The clinical characteristics of posttraumatic epilepsy following moderate-to-severe traumatic brain injury in children.

Purpose: Children with traumatic brain injury (TBI) are at increased risk of posttraumatic epilepsy (PTE); the risk increases according to TBI severity. We examined the long-term incidence and risk factors for developing PTE in a cohort of children hospitalised at one medical centre with moderate or severe TBI.

Methods: Moderate brain injury was classified as Glasgow Coma Score on Arrival (GCSOA) of 9-13, and severe brain injury as GCSOA ≤8.

Prolonged febrile seizures, clinical characteristics, and acute management.

Purpose: Prolonged febrile seizures (PFS) lasting ≥15 min have been associated with increased risk for epilepsy in later life. Initial treatment, mostly prehospital, aims to prevent its evolution to febrile status epilepticus (FSE) and reduce adverse outcome. Paucity of information is available on the immediate treatment before reaching a hospital facility.

Steroids for deteriorating herpes simplex virus encephalitis.

A 16-month-old girl presented with herpes simplex virus type 1 encephalitis with involvement of bilateral parietofrontal lobes, left thalamus and cerebellum. She was treated with intravenous acyclovir. As her condition deteriorated, high-dose methylprednisolone was administered, resulting in remarkable improvement.

Overuse of EEG in the evaluation of common neurologic conditions.

The objective of the present study was to analyze the diagnostic indications that most often prompt the referral of children and adolescents in the outpatient clinical pediatric practice for electroencephalographic evaluation and to check its utility in these clinical conditions. The electroencephalographic records of 547 consecutive children and adolescents (5-16 years of age) referred to a single community laboratory for the evaluation of various neurologic disorders were prospectively read by a single blinded investigator. Common diagnostic indications included the following: clinical seizures (42%), attention-deficit-hyperactivity disorder (23%), headaches (10.

Three novel COLQ mutations and variation of phenotypic expressivity due to G240X.

Objective: To determine the molecular basis and consequences of endplate (EP) acetylcholinesterase (AChE) deficiency.

Background: The EP species AChE is an asymmetric enzyme consisting of a tail subunit composed of three collagenic strands (ColQ), each attached to a tetramer of catalytic subunits. The tail subunit is essential for insertion of AChE into the synaptic basal lamina.

Idiopathic trigeminal sensory neuropathy in childhood.

Harris first reported transient idiopathic trigeminal sensory neuropathy in 1935, although it later appeared that, in some of his patients, this condition evolved to typical chronic and painful trigeminal neuralgia. The patients who were later described by Hill and Hughes suffered a combined motor-sensory Vth cranial nerve dysfunction, and most cases reported by Spillane and Wells developed sustained permanent trigeminal neuropathy. The largest reported series of pure trigeminal sensory neuropathy includes 10 adults with varying degrees of sensory disturbance confined to all three nerve divisions.

Inhibitory effect of carbamazepine on inflammatory mediators produced by stimulated glial cells.

Exposure of rat glial cells to lipopolysaccharide (LPS) induces the production of nitric oxide (NO) and prostaglandin E(2) (PGE(2)), the inflammatory mediators implicated in the pathogenesis of seizures and epilepsy. To determine the effect of the anticonvulsant drug carbamazepine (CBZ) on the inflammatory process, LPS-stimulated rat primary glial cultures were exposed to this agent. Dose-dependent inhibition of NO and PGE(2) production was observed of up to 77 and 88%, respectively.

Glial cells production of inflammatory mediators induced by Streptococcus pneumoniae: inhibition by pentoxifylline, low-molecular-weight heparin and dexamethasone.

Exposure of primary rat glial cells to heat inactivated Streptococcus pneumoniae, induced dose-dependent production of tumor necrosis factor alpha (TNF alpha), nitric oxide (NO) and prostaglandin E2 (PGE2). Concomitant addition of the bacterium and the synthetic glucocorticoid dexamethasone resulted in complete suppression of TNF alpha, NO and PGE2 production. Pentoxifylline, a phosphodiesterase inhibitor completely blocked TNF alpha secretion, whereas NO and PGE2 were not affected.

Subclinical status epilepticus in a child after closed head injury.

A 14-year-old girl with closed head injury and a normal computerized tomographic scan underwent an electroencephalographic tracing that surprisingly revealed typical status epilepticus electrical activity. No episodes of motor clinical convulsions were observed from the moment of trauma throughout the admission period. Treatment with phenytoin caused a dramatic clinical improvement.

Cross-sectional and longitudinal study of the pituitary-thyroid axis in patients with thalassaemia major.

Objective And Design: Thyroid dysfunction is known to occur frequently in thalassaemia major, but its prevalence and severity varies in different cohorts, and the long-term natural history is poorly described. We evaluated the pituitary/thyroid axis in thalassaemia major patients in a cross-sectional study and correlated abnormalities with indices of iron overload. Furthermore, the course of thyroid disease in thalassaemia major patients was assessed in a 15-year longitudinal study.

Gastrointestinal protein loss in children recovering from burns.

Qualitative gastrointestinal protein loss was evaluated in 10 children with second- and/or third-degree burns covering 10% or more of their body surface area (BSA) by using fecal alpha-1-antitrypsin (FA-1-AT) as a marker. Patients were subdivided according to the extent of the burned area: group I (5 patients) had burns covering less than 20% of BSA; group II (5 patients) had burns covering more than 20% of BSA (mean, 37.2% = 24.

Giardiasis with protein-losing enteropathy: diagnosis by fecal alpha 1-antitrypsin determination.

Giardia lamblia infection was documented by jejunal biopsy in a previously healthy 2-year-old boy with acute onset of hypoproteinemia due to protein-losing enteropathy. All symptoms and abnormal laboratory findings resolved with anti-Giardia therapy. This is only the second case report of giardiasis with documented protein-losing enteropathy.

Need for endotracheal intubation and suction in meconium-stained neonates.

In a prospective study, we determined whether routine immediate tracheal aspiration at birth is necessary in meconium-stained but otherwise normal infants delivered vaginally and having a 1-minute Apgar score greater than 8. A total of 572 newborn infants who met these criteria were randomly allocated to one of two groups. All infants underwent oropharyngeal suctioning with a DeLee catheter while the head was still on the perineum.

Unexplained neonatal jaundice as an early diagnostic sign of septicemia in the newborn.

This prospective study was performed to determine the frequency of unexplained unconjugated hyperbilirubinemia associated with bacterial infection during the first week of life. Of 5805 infants delivered between September 1984 and December 1986, 93 jaundiced newborns without evidence of septicemia fulfilled the following criteria to be enrolled in the study: weight greater than 2500 g, gestational age greater than 38 weeks, age less than 7 days, and unexplained unconjugated bilirubin greater than 170 mumol/L (greater than 10 mg/dL) during the first 48 hours of life and/or greater than 255 mumol/L (greater than 15 mg/dL) thereafter. Evaluation for septicemia included blood and urine cultures, and white cell and thrombocyte counts.

L-deamino-8-d-arginine vasopressin treatment in pregnancy and neonatal outcome. A report of three cases.

Three women with neurohypophyseal diabetes insipidus, treated for prolonged periods, including pregnancy, with L-deamino-8-d-arginine vasopressin, gave birth in our hospital. Two of the infants had severe congenital heart disease, one of which was associated with trisomy 21. The third baby, born prematurely, presented with mild intrauterine growth retardation; at the age of 21 months, the boy had severe failure to thrive, hypotonia, and motor retardation.

Functional gastrointestinal obstruction in a child with chronic granulomatous disease.

A child with chronic granulomatous disease developed an antral-pyloric obstruction, followed a month later by a postbulbar duodenal obstruction. At both areas, there was no evidence of an anatomical lesion, and some improvement in the passage of barium was observed following glucagon and metoclopramide administration. Presumably, symptoms have resulted from a functional disturbance of gastrointestinal motility.