Post-craniotomy headache: a proposed revision of IHS diagnostic criteria.

Seventy-nine patients with intracranial aneurysms were evaluated in the presurgical period, and followed up to 6 months after surgery. We compare patients who fulfilled with those that did not post-craniotomy headache (PCH) diagnostic criteria, according to the International Classification of Headache Disorders. Semistructured interviews, headache diaries, Short Form-36 and McGill Pain Questionnaire were used. Seventy-two patients (91%) had headaches during the follow-up period. The incidence of PCH according to the International Headache Society diagnostic criteria was 40%. Age, sex, type of surgery, temporomandibular disorder, vasospasm, presence and type of previous headaches, and subarachnoid haemorrhage were not related to headache classification. There were no differences in the quality of life, headache frequency and characteristics or pain intensity between patients with headache that fulfilled or not PCH criteria. We proposed a revision of the diagnostic criteria for PCH, extending the headache outset after surgery from 7 to 30 days, and including the presence of headaches after surgery in patients with no past history of headaches, or an increase in headache frequency during the first 30 days of the postsurgical period followed by a decrease over time. Using these criteria we would classify 65% of our patients as having PCH.

Post-craniotomy headache: characteristics, behaviour and effect on quality of life in patients operated for treatment of supratentorial intracranial aneurysms.

We prospectively studied headache characteristics during 6 months after craniotomy performed for treatment of cerebral aneurysms in 79 patients. Semistructured interviews, headache diaries, the Hospital Anxiety and Depression Scale and the Epworth Sleepiness Scales, the Short Form-36 Health Survey (SF-36) and McGill Pain Questionnaire were used. Seventy-two patients had headaches, half before the fifth day after surgery. Changes were observed in headache diagnosis, side and site in the postoperative period. Headache frequency increased immediately after surgery and then decreased over time. Headache frequency was associated with depressive and anxiety symptoms. Pain intensity was higher in women and in patients with more anxiety symptoms. An incidence of post-craniotomy headache of 40% was observed according to International Headache Society classification criteria, 10.7% of the acute and 29.3% of the chronic type. The bodily pain domain of the SF-36 was worse in patients with more anxiety symptoms. Greater frequencies of headache were associated with lower scores on bodily pain and social functioning.

Chronic daily headaches in children and adolescents: a study of clinical characteristics.

The clinical characteristics of chronic daily headache were studied in 40 children and adolescents, as well as the associated factors responsible for maintenance of the continuous headache pattern. The study of the clinical headache characteristics, showed a female preponderance (75%), mean age of 11 years old at the first consultation, and onset of headache symptomatology at a mean age of 8.5 years old. The average time interval for the evolution of sporadic headache into chronic daily headache was 1.4 years, and psychosocial stressors were present, acutely or chronically, during the period of headache-frequency increase in 47% of the children. Headaches were classified as transformed migraine (65%), mixed pattern (17.5%) and chronic tension-type headache (17.5%). Sixty per cent of patients had mothers with migraine. Data regarding common analgesic use showed an average intake of 11.2 days/month.

Migraine in childhood and adolescence. A critical study of the diagnostic criteria and of the influence of age on clinical findings.

We studied 253 children aged <15 years. Phase 1 included 193 children with migraine (1.1 and 1.2) divided into two groups (<10 and > or = 10 years). We studied the relationship between age and migraine type, headache characteristics, and associated symptoms of the International Headache Society (IHS) definition. A higher frequency of migraine with aura, pulsatile quality, and unilateral location was observed in older children. In phase 2 we studied 176 children with headache (excluding migraine with aura), comparing diagnostic criteria, definition items, sensitivity, and specificity. The results showed that item B of the definition was the most frequent cause of exclusion in the 1.7 diagnostic group. Compared with Vahlquist and the IHS, the Prensky criteria were the most sensitive. Sensitivity was >70% for pain of moderate/severe intensity, duration between 2 and 48 h, isolated photophobia, isolated phonophobia, and aggravation with physical activity. Specificity was >70% for nausea, vomiting, phonophobia and photophobia, isolated photophobia, aggravation with physical activity, and isolated phonophobia. Based on three alternative definitions, each modifying one item of the IHS definition, the sensitivity and specificity of these alternative definitions were compared with the "extended" criteria (children with migraine without aura and migrainous disturbance, according to the IHS criteria, grouped together). Exclusion of headache duration increased sensitivity by 10%, compared to restrictive IHS criteria, without decreasing specificity.

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 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 child's 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.

Vigabatrin in refractory childhood epilepsy. The Brazilian Multicenter Study.

Children, 47, with various types of severe drug-resistant epilepsy were entered into a prospective, add-on, open trial with vigabatrin. Patients with West syndrome and idiopathic generalized epilepsies were excluded. Seven children had the drug withdrawn, five because of increase in seizure frequency and two because of adverse effects. Drug efficacy, measured according to seizure type, showed a 100% decrease in seizure frequency in 18.6% of partial seizures and 17.3% of the generalized seizures. There was a higher than 50% decrease in 39.5% of partial and 60.8% of generalized seizures, and less than 50% decrease or increase in seizure frequency in 41.8% and 21.8% of partial and generalized seizures, respectively. Vigabatrin mean dosage during phase 3 was 63.6 mg/kg per day (S.D. = 30.5), ranging from 19.3 to 110.5 mg/kg per day. Parametric statistical analysis (Student's t-test) of seizure frequency between phases 1 and 3 showed a significant decrease in seizure frequency for partial (P = 0.022), and generalized seizures (P < 0.0001). Drug-related adverse effects were observed in 18/47 cases (38.3%), consisting mainly of irritability, hyperactivity, dizziness, somnolence and gastrointestinal symptoms.

DOPA-sensitive progressive dystonia of childhood with diurnal fluctuations of symptoms: a case report.

Progressive dystonia with diurnal fluctuations sensitive to levodopa, also known as Segawa's disease, is a rare form of autosomal dominant extrapyramidal disease in the pediatric age group. The dystonic and Parkinson-like symptoms are the main clinical features of the disease and, characteristically but not in all cases, show a diurnal variation. They are absent or present to a lesser extent in the morning, worsening during the day. Treatment with small doses of levodopa results in remission or marked improvement of the symptomatology. We present the case of a 11 years old female patient that developed a dystonic posture in her feet that led her to a tip-toe walking pattern, since the age of 2. Diurnal fluctuations of the symptomatology were noticed by her mother. At 7 years of age she developed a left deviation of the head and an abnormal flexor posture of the left arm. In the next years the symptoms progressed and the fluctuations became less evident. At the age of 10, they were present soon after she woke up in the morning. The neurological examination disclosed a dystonic posturing of the head and left arm, a generalized rigidity of the extremities and a palpebral tremor. Laboratory examinations, including copper and ceruloplasmin, and neuro-imaging studies were negative. She was started on levodopa 150 mg/day with prompt disappearance of the symptomatology. After one-year follow-up she is symptom-free with only 100 mg/day of levodopa. No adverse effect was observed so far.

Hyperekplexia, a cause of neonatal apnea: a case report.

We report a case of non-familial hyperekplexia which characteristically developed apnea and feeding difficulties in the neonatal period. The abnormal startle response was evident from the second week of life onwards. The infant showed a marked improvement of the startle response and muscle hypertonia with clonazepam. Clobazam was also tried with no apparent response. A prominent long latency C response was observed on EMG examination, suggesting a possible cortical neuronal hyperexcitability origin for the abnormal startle response observed in hyperekplexia.

Midazolam for treatment of refractory neonatal seizures. A case report.

Midazolam is a short-acting water soluble benzodiazepine that has been used with an increasing frequency in the last years. Although there are reports on its use in status epilepticus, there is none in the neonatal period. A pre-term (35 w) AGA newborn infant with a severe hypoxic-ischemic encephalopathy secondary to grade III hyaline membrane disease developed status epilepticus in the first 6 hours of life and was successfully treated with midazolam after phenobarbital and phenytoin failed to achieve seizure control. Dosage schedule was 0.2 mg/kg IV, followed by continuous infusion of 0.025 mg/kg/h. Midazolam is an effective drug for neonatal status epilepticus and more experience should accumulate before it can be routinely employed in the neonatal period. This case shows that it is a possible option before using more dangerous drugs, such as thionembutal.

Phenobarbital in newborns with neonatal seizures. A study of plasma levels after intravenous administration.

Phenobarbital plasma levels were studied in a group of 25 newborn infants. Phenobarbital was administered i.v. in all cases throughout the study period. The mean loading dose was 19.4 mg/kg, ranging from 16.4 to 20.5, and the mean maintenance dose was 4.0 mg/kg/day, varying from 2.6 to 5.0. We obtained mean plasma levels of 22.9 micrograms/ml, 24 h after administering the loading dose. Mean plasma levels at 4, 7, 14 and 21 days were in the therapeutic range (15-40 micrograms/ml), with only a few cases falling outside of it. There was no difference in plasma phenobarbital levels between term and pre-term infants. Side effects were not seen in infants without a severe neurological impairment prior to drug administration.

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.

[Hyperexcitability syndrome in the neonatal period: study of etiological factors]. / Síndrome de hiperexcitabilidade no período neonatal. Estudo de fatores etiológicos.

Forty-five newborn infants with hyperexcitability lasting more than 72 hours were studied in the neonatal period. The association with metabolic disturbances, mainly hypocalcemia, was observed in 53% of cases. Neonatal asphyxia was observed in 21 cases (46%), although all infants had 5 minutes Apgar scores above 6. Only 6 cases had association between neonatal asphyxia and metabolic disturbances. The hyperexcitability syndrome faded away in 1/3 of the infants with metabolic abnormalities after their correction.

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.

[Incidence of peri-intraventricular hemorrhage in preterm newborn infants with birth weight less than 1500 gms: evaluation of brain ultrasonographic studies and necropsy]. / Incidéncia de hemorragia peri-intraventricular em recém-nascidos pré-termo com peso ao nascimento inferior a 1500 gramas. Avaliação dos estudos ultrassonográficos de crãnio e necrópsia.

From January to December 1986, 120 preterm infants were born with birth-weight under 1500 gm. Eighty-five infants were included in the study group either because they had a cranial sonographic evaluation or a necropsy. The mortality rate was 70% for the study group, but was 52.5% for the whole period. The sonographic examination was performed in 41 infants. The incidence of periventricular-intraventricular hemorrhage (PIVH) was 42.3%. Twenty-three cases had PIVH grade II (63.8%), 10 grade I (27.7%), and 3 (8.3%) grade IV. Thirteen infants had sonographic and necroscopic examinations. We observed a total agreement between sonographic and necroscopic diagnosis in 9 cases (69%). Two cases were classified as partial agreement as they differed only in the grading of PIVH, whereas in two cases there was a false negative diagnosis in the ultrasound examination. The interval between the examinations in these two false negative cases was 53 and 20 days, respectively. We conclude that the incidence of PIVH found in our study is comparable to that of the literature and that cranial ultrasonography is a sensitive neuroimage technique for the diagnosis of PIVH in the newborn preterm infant.

Discontinuing medication in epileptic children: a study of risk factors related to recurrence.

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 less than 6 months after discontinuation. Risk factors statistically related to seizure recurrence were greater than 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.

Follow-up study of macrocephalic children with enlargement of the subarachnoid space.

Eighteen macrocephalic children with enlargement of the subarachnoid space (ESAS), with or without mild ventricular dilatation, were followed prospectively to a mean age of 56 months. All were born at term, with uneventful neonatal period and negative tests for congenital infections. There were 17 boys and 1 girl and the mean follow-up period was 46 months (8-58 months). The initial neurologic evaluation, between ages of 2 to 33 months, disclosed abnormalities in 2 cases. At the follow-up one was still abnormal and the other had a normal neurological examination. Another child, who had a normal neurological examination at the age of 5 months, at the age of 7 years and 7 months had an IQ of 77. Thus the abnormality rate at follow-up was 11%. The OFC returned to the normal range in 45% of the children at the follow-up period. There were no cases of intracranial hypertension. One infant had subdural taps performed at the age of 13 months that disclosed a fluid with the same characteristics as the CSF. All the children had a CT-scan performed at the beginning of the study that revealed a large subarachnoid space; in 77% it was associated with mild ventricular dilatation. Eleven had CT-scans repeated, during the study period, which showed resolution of the process in 3 cases, improvement in 2, and unchanged in 6. We conclude that enlargement of the subarachnoid space in macrocephalic children is often a benign entity. ESAS and macrocephaly will still be present in the majority of children in the long-term follow-up.

Follow-up study of macrocephalic children with enlargement of the subarachnoid space

Trata-se de estudo prospectivo de 18 crianças com macrocefalia por aumento do espaço sub-aracnóideo, com ou sem dilataçäo ventricular, seguidas até idade em média de 56 meses. Todas nasceram a termo, sem intercorrências perinatais e com testes negativos para TORCH. O tempo médio de seguimento foi 46 meses. Havia 17 meninos e apenas uma menina no grupo estudado. A porcentagem de anormalidades neurológicas no seguimento foi de 11%. Durante o seguimento, o perímetro cefálico retornou aos níveis da normalidade em 45% das crianças. Nenhum caso desenvolveu hipertensäo intracraniana durante o estudo. Todas as crianças realizaram TAC de crânio como parte da avaliaçäo inicial e, além do aumento do espaço sub-encefálico, 77% delas apresentavam discreta dilataçäo ventricular. No seguimento, 11 realizaram TAC de controle que revelaram resoluçäo completa do processo em 3 casos, melhora em 2 e permaneceram inalteradas em 6. Concluimos que o aumento do espaço sub-aracnóideo em crianças macrocefálicas é entidade que apresenta bom prognóstico neurológico na maioria dos casos e que a macrocefalia e o aumento do sub-aracnódeo continuaräo presentes, na maioria das crianças, no seguimento a longo prazo

Seizure recurrence in infants with neonatal convulsions. A follow-up study.

Twenty three infants with neonatal seizures were followed prospectively to a mean age of 11 months. Only 2 were pre-term and birth weight ranged from 1700 to 4230 grams, with 17 male and 6 female infants. Hypoxic-ischemic encephalopathy was the most common etiology (82.6%). Focal clonic convulsions were the predominant seizure type, present in 7/16 infants in which the seizure type could be identified. All infants had a neurological examination and EEG, and 18 had a cranial ultrasonography performed at the follow-up. Anticonvulsant medication was discontinued, if follow-up EEG and neurological examination were normal. At the follow-up, seizure recurrence was observed in 7/23 (30%) infants. Abnormal EEG, neurological examination and cranial ultrasonography were statistically correlated with seizure recurrence. We conclude that infants with neonatal seizures can remain free of anticonvulsant medication provided they have normal neurological examination, EEG and cranial ultrasonography.

Seizure recurrence in infants with neonatal convulsions: a follow-up study

Vinte e três crianças com crises convulsivas neonatais foram seguidas, prospectivamente, até idade em média de 11 meses. O peso ao nascimento variou de 1700 a 4230 gramas; 2 eram prê-termo; 17 eram meninos e 6, meninas. A encefalopatia hipóxico-siquênica foi a etiologia mais frequente (82,6%. Houve predomínio das crises clônicas focais, presentes em 7/16 crianças nas quais o tipo de crise foi identificado. Todas as crianças foram submetidas a exame neurológico e avaliaçäo eletrencefalográfia e, em 18 delas, foi realizado exame ultrassonográfico (US) de crânio durante o seguimento ambulatorial. A medicaçäo anticonvulsivante foi interrompida se o EEG e o exame neurológico eram normais no seguimento. A recorrência de crises foi observada em 7/23 crianças (30%). Houve relaçäo estatisticamente significante entre a recorrência de crises e anormalidades do exame neurológico, EEG e US de crânio. Concluimos que as crianças com crises convulsivas neonatais podem permanecer sem medicaçäo anticonvulsivante desde que näo apresentem anormalidades ao exame neurológico, ao EEG e ao US de crânio