Convulsive status epilepticus in Thai children at Ramathibodi Hospital.

BACKGROUND: Convulsive Status Epilepticus (SE) is an emergency neurological condition with high morbidity and mortality. The outcome of this condition in children depends on the etiology and the duration of convulsion. There is no report of this condition in Thai children. OBJECTIVE: To study the etiology, clinical course and outcome in children with convulsive SE in a referral hospital in Thailand. MATERIAL AND METHOD: The medical records of infants and children aged between one month and 15 years with the diagnosis of SE who were admitted to the Department of Pediatrics, Ramathibodi Hospital, Bangkok from January 1st, 1981 to December 31st, 2000 were retrospectively reviewed. The demographic data, types of seizure, duration of seizure, underlying diseases, precipitating factors, laboratory results, treatment, clinical course and outcomes were collected for descriptive analysis. RESULTS: Thirty-two patients (15 boys, 17 girls) whose ages ranged from 2 months to 14.4 years (mean 6.5 years) were included Twenty-four patients had underlying epilepsy. Twelve patients had prior diagnosis of symptomatic and idiopathic/cryptogenic epilepsy. Seven patients had acute insults to the central nervous system leading to SE. One patient with acute lymphoblastic leukemia presented with SE without association to either the underlying disease or the treatment. Fever with or without specific infection was the most common precipitating factor observed in these patients. The mean duration of SE was 64.4 minutes. The mean duration from initiation of treatment to the cessation of seizure was 41.4 minutes. Twelve patients were lost to follow up. Of the two patients who died, one had severe infection and the other had renal failure. Twelve patients had severe neurological deficits and six had mild neurological deficits. Among the thirteen patients who had > or = 1 hour of convulsion, eleven had severe neurological deficits or died. CONCLUSION: Infantile SE occurred more frequently in children with pre-existing epilepsy or neurological disorder Acute febrile illness and infection were the most common precipitating causes in the present study. Early recognition and treatment of fever and infection in conjunction with prompt and appropriate termination of seizure in epileptic children may prevent the occurrence of SE and its morbidity.

Flunarizine for prophylactic treatment of childhood migraine.

OBJECTIVE: To determine the effectiveness of flunarizine for migraine prophylaxis in children. PATIENTS AND METHOD: Children aged between 7 and 15 years who had the indication for prophylactic treatment of migraine were recruited into a prospective study at the Department of Pediatrics, Ramathibodi Hospital, from January 1st to December 31st 1999. After verbal consent was obtained, flunarizine was administered either at 5-mg daily in those who had never received it or at 10-mg daily in those who previously took this drug within one year Serial evaluation for the severity of migraine including duration, intensity, and frequency of headache attacks was performed every 2 weeks for 6 months. RESULTS: Twenty-one children (10 boys, 11 girls) with a mean age of 11.3+/-2.48 years (range 7-15 years) were enrolled in the study. There were ten children who had migraine with aura. Initially, 5-mg daily and 10-mg daily of flunarizine were administered in 19 and 2 patients respectively. The dosage was increased to 10-mg daily after two weeks in 5 patients because of the unresponsiveness to the initial dose. Improvement was observed in 14 patients (66%) including 13 of 14 patients who received 5-mg daily and 1 of 7 patients who received 10 mg daily. Five patients (23%) had no recurrent attack. Nine patients (42%) had more than 50%-reduction of frequency of migraine and 3 of these had either shorter duration or less intensity of the attack. Clinical improvement was observed between 2 and 4 weeks after initiation of treatment. There was no adverse effect observed CONCLUSION: This is a preliminary result demonstrating that flunarizine is one of the effective drugs for migraine prophylaxis in children.

Nonketotic hyperglycinemia in two siblings with neonatal seizures.

Seizures are a common problem in neonates. Differential diagnoses include infection, trauma, hypoxia and congenital metabolic disorders. Among these, congenital metabolic disorder is less familiar to general pediatricians. We report two patients with nonketotic hyperglycinemia (NKH), a rare and lethal congenital metabolic disease. Transient hyperammonemia and transient hypouricemia, uncommon features found in NKH, were detected in one patient. High doses of sodium benzoate and dextromethorphan failed to modify the clinical course. Neuropathology denoted characteristic diffuse vacuolization and changes in reactive and gliotic astrocytes. The clinical course, biochemical findings, diagnostic approaches and diagnostic tests are discussed in detail. Recent modalities of treatment are reviewed. Because of its rarity and rapidly progressive course, it maybe underdiagnosed resulting in death before being recognized. Awareness of the possibility of congenital metabolic disorder in early neonatal catastrophe will increase the diagnostic rate.

Isolated lissencephaly sequence with contiguous gene deletion detected by FISH analysis: a case report.

BACKGROUND: Lissencephaly is a clinically and genetically heterogeneous malformation of the brain, usually leading to a severe disabling condition and seizures. The recent discovery of molecular techniques and identification of lissencephaly genes (e.g. LISI and DCX) has allowed etiologic diagnosis of this disorder feasible. OBJECTIVE: To describe a patient with lissencephaly in whom fluorescence in situ hybridization (FISH) determined etiologic diagnosis, providing precise genetic counseling and possible prenatal diagnosis for the family. CLINICAL REPORT AND STUDY RESULTS: The authors report a 4 month-old girl who presented with intractable, generalized myoclonic seizures at I month of age. The patient was born at 37 weeks' gestation, to a G4P1A2 36-year-old woman. Chromosome analysis from amniotic fluid performed for advanced maternal age revealed normal karyotype. Pregnancy was complicated by polyhydramnios. Computed tomographic scan of the brain at age one month showed a total absence of gyral formation. FISH of the metaphase chromosome from the patient, using Smith-Magenis and Miller-Dieker/ILS probe showed two signals of Smith-Magenis probe but only one signal of Miller-Dieker/ILS probe, indicating a microdeletion of 17pl3.3 region including LIS1 gene. Hybridization of the ILS probe on the metaphase chromosome of both parents was normal. CONCLUSION: A confirmation of contiguous gene deletion in this patient lead to an etiologic diagnosis of lissencephaly. This information allowed precise genetic counseling, estimation of recurrent risk, and definite prenatal diagnosis available to the family. The authors suggest FISH 17p13.3 studies be performed in addition to a standard metaphase analysis in all patients with type I lissencephaly.

Serum diazepam levels after oral administration in children.

OBJECTIVE: To determine serum levels of diazepam after oral administration in children. PATIENTS AND METHOD: Forty six children admitted with febrile seizures were orally administered with 0.25 mg/kg/dose of diazepam six hourly for four doses. Trough (prior to the next dose) and peak (at 1 hour 20 minutes after the dose) serum levels of diazepam were analyzed. The patients were observed for adverse effects of the medication. RESULTS: The peak levels after 1st, 2nd, 3rd and 4th doses were above 0.15 microg/ml which is considered the therapeutic level in 93.5, 97.8, 97.7, and 100 per cent of the patients, respectively. The trough levels prior to the 2nd, 3nd, and 4th doses were greater than 0.15 microg/ml in 75.0, 84.0, and 91.3 per cent, respectively. Neither recurrent seizure nor serious adverse effects occurred in any of the patients. CONCLUSION: Serum concentrations above the therapeutic level were achieved after orally administered diazepam at 0.25 mg/kg/dose six hourly for four doses. Oral diazepam may be used as another method in the prevention of recurrent febrile seizures.

Sebaceous nevus syndrome as the underlying cause of intractable seizures in a one-month-old infant.

A one-month-old male infant with generalized seizures since 2 days old was evaluated at the Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. His seizures were initially characterized as focal movement of the right upper and lower limbs followed by generalized tonic. Initially, phenobarbital was administered but failed to control his seizures. Physical examination revealed generalized mild hypotonia with a hyperpigmented brownish patch affecting the left side of his face. The initial diagnosis was sebaceous nevus syndrome which is one of the neurocutaneous syndromes. The diagnosis was confirmed by skin biopsy of the affected lesion. The literature was reviewed and discussed. The authors emphasized the importance of thorough physical examination including evaluation of specific skin lesion which would be a leading clue in making the diagnosis of symptomatic epilepsy in infants.

Outcome of intracranial hemorrhage in infants with congenital factor VII deficiency.

The outcome of 8 episodes of intracranial hemorrhage in 7 patients (4 males, 3 females) with congenital factor VII deficiency was evaluated. Their levels of factor VII clotting activity (FVII:C) were less than 1 per cent (n = 3) and ranged from 1.7 to 2.3 per cent (n = 4). The onset varied from the first week (n = 2), first month (n = 3), and at the ages of 6, 11 and 12 months (n = 3). The replacement therapy of 10 ml/kg of fresh frozen plasma (FFP) every 6-12 hours for 5-7 days was given to 6 patients. Only one craniotomy for the removal of hematoma was performed. The seventh patient experienced two episodes of bleeding. First, she received 20 microg/kg of recombinant factor VIIa (rFVIIa) every 6 hours for 4 days (1,200 microg) followed by FFP in one episode. Second, a craniotomy for the removal of a 7 cm diameter hematoma was performed by giving 20 microg/kg of rFVIIa every 6 hours for 12 days (9,600 microg) followed by FFP in another episode. As a result of these treatments, 2 died and 5 survived with sequelae, except for one who received rFVIIa. The sequelae included seizure disorder (n = 1) and hydrocephalus (n = 3). Subsequently, the surviving patients received 15 ml/kg of lyophilized fresh plasma every 3-5 days as prophylactic treatment. In conclusion, rFVIIa in the dose of 20 microg/kg every 6 hours has been shown to be effective in controlling intracranial hemorrhage in patients with congenital factor VII deficiency.