Ischemic cerebrovascular disease in children: retrospective study of 35 patients.

A 10-year review of a neuropediatric department experience with childhood ischemic cerebrovascular disease identified 35 patients with arterial ischemic stroke. The ability to diagnose stroke in children has improved with the development of imaging techniques in the past few years. Children have a wide array of risk factors for ischemic strokes, since some are acquired and others are congenital.

Relapsing acute encephalopathy: a complication of diphtheria-tetanus-poliomyelitis immunization in a young boy.

Unlabelled: Neurological complications of immunizations are rare. We report the case of relapsing acute encephalitis in a boy after two subsequent diphtheria-tetanus-poliomyelitis vaccinations. First the clinical signs were those of acute disseminated encephalitis.

Plasma concentrations and pharmacokinetics of idebenone and its metabolites following single and repeated doses in young patients with mitochondrial encephalomyopathy.

Objective: The pharmacokinetics and tolerance of idebenone after single or repeated doses have been studied in young patients with mitochondrial encephalomyopathy.

Results: No significant adverse effects were noted. In 3 out of 7 patients idebenone induced overall stimulation and improvement in arousal.

Genetic heterogeneity of familial hemiplegic migraine.

Familial hemiplegic migraine (FHM) is an autosomal dominant variety of migraine with aura. We previously mapped a gene responsible for this disorder to the short arm of chromosome 19, within a 30-cM interval bracketed by D19S216 and D19S215. Linkage analysis conducted on two large pedigrees did not show any evidence of heterogeneity, despite their clinical differences due to the presence, in one family, of cerebellar ataxia and nystagmus.

Valproate-induced hepatic failure in a case of cytochrome c oxidase deficiency.

We report a 3 year-old girl with a myoclonic epilepsy. A fatal hepatic failure occurred after 3 months of valproate (VPA) therapy. In this patient, a defect of cytochrome c oxidase (COX) was demonstrated in her circulating lymphocytes.

[Generalized epilepsy disclosing medium-chain-acyl-CoA dehydrogenase deficiency].

Background: Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is the most common inherited defect of fatty acid beta-oxidation. As it causes life-threatening symptoms, it is mandatory to diagnose deficiencies of this enzyme in families.

Case Report: A boy was admitted at the age of 3 months because of a recent attack of generalized seizures.

Mevalonic aciduria in 3 siblings: a new recognizable metabolic encephalopathy.

Mevalonic aciduria, due to mevalonate kinase deficiency, is the first recognized defect in the biosynthesis of cholesterol and isoprenoids. Very few patients with this disorder have been reported. Three siblings born from consanguineous parents are reported.

[Ischemic cerebrovascular accidents in homozygous sickle cell anemia. Two case reports].

Two cases of ischemic stroke in children of Tunisian descent with sickle cell anemia are reported. Patients were aged 3 years and 14 years, respectively. Periodic partial exchange transfusions with sedimented red blood cells of identical phenotype prevented further episodes of ischemic stroke.

[Spinal cord tumor with meningeal invasiveness revealed by intracranial hypertension syndrome].

The association of hydrocephalus and intramedullary spinal-cord tumor with leptomeningeal spread is described in a 20 month-old boy. The pathogenesis of increased intracranial pressure in spinal cord tumor is discussed. Meningeal gliomatosis is rare and is more often observed in the follow-up of cerebral gliomas.

[Outcome of herpetic encephalitis. Apropos of 10 cases].

Ten cases of herpetic encephalitis in children aged 2 months to 13 1/2 years at onset are reviewed retrospectively. There were four infants and six children 3 to 13 1/2 years old. Diagnosis was established on the basis of widely accepted electroclincal and neuroradiological criteria.

[A cerebral abscess due to Listeria monocytogenes in a 15-month-old infant].

We report a case of brain abscess due to Listeria monocytogenes in an infant. We recall that listeriosis is infrequent in pediatric patients beyond the neonatal period, that most cases occur in immunocompromised hosts, and that clinical features are non-specific in neuromeningeal forms. Management of brain abscesses is discussed.

[A therapeutic trial of mazindol versus placebo in Duchenne muscular dystrophy. A one-year follow-up study of 14 children].

A simple-blind therapeutic trial of mazindol (2 mg/d) versus placebo in 14 boys with Duchenne muscular dystrophy, 5 to 13 years old, for 12 months was conducted in order to analyse the efficacy of this drug on the natural history of the disease. Evaluation of muscle strength by manual testing, motor ability by functional testing and timed tests, weight, height, serum CK and pulmonary function were performed every two months. The differences in evolution between groups were significant only for weight and height.

Magnetic resonance imaging in partial epilepsy of childhood. Seventy-nine cases.

A heterogeneous series of 79 children with partial epilepsy was studied by computerized tomography (CT) and magnetic resonance imaging (MRI) to evaluate the contribution of MRI to the aetiological diagnosis of that disease. Both CT and MRI were normal in 13 cases and abnormal in 41 cases showing the same pathology (malformation, atrophy, tumour, scar). In 4 cases of continuous partial epilepsy the lesions observed were either diffuse or focal corresponding to the electrical focus, and they could be due to Rasmussen's encephalitis.

Indications of radiological explorations in partial epilepsy of childhood.

In partial epilepsy of childhood the decision to request radiological exploration rests on a good knowledge of the disease. The authors summarize the electrical and clinical features of the various types of partial epilepsy. Most of these types, and in particular benign partial epilepsy with rolandic paroxysms, require no exploration.

Lissencephaly (agyria-pachygyria): clinical findings and serial EEG studies.

Fifteen cases of lissencephaly were studied and the literature reviewed. The authors conclude that the clinical findings of lissencephaly in infancy are non-specific, consisting of developmental delay and hypotonia. While the CT scan establishes the diagnosis, it may also be strongly suggested by an EEG showing 'major fast dysrhythmia', characterized by abnormally rapid, very high-voltage activity, predominantly in the alpha and beta frequency bands.

Farber's disease in two siblings, sural nerve and subcutaneous biopsies by light and electron microscopy.

Two siblings born from consanguineous tunisian parents are reported. They showed a severe form of Farber's disease with prominent involvement of the central and peripheral nervous system: low conduction velocity was noticed in both children. Macular cherry red spots were observed in one of them.

Radiology of ischemic strokes in children.

Arterial ischemic strokes are a relatively frequent diagnostic occurrence in pediatric neuroradiology. They occur mostly in three main etiologic contexts: congenital heart disease; neonatal distress; infections, focal or general inducing vasculitis, but many cases are considered idiopathic. The positive diagnosis is made by CT; in neonates, however, ultrasound appears as a promising tool.

[Epileptic evolutive encephalopathies in infants (West tsyndromee and Lennox-Gastaut syndrom) (author's transl)].

Electroclinical aspects of the West syndrome are studied in their atypical forms: clinical and EEG characteristics, partial forms, Aicardi syndrome. Etiological investigations (especially CT scan) are necessary to distinguish the more frequent 'secondary forms' from the 'primary forms'. The etiological factors are: (i) Prenatal causes (hereditary affections, chromosome-related etiology, fetal etiology; (ii) Perinatal causes (prematurity, traumatic delivery); (iii) Postnatal causes (infectious diseases).

[Ataxia-opsoclonus-myoclonus syndrome].

The ataxia-opsoclonus-myoclonus syndrome that was well individualized by Kinsbourne is mostly observed in young children (less than three years old in 90 percent of the cases). From six personal cases, and from a review of ninety cases of the literature, the clinical and etiological features, as well as the evolution of the syndrome, are studied. Prodromes (infectious and digestive manifestations) and comportmental changes usually precede the sudden onset of the clinical triad.