A prospective open-labeled trial with levetiracetam in pediatric epilepsy syndromes: continuous spikes and waves during sleep is definitely a target.

Although LVT is currently extensively prescribed in childhood epilepsy, its effect on the panel of refractory epilepsy syndromes has not been entirely evaluated prospectively. In order to study the efficacy and safety of LVT as adjunctive therapy according to syndromes, we included 102 patients with refractory seizures (6 months to 15 years) in a prospective open-labeled trial. The responder rate was respectively 36% and 32% at 3 and 6 months with 6% and 7% patients becoming seizure free.

Ring chromosome 17 epilepsy may resemble that of ring chromosome 20 syndrome.

A four-year-old boy with ring chromosome 17 presenting with early-onset, pharmacoresistant epilepsy underwent repeated 24-hour video-EEG monitoring and cytogenetic analyses, including fluorescent in situ hybridization with telomeric and locus-specific probes of chromosome 17. Epilepsy was characterized by nocturnal motor seizures and by prolonged diurnal electrical status epilepticus. The 46, XY, r (17) karyotype was observed in the majority of cell lines.

Coincidence of two genetic forms of Charcot-Marie-Tooth disease in a single family.

The authors report a family in which two affected first cousins had a severe demyelinating Charcot-Marie-Tooth disease (CMT) phenotype. One had related parents, and there were no other affected relatives, suggesting an autosomal recessive mode of inheritance. Molecular studies showed that a de novo duplication in 17p11.

West syndrome and mitochondrial diabetes: relationship or coincidence?

West syndrome occurs in infancy and in early childhood. It is characterized by intractable seizures occurring almost daily, severe psychomotor retardation, poor prognosis and EEG abnormalities, known as hypsarrhythmia. We report here the case of a 28-year-old patient, who was diagnosed with West syndrome when he was 8 months old and with diabetes mellitus when he was 25 years old.

[Electroencephalographic effects of sevoflurane in pediatric anesthesia: a prospective study of 20 cases].

Objective: To study electroencephalographic (EEG) changes in children during induction of anaesthesia with 8% sevoflurane.

Patients: Twenty patients were consecutively included (ASA I-II; aged: 13-101 months).

Material And Method: Prospective study; approved by the Ethics Committee; written informed consent obtained from parents; anaesthesia induced with 8% sevoflurane in oxygen; no premedication was given; to collect the EEG data, non-invasive electodes were installed before induction; EEG was recorded continuously and stored on a computer for later analyses (descriptive analysis).

[Dermal sinus and dermoid cyst revealed by abscess formation in posterior fossa. Report of 2 pediatric cases and review of the litterature].

Cranial dermal sinus, usually associated with dermoid cyst, is the persistance of an abnormal embryonal communication between the skin and the central nervous system. It may be the source of intracranial infection, most often a meningitis and rarely an abscess formation. Two cases of little girls (18 months and 2 years) having dermal sinus with dermoid cyst revealed by cerebellar abscess formations are reported.

[Benign infantile convulsions. French collaborative study].

Background: Benign infantile non febrile seizures are not well known, leading us to study their clinical and EEG characteristics.

Methods: Between 1981 and 1994, we assembled 34 patients with the following inclusion criteria: non febrile seizures between 1 month and 2 years of age, normal personal history, no abnormality on clinical, biological and radiological investigations, normal developmental outcome with at least 1 year follow-up.

Results: These 34 patients were recognized as 14 familial cases (identical seizures affecting parents) and 11 non familial cases.

Invasive character of an intradural spinal meningioma in early childhood.

The authors report the case of a 2-year-old baby girl with an intradural meningotheliomatous meningioma of the cauda equina which recurred three times. Despite four operative procedures and localized radiotherapy, the lesion kept on growing to reach the retroperitoneal space. Extensive laminectomy and associated radiotherapy were probably responsible for a spinal dislocation which had to be operated on.

Recurrent intrinsic brain stem epidermoid cyst.

The authors report the case of a 14-month-old baby boy with an epidermoid cyst located entirely within the pons and medulla, without an exophytic component. The lesion was examined by computed tomography and magnetic resonance imaging. The child was operated upon three times after two recurrences of the lesion.

Double-blind, randomized trial of diazepam versus placebo for prevention of recurrence of febrile seizures.

The aim of this study was to evaluate the efficacy and tolerance of intermittent oral administration of diazepam during hyperthermia for reducing the recurrence of febrile seizure: 185 children, between 8 months and 3 years of age, with a first febrile seizure and normal neurologic development, were randomly assigned in a double-blind fashion to receive orally administered diazepam (0.5 mg/kg, then 0.20 mg/kg, every 12 hours) or placebo, whenever the rectal temperature was more than 38 degrees C.

[Use of acetazolamide in external hydrocephalus in infants].

External hydrocephalus is believed to be benign but published cases do not clearly establish this prognosis. Five children with typical external hydrocephalus were given acetazolamide. Head circumference measurements or other investigations demonstrated therapeutic effectiveness in four of the five patients.

[Congenital cutaneous aplasia of the scalp. Apropos of 4 cases].

Report of four cases of congenital defects of the scalp. In two of these cases, the skin defect was associated with a skull defect and in one, there were other cranial deformities. In each case, the lesion seemed isolated in otherwise normal children.

[Brain stem auditory evoked potentials in children with partial seizures].

BAEP were recorded in 34 children with partial seizures. Epilepsy with rolandic spikes and occipital paroxysms were excluded. Results were then compared with data from subjects without neurological diseases.

[Neurological manifestations in relation to vitamin E deficiency, caused by a defect of biliary acid synthesis].

A progressive neurological syndrome with cerebellar signs, abnormal proprioception, areflexia and Babinski response was observed in a child with chronic intestinal malabsorption. There was no ophtalmoplegia or retinitis pigmentosa. Electromyography and biopsy showed no axonopathy or myopathy.

[Familial distal progressive spinal amyotrophy with asymmetry of the lower limbs].

Two cases of spinal muscular atrophy, localized on the lower limbs and strictly unilateral, in two half brothers, are reported. Such a distribution of clinical changes and such a hereditary transmission appear to be unusual in hereditary motor-neuronopathies. A recessive heterotopic transmission may be suspected.

Combined continuous wave Doppler and radionuclide delay methods versus angiography in detection of vertebral basilar arterial obstruction.

Noninvasive diagnosis of vertebral artery disease is difficult, but the combined use of Doppler ultrasonography and dynamic radionuclide studies, with measurement of the carotid-basilar delay (CBD), increases the sensitivity and can indicate the level (cervical or distal) of the lesion. Forty patients with cerebrovascular disease were studied by continuous wave Doppler and CBD and the results were compared with vertebral angiograms. The mean normal CBD value was determined for a control group of 18 patients.

[Rolandic paroxysmal epilepsy. Prognostic, electric, clinical limits. Study of 28 cases (author's transl)].

Rolandic paroxysmal epilepsy remains a frequent eventuality in children and even more so than the "minor epilepsy". In more cases, it is easily diagnosed and enables the physician to reassure the family which is usually frightened by the word "epilepsy". Both complementary examinations and therapeutic excesses can also be reduced.