José Luiz Dias Gherpelli

Discontinuing Medication in Epileptic Children: A Study of Risk Factors Related to Recurrence

Summary: We studied 70 children who had experienced at least two seizures before age 12 years, excluding febrile seizures, neonatal seizures, or seizures occurring during a metabolic, or infectious insult to the central nervous system (CNS) and who had been seizure free for at least 2 years. Twenty children (28.5%) experienced a recurrence, 75% during antiepileptic (AED) drug discontinuation or <6 months after discontinuation. Risk factors statistically related to seizure recurrence were >10 seizures before seizure control, an abnormal EEG in the year before AED discontinuation, presence of focal neurologic signs and/or mental retardation, and presence of a mixed seizure pattern. Fourteen children (70%) with recurrence had two or more risk factors, whereas 36 (72%) without recurrence had no risk factor or only one. We conclude that a selected group of epileptic children who remain seizure‐free for a period of at least 2 years can have AEDs discontinued based on presence or absence of risk factors.

Comparison of chronological and corrected ages in the gross motor assessment of low-risk preterm infants during the first year of life

OBJECTIVE To evaluate the need of chronological age correction according to the degree of prematurity, when assessing gross motor development in preterm infants, during the first year of life. METHOD Cohort, observational and prospective study. Alberta Infant Motor Scale (AIMS) was used to evaluate 43 preterm infants with low risk for motor neurological sequelae, during the first year of corrected age. Mean scores were analyzed according to chronological and corrected ages. Children with motor neurological sequelae were excluded during follow-up. RESULTS Gross motor mean scores in preterm infants tended to be higher when corrected age was used compared with those obtained when using chronological age, during the first twelve months. At thirteen months of corrected age, an overlapping of confidence intervals between corrected and chronological ages was observed, suggesting that from that period onwards correction for the degree of prematurity is no longer necessary. CONCLUSION Corrected age should be used for gross motor assessment in preterm infants during the first year of life.

The role of spontaneous general movement assessment in the neurological outcome of cerebral lesions in preterm infants

OBJECTIVE To study the relationship among the quality, type, and trajectory of general movements in preterm infants and neonatal cranial ultrasonography findings and neurological outcome. METHOD Forty preterm newborn infants, with gestational ages under 35 weeks, had their general movements recorded through video-tape during the preterm, term (37th - 42nd postconceptional weeks of age) and post-term (49th - 56th postconceptional weeks of age) periods, and were prospectively followed up to one-year conceptional age. RESULTS Our results showed that the quality of general movements, particularly in the post-term period (p = 0.009), were related with the presence of severe cerebral lesions in the neonatal cranial ultrasonography and the neurological outcome. While the presence of severe ultrasonography lesions was associated with an adverse neurological outcome (p = 0.01), the finding of normal general movements patterns was associated with a normal neurological outcome, with negative predictive values of 100%, for the preterm, and 80%, for both term and post-term periods. CONCLUSIONS When concurrently used, these evaluation methods may increase the specificity and sensitivity in detecting the group of preterm infants at high risk for neurological disturbances in long-term follow-up.

Brain tumors in the first two years of life: a review of forty cases

From 1962 to 1989, 40 infants with brain tumors and less than 2 years old were treated at the Department of Neurology of the Clinical Hospital of the University of São Paulo Medical School. The clinical and neuropathological findings were reviewed as to histological diagnosis, age, sex, signs and symptoms, therapy and outcome. Medulloblastoma was the most common histological type (n = 11), followed by ependymoma (n = 9), choroid plexus tumor (n = 6), astrocytoma (n = 3) and primitive neuroectodermal tumor (n = 2). The tumor was infratentorial in 21 infants, supratentorial in 18 and disseminated in 1.

Botulinum toxin for treatment of cocontractions related to obstetrical brachial plexopathy

Botulinum toxin type A was recently introduced for treatment of biceps-triceps muscle cocontraction, which compromises elbow function in children with obstetrical brachial plexopathy. This is our preliminary experience with this new approach. Eight children were treated with 2-3 U/kg of botulinum toxin injected in the triceps (4 patients) and biceps (4 patients) muscle, divided in 2 or 3 sites. All patients submitted to triceps injections showed a long-lasting improvement of active elbow flexion and none required new injections, after a follow-up of 3 to 18 months. Three of the patients submitted to biceps injections showed some improvement of elbow extension, but none developed anti-gravitational strength for elbow extension and the effect lasted only three to five months. One patient showed no response to triceps injections. Our data suggest that botulinum toxin can be useful in some children that have persistent disability secondary to obstetrical brachial plexopathy.

Neurological follow-up of small-for-gestational age newborn infants: a study of risk factors related to prognosis at one year of age

To examine the relative importance of some risk factors and neurological prognosis in the first year of life, 37 small-for-gestational age newborns were followed prospectively to 1 year of conceptional age. An abnormal neurological examination was found in 51.3% of the newborns and, at 12 months, 32.5% were still considered abnormal. Only 8.1% of the group had severe neurological sequelae at 1 year of corrected age. The developmental tests showed little changes during the first year, with abnormality rates varying from 16.1 to 25%. The following risk factors were analyzed concerning their relation to neurological and developmental abnormalities: high-risk pregnancy, maternal hypertension, social class, pre-term birth, neonatal asphyxia and weight and height less than 2.5 percentile at the age of 1 year. The statistical analysis showed a high correlation between subnormal weight gain and neurological (p = 0.0001) and developmental (p = 0.001) abnormalities at 1 year. None of the other risk factors were statistically related to neurological prognosis at 1 year.

MOTOR CONDUCTION STUDIES FOR PROGNOSTIC ASSESSMENT OF OBSTETRICAL PLEXOPATHY

Early prognostic assessment of obstetrical brachial plexopathies (OBP) would facilitate rational selection of infants for brachial plexus surgery. We performed bilateral motor nerve conduction studies (MNCS) of axillary, musculocutaneous, radial, median, and ulnar nerves in 33 babies (age 10–60 days) with OBP in order to compare the amplitude of compound muscle action potentials (CMAPs). All babies were followed up until 6 months of age and the outcome was classified according to muscle strength and arm function. A CMAP amplitude reduction of more than 90%, compared to the unaffected side, predicted severe weakness of the corresponding root level (p < 0.01). Our results indicate that MNCS are a useful tool for very early prognostic assessment of OBP. Muscle Nerve 30: 451–455, 2004

Treatment of febrile seizures with intermittent clobazam

Fifty children, 24 female and 26 male, with ages varying from 6 to 72 months (mean = 23.7 m.) that experienced at least one febrile seizure (FS) entered a prospective study of intermittent therapy with clobazam. Cases with severe neurological abnormalities, progressive neurological disease, afebrile seizures, symptomatic seizures of other nature, or seizures during a central nervous system infection were excluded. Seizures were of the simple type in 25 patients, complex in 20 and unclassified in 5. The mean follow-up period was 7.9 months (range = 1 to 23 m.), and the age at the first seizure varied from 5 to 42 months (mean = 16.8 m.). Clobazam was administered orally during the febrile episode according to the childs weight: up to 5 kg, 5 mg/day; from 5 to 10 kg, 10 mg/day; from 11 to 15 kg, 15 mg/day, and over 15 kg, 20 mg/day. There were 219 febrile episodes, with temperature above 37.8 degrees C, in 40 children during the study period. Twelve children never received clobazam and 28 received the drug at least once. Drug efficacy was measured by comparing FS recurrence in the febrile episodes that were treated with clobazam with those in which only antipyretic measures were taken. Ten children (20%) experienced a FS during the study period. Of the 171 febrile episodes treated with clobazam there were only 3 recurrences (1.7%), while of the 48 episodes treated only with antipyretic measures there were 11 recurrences (22.9%), a difference highly significant (p < 0.0001). Adverse effects occurred in 10/28 patients (35.7%), consisting mainly in vomiting, somnolence and hyperactivity. Only one patient had recurrent vomiting which lead to drug interruption. These effects did not necessarily occurred in every instance the drug was administered, being present in one febrile episode and not in the others. We conclude that clonazepam is safe and efficacious in preventing FS recurrence. It may be an alternative to diazepam in the intermittent treatment of FS recurrence.

Choreoathetosis after cardiac surgery with hypothermia and extracorporeal circulation

Eleven children, 4-48 months old, with congenital cyanotic heart defects developed choreoathetoid movements 2-12 days after cardiac surgery with hypothermia and extracorporeal circulation (ECC). The abnormal movements mainly involved the limbs, facial musculature, and tongue, leading to a severe dysphagia. The symptoms had an acute onset, after a period of apparent neurologic normality, and had a variable outcome. Of the nine children that survive, three had abnormal movements when last seen (41 days to 12 months of follow-up). The other six children had a complete regression of the choreoathetoid movements 1-4 weeks after onset. No specific finding was observed in the CT scans, cerebrospinal fluid examination, or EEG that could be related to the abnormal movements. Symptomatic therapy with haloperidol with or without benzodiazepines led to symptomatic improvement in six children, although there was no evidence that this treatment modified the evolution of the disease. The authors conclude that the choreoathetoid syndrome after cardiac surgery with deep hypothermia and ECC is an ill-defined entity requiring additional study to better understand its pathogenesis so that preventive measures can be taken to avoid a condition that can lead to permanent and incapacitating neurologic sequelae.

Treatment of headaches

Objetivo: realizar revisao bibliografica sobre o tema tratamento da cefaleia na infância e adolescencia. Fontes dos dados: revisao bibliografica atraves do banco de dados Medline, utilizando os termos: cefaleia, enxaqueca ou migrânea, infância ou adolescencia e tratamento, no periodo de 1966 a 2001, excluindo artigos de revisao e registros de casos. Foram selecionados artigos cientificos que relataram estudos sobre a eficacia da terapeutica farmacologica e nao farmacologica no tratamento das cefaleias primarias. Sintese dos dados: foram encontrados 104 artigos cientificos, sendo selecionados aqueles que relataram estudos do tipo caso-controle, ou droga versus placebo, cegos ou abertos, e que estudassem criancas ou adolescentes. Conclusoes: e digna de nota a pobreza de estudos controlados sobre o tema na faixa etaria pediatrica, apesar da importância que as cefaleias primarias apresentam na pratica clinica. Apenas algumas drogas foram estudadas de forma cientificamente adequada e, mesmo assim, o numero de estudos e pequeno. Tanto o tratamento da crise algica aguda quanto a profilaxia da cefaleia foram abordados.