemiplegic cerebral palsy: clinical description of 13 children in Stockholm, Sweden.

PURPOSE: The aim of this research was to estimate the prevalence of postnatal cerebral infection leading to hemiplegic cerebral palsy (CP) in Stockholm County and to describe the motor impairments, associated impairments and involvement of the non hemiplegic side. METHOD: Children with hemiplegic CP subsequent to a cerebral infection in the perinatal period up to the age of seven years were identified. The assessments of child psychologists and speech therapists and EEG-studies, CT-scan or MRI of the brain were extracted from the children's files. Thirteen children, with a mean age of 9.5 years, participated. The prevalence was 0.03/1000. RESULTS: Nine children suffered from mental retardation, seven took antiepileptic drugs and six had bilateral radiological anomalies. The non-hemiplegic side was involved in six of the children. CONCLUSION: Cerebral infection at an early age can cause hemiplegic CP with a high frequency of associated impairments and with involvement of the non-hemiplegic side. The infectious origin probably gives rise to a more widespread brain injury.

Participation in age-related activities and influence of cultural factors--comments from youth and parents of children with postnatal post infectious hemiplegia in Stockholm, Sweden.

PURPOSE: To investigate whether children with postnatal post-infectious hemiplegic cerebral palsy, and their parents, felt that participation in activities typical for the child's age and gender was affected. To identify factors, intra- and extra-personal that influenced disability with emphasis on factors related to immigration. METHOD: Interviews with six youths and 15 caregivers regarding the child's ability to participate in age-related activities, the consequences of disability in their home country compared to Sweden, and whether immigration influenced being a parent to a disabled child. RESULTS: All reported large difficulties participating in age-related activities. Positive and negative differences in participation in different cultures were described. War, culture, eating habits, hygiene habits, and school could differ and create difficulties when in Sweden. Language skills and intra personal factors influenced information gathering and participation in parental activities. CONCLUSIONS: Surprisingly large difficulties in participation may separate those with a postnatal post-infectious aetiology from hemiplegia of other aetiologies. High degrees of additional impairments may influence participation more than the physical disability. Language skills and cultural factors influence ability to seek and gain information. A mutual process where both Swedes and immigrants know traditions of care for disabled in both places may create understanding and improved dialogue.

Neurovesical dysfunction in children after surgery for high or intermediate anorectal malformations.

AIM: To evaluate problems with bladder dysfunction in patients operated on for imperforate anus. METHODS: All patients (11M, 25F) with high or intermediate imperforate anus seen in this institution between 1987 and 1997 were subjected to the investigation. They had all undergone a posterior sagittal anorectoplasty procedure with the aim of preserving as much as possible of the fistula. Hospital charts were reviewed. A detailed and structured interview regarding micturition habits was performed. If the results of this were abnormal the patients were also subjected to flow registration, measurements of residual urinary volume and in some cases also cystometry. RESULTS: Micturition habits were normal in 30 out of 36 children. The remaining six, considered to have neurovesical dysfunction (NVD), had difficulties in emptying their bladder and exhibited pathological residual volumes. Two were boys with rectourethral fistulae, two were girls with cloacal malformations and two were girls with rectovestibular fistulae. Four out of six children with NVD had sacral anomalies. CONCLUSION: NVD may occur even in the absence of sacral anomalies. A 4 h micturition observation is recommended in all newborns with intermediate or high anorectal anomalies to recognize the occurrence of NVD at an early stage.

Reduction of epileptiform activity in response to low-dose clonazepam in children with epilepsy: a randomized double-blind study.

PURPOSE: To evaluate the effect of low-dose clonazepam (CZP) on the amount of epileptiform activity in children with focal and generalized epilepsy. METHODS: In a single-blind pilot study, followed by a double-blind, placebo-controlled, randomized, crossover study, 15 children with epilepsy were evaluated by using 24-h long-term EEG recordings during baseline days and days after injections of placebo and CZP. The drug was given as a single i.m. injection of 0.02 mg/kg BW. Blood samples were obtained regularly for analysis of plasma concentrations of CZP. The number of epileptiform discharges was determined during corresponding periods with the individual child in the same state of alertness, the same real time of day, and with concomitant antiepileptic drugs (AEDs) unchanged. RESULTS: In the double-blind study, low-dose CZP produced a highly significant (p = 0.0015) decrease in the amount of epileptiform activity (mean, -69% vs. placebo, -2%) obtained during periods when median plasma concentrations ranged from 18 to <14 nM. The maximal plasma level (median, 24 nM) was reached before the start of the analysis periods. The pilot study showed reductions of epileptiform discharges within the same range as the double-blind study. In the children with daily seizures, a parallel decrease in seizures and the number of epileptiform discharges was seen after the administration of CZP. CONCLUSIONS: Our data demonstrate a significant reduction of epileptiform discharges on long-term EEGs after a single low dose of CZP with concomitant low plasma levels, which were considerably lower than the doses and plasma levels usually recommended. A concomitant reduction of seizures also was seen.

Variability of epileptiform activity in long-term EEGs with short and long intervals in children with epilepsy.

OBJECTIVE: To evaluate the impact of the time factor on the amount of epileptiform activity in long-term EEG recordings in children with epilepsy. METHODS: Ten children with epilepsy of different types underwent three 24 h EEG examinations during two consecutive days and with a month's interval. The number of epileptiform discharges during selected corresponding periods of time was counted. RESULTS: The number of epileptiform discharges on three repeated examination days showed no significant difference (ANOVA P = 0.88) as intraindividual increases and decreases on different days counterbalanced each other within the group. However the standard deviations of the relative changes were larger between recordings with a month's interval compared to those for consecutive days (86% and 33%). The mean magnitude of change was 55% between days separated by a month compared to 24% on consecutive days. The difference was non-significant but showed a trend towards larger changes with a longer interval (P = 0.07). CONCLUSIONS: The variability of epileptiform activity was larger when the interval between recordings was 1 month compared to consecutive days. The magnitude of the relative changes between intervals of 1 and 30 days showed a trend towards a difference although not statistically significant. When evaluating repeated long-term EEGs in relation to therapy in children, these variations should be considered.

Vagus nerve stimulation in 16 children with refractory epilepsy.

PURPOSE: Vagus nerve stimulation (VNS) has been reported to produce >90% reduction in the number of seizures in children with intractable epilepsy. These encouraging results need confirmation. METHODS: Sixteen children, 10 boys and 6 girls aged 4-19 years, were treated with VNS (Cyberonics, Webster, TX, U.S.A.) for 12-24 months. Seizure frequency, seizure severity, changes in quality of life (QOL: visual analogue scale), and side effects were recorded. Eight children had partial and 8 had generalized seizures; 4 of the latter had Lennox-Gastaut syndrome (LGS). RESULTS: During the tenth to twelfth month of VNS, 6 of 16 children experienced > or =50% reduction in seizure frequency. One girl became seizure-free. Seizure severity showed an average decrease in the score from 15 to 11. After 10 months of treatment, QOL was estimated to have improved > or =50% in 6 of 16 children. Reduction in seizure frequency, decreased seizure severity, and reported improvement in QOL did not entirely coincide. Six children experienced hoarseness, 1 had neck pain, 2 had hypersalivation, 2 experienced tiredness, 2 had aspiration episodes during liquid intake, and 6 had electrical transmission problems; in 4 the problem has been surgically corrected. Five stimulators were turned off due to lack of efficacy. CONCLUSIONS: Six of 16 children with refractory epilepsy treated with VNS improved, with a reduction not only in seizure frequency but also in seizure severity and in QOL.

Follow-up of long-time treatment with intravesical oxybutynin for neurogenic bladder in children.

OBJECTIVE: Anticholinergic treatment for the hyperreflexic neurogenic bladder in childhood is an established method, together with clean intermittent catheterization (CIC), to promote continence and protect the upper urinary tract from deterioration. Recently, the use of oxybutynin, a compound with anticholinergic, smooth muscle relaxant and local anesthetic effects, has become widely used with both oral and intravesical administration. METHOD: In this study we report 39 children with myelodysplasia, neurogenic bladder disturbance with detrusor hyperreflexia and/or high bladder pressure treated with CIC to which intravesical oxybutynin 0.1 mg/kg twice daily was added and administered as a sterile pharmacy-produced solution. The follow-up period was 0.66-5 years (mean 2.25). RESULTS: Continence was clearly promoted and urodynamic parameters improved whereas an increased occurrence of asymptomatic bacteriuria and lower urinary tract infections was noted. Compliance was good, adverse reactions rare, and in some cases vesicoureteral reflux (VUR) resolved. Also infants and very young children were treated without complications. CONCLUSIONS: Intravesical oxybutynin is effective to diminish bladder pathophysiology and promote continence in this patient group and is also well tolerated. Attention should be paid to the occurrence of urinary tract infections and VUR may resolve.

Pharmacokinetics and effects of intravesical oxybutynin on the paediatric neurogenic bladder.

OBJECTIVE: To evaluate the pharmacokinetics of both oxybutynin and its active metabolite, N-desethyl oxybutynin (NDO), when the drug is instilled directly into the bladder in children with myelodysplasia and neurogenic bladder disturbance, in whom it may improve continence and decrease the risk of upper urinary tract deterioration. PATIENTS AND METHODS: The study comprised 13 children (five girls and eight boys, mean age 9.3 years, range 1-15) with neurogenic bladders who were treated using clean intermittent catheterization and intravesical instillation of a sterile, pharmacy-produced solution of oxybutynin. Steady-state minimum plasma levels of oxybutynin and NDO, together with their effect on urodynamic variables and incontinence, were evaluated. The dose (0.04-0.17 mg/kg, mean 0.1 mg/kg) was instilled twice daily. RESULTS: The effects of the drug on incontinence and urodynamic variables were pronounced, improving both in most cases. Minimum plasma levels were < 0.3-7.2 ng/mL for oxybutynin and 0.8-14 ng/mL for NDO. The ratio of oxybutynin to NDO was 0.29-0.83 (mean 0.47). CONCLUSION: There was no clear relationship between minimum plasma levels of the drug or NDO and their clinical effects; however, the combination of oxybutynin and NDO seemed to be more strongly correlated with the clinical effects.

Effect of phenylpropanolamine on incontinence in children with neurogenic bladders. A double-blind crossover study.

In children with myelodysplasia and a low-level spinal cord lesion (low lumbar-sacral), detrusor hyperactivity together with dyssynergic urethral function forms the main pathophysiological basis for incontinence. Pharmacological treatment of incontinence due to neurogenic bladder dysfunction has been tried, mainly with anticholinergics and alpha-adrenoceptor antagonists. In this study, the effects of the alpha-adrenoceptor agonist phenylpropanolamine on urodynamic parameters and incontinence were investigated in 10 patients. Effects on incontinence were evaluated in a double-blind crossover trial. Plasma concentrations of phenylpropanolamine were measured by means of gas chromatography-mass spectrometry. Phenylpropanolamine reduced detrusor hyperactivity and improved continence, but the effect was not so pronounced as to make the patients continent.

Alpha-adrenoceptor function before and after chemical sympathectomy in human and feline detrusor muscles.

Isolated bladder segments from man and cat were treated with 6-hydroxydopamine (6-OHDA) in vitro. Chemical sympathectomy was evaluated with fluorescence microscopy and found to be very similar to the effect of 6-OHDA administered in vivo to cats. Isometric smooth muscle contractile responses were induced by field stimulation (FS). The amplitude of the responses increased after denervation. The effects of alpha-adrenoceptor agonists and antagonists on the FS-induced contractile responses were compared before and after treatment with 6-OHDA. The reduction in the contractile responses after the addition of noradrenaline to the feline bladder strips was more pronounced after treatment. Phentolamine induced an increase in contractile responses before treatment, an effect not seen afterwards in human bladder strips but which persisted in feline bladder strips. Selective alpha-adrenoceptor agonists did not alter the contractile responses in denervated strips. It is suggested that the function of the alpha-adrenoceptors in the detrusor is to inhibit neuronally mediated contractile responses of smooth muscle.

Receptor function studies in specimens from the proximal human urethra obtained by transurethral resection.

During transurethral resection (TUR) for prostatic hyperplasia, specimens were taken from the proximal urethra. Muscle strips thus obtained were mounted in an organ bath and muscle contraction was induced by adding increasing concentrations of noradrenaline (NA), methoxamine (alpha 1-agonist) and clonidine (alpha 2-agonist). NA and methoxamine induced a dose-dependent muscle contraction, but clonidine had no effect. The influence of prazosin (alpha 1-antagonist) and yohimbine (alpha 2-antagonist) on the NA-induced muscle contraction was also evaluated. Both antagonists had an inhibitory effect, which was much more potent with prazosin. The specimens taken during TUR were found to be suitable for in vitro receptor function studies. The alpha-adrenergic receptor function in the proximal human urethra was found to be mainly of the alpha-type.

Influence of adrenergic agonists and antagonists on urethral pressure, bladder pressure and detrusor hyperactivity in children with myelodysplasia.

In children with myelodysplasia and a low lumbar or sacral level of spinal cord lesions detrusor hyperactivity with pressure fluctuations is an almost constant phenomenon contributing to incontinence. In thirteen children with this type of dysfunction the effect of adrenergic agonists and antagonists on bladder and urethral pressures were studied by means of intravesical and urethral pressure recordings during the normal bladder-filling phase. Intravenous infusion of noradrenaline during the bladder-filling phase slightly reduced detrusor hyperactivity and the urethral pressure was increased. Following i.m. injection of the alpha-adrenergic antagonist phentolamine both the intravesical and proximal urethral pressures were reduced to about the same extent and the detrusor hyperactivity decreased. It is concluded that noradrenaline mainly changed urethral pressure while alpha-adrenergic blockade caused decreased tone in both the detrusor and the urethra as well as decreased detrusor hyperactivity.

The effect of noradrenaline on the contractile response of the urinary bladder. An in vitro study in man and cat.

In this study, bladder muscle strips from the detrusor of man and cat were used to evaluate the modulating effects of adrenergic agonist and antagonists on the field stimulation induced contractile response. Noradrenaline (NA) inhibited and phentolamine enhanced the contraction in a dose-dependent manner. Propranolol did not influence the field stimulation response. When a study of the combined effect of adrenergic drug influence was performed, the NA-induced inhibition was partly reversed by propranolol but a further increase of the contractile response compared to the control was seen, when phentolamine was added. No species differences were found. The conclusion drawn from these results is, that the inhibiting effect of NA on the contractile response is mediated via alpha- and beta-adrenergic receptors. The former could be located on the short parasympathetic intramural neurons while the latter probably are located on muscle cells.