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Biomedical subjects

J Boulloche

Publications and source records attributed to J Boulloche.

31 records · Page 2Linked to original sources

[Dysphagia, speech disorders and centrotemporal spikes-waves].

Repeated paroxysmal oropharyngeal fits consisting of sialorrhea and speech arrest are described in a 5 year-4-month old patient. Investigations were initially oriented towards a laryngeal or a gastrointestinal disease. Electroencephalographic tracings showed repeated bilateral centrotemporal spikes-waves discharges which sometimes were associated with bilateral facial clonic contractions synchronous with anarthria and sialorrhea. Consciousness was preserved. Attacks were related with a cluster of fits of a partial epilepsy. Transient pseudo-bulbar palsy (anterior opercular syndrome) resulted from seizures: loss of identifiable speech, drooling with difficulties in swallowing, absence of palatal movements and of the gag reflex. Intellectual functions remained normal. Other investigations were all normal (CT scan, MRI, cytologic and immunological CSF studies). With common anticonvulsant drugs (Valproate and Carbamazepine) seizures resolved within a few weeks. These findings suggest that this particular epilepsy is an unusual type of benign partial epilepsy with centrotemporal (or Rolandic) spikes.

Child, Preschool↗

Postinfectious encephalitis with multifocal white matter lesions.

Two cases of multifocal white matter lesions occurring after viral illness are reported. Evoked potentials study and cranial magnetic resonance imaging (T2-weighted image) showed early abnormalities while CT scan was initially normal. Patients improved dramatically with steroid therapy. It would seem that because of a considerable responsiveness to steroids this affection should be differentiated from other types of encephalitis. Relations with multiple sclerosis are discussed.

Child↗

Risk of recurrence after a single, unprovoked, generalized tonic-clonic seizure.

A cohort of 119 two- to 16-year-old patients was followed to assess the risk of recurrence after a single, unprovoked, generalized tonic-clonic seizure. Only children with a normal neurological examination and intellectual development, and an EEG showing no focal abnormality, were studied. The risk of recurrence at six months was 22 per cent, at one year 28.5 per cent, at three years 32.6 per cent and at eight years 37.7 per cent. 58 per cent of recurrences occurred within the first six months and 87 per cent within the first two years. Initial EEGs showing generalized, unprovoked spikes and waves, generalized paroxysmal discharges precipitated by intermittent photic stimulation, or isolated slowing, were associated with risks of recurrence at two years of 40, 50 and 26 per cent, respectively. These results do not differ significantly from those obtained when the initial EEG was normal (29 per cent). Age, seizure duration and history of epilepsy in the family were not associated with a significantly higher risk of recurrence. The risk of recurrence was similar for treated and untreated patients.

Adolescent↗

[Multifocal epileptic crises following mumps].

A 15 years-old girl with no previous history of epilepsy or neurological disease presented three types of epileptic symptoms the same day: 1) clusters of rhythmic myoclonus of the left hemiface; 2) episodes of painful paresthesias of the left arm followed by secondary generalised seizures; 3) episodes of elementary visual hallucinations of the right hemifield. She had several seizures each hour and some were recorded. There were no EEG abnormalities during the facial myoclonus but rapid rhythms were seen during the sensory and visual partial seizures on the right parietal and left occipital lobes. There was no fever and no drowsiness. The CSF tap showed pleocytosis. Serological studies indicated recent mumps. The drugs were initially inefficient. The seizures disappeared after a month. The drugs were stopped after three months and the seizures had not relapsed after a one year's follow-up. Though there were no other sign of encephalitis, we believe that episode of multifocal seizures was due to mumps encephalitis.

Adolescent↗

[Dystonia in the child].

A classification of child dystonias is proposed as a guide to etiological diagnosis. The analysis of symptoms and signs provides a distinction between dystonia and other involuntary movements: dystonia is a tonic involuntary movement which appears during voluntary activity as a slow and involuntary movement. Two groups of child dystonic syndromes can be distinguished: 1) dystonic syndromes where dystonia is the main neurologic abnormality; they result mainly from toxic and anoxic disorders and from torsion dystonia; 2) dystonic syndromes with associated dystonia and intellectual impairment; they are often familial neurometabolic disorders. Analysis of child dystonias show some common features: a long interval between the causative brain lesion and the onset of dystonia is possible, and may last several years. In neuro-metabolic disorders also dystonia appears after the first year of life, when psycho-motor impairment has already appeared. Etiologic investigations can provide a diagnosis and sometimes a treatment in several varieties of dystonia, e. g. L-Dopa in torsion dystonia, correction of metabolic disturbance in Wilson disease or glutaric aciduria. Genetic counselling should be provided.

Child↗

Dystonic reactions with metoclopramide: is there a risk population?

The authors examine 18 cases of early dystonic syndromes and review all the pediatric cases published previously in order to determine the evolutive and epidemiological characters of these intolerance reactions. These unrecognized manifestations normally occur within the first 24 hours of treatment and disappear within a few hours when the treatment is discontinued. The use of diazepam and trihexyphenidyl accelerates the regression of the disorders. The doses of metoclopramide were mostly above the recommended doses, 60% of the cases correspond to doses in excess of 0.5 mg/kg per day or 0.2 mg/kg in a single dose. In the absence of overdosage, dystonic reactions occur essentially in female subjects aged over 9 years. For this part of the population, the possible occurrence of such a reaction should be mentioned to the family when the drug is prescribed.

Adolescent↗

[Neonatal hypoglycemia caused by hyperinsulinism and subsequent epilepsy].

A historical cohort study was undertaken to determine the risk of epilepsy in a population of 18 newborns with neonatal hypoglycemia due to insulin excess. Follow-up was 3 years 8 months (range 7 years-1 year 3 months). Insulin excess was associated with maternal diabetes in 13 infants, with an isolated macrosomia in 2 infants, in one case with probable Langerhans hyperplasia, and in 2 newborns only prolonged beta-sympathomimetic therapy was the possible cause of insulin excess. Newborns with anoxia, brain malformation or small for date were excluded. Two newborns had hypoglycemia with epileptic clonic seizures, but only one was later epileptic. In this case, hypoglycemia was severe and persistent (16 hours). Hyperinsulinism was related to Langerhans hyperplasia. CT scan made at ages one and 6 months showed large hypodensity of the frontal and occipital white matter. Visual evoked responses were also abnormal. Visual evoked responses and CT scan normalized at 1 year. In other children, asymptomatic hypoglycemia (n = 11) or symptomatic hypoglycemia without epileptic seizures (n = 5) did not increase the epileptic risk as none of them had epileptic seizure later on. Thus, seizures associated with neonatal hypoglycemia alone seem to increase the epileptic risk with duration and severity of hypoglycemia being also essential prognostic factors.

Epilepsy↗

The value of serum IgE assay in milk aspiration and the sudden infant death syndrome.

Global serum levels of IgE (PRIST, Phadebas) and specific anti-cow's milk IgE (RAST) were measured in populations of infants with a history of one or more episodes of milk aspiration (n = 12), victims of the sudden infant death syndrome (SIDS) (n = 18), "near miss" SIDS infants (n = 23), siblings of a SIDS victim ((n = 21) and control infants (n = 38). Only the infants with a past history of milk aspiration had significantly raised global and specific IgE. The absence of raised specific IgE in the subjects who died from SIDS argues against the hypothesis of sensitisation to cow's milk proteins during a previous episode of aspiration. Nasopharyngeal reflux is frequently seen in near-miss infants. This reflux is more often responsible for reflex apnoea due to stimulation of the laryngeal chemo-receptors than to a fatal aspiration.

Animals↗

Pelizaeus-Merzbacher disease: clinical and nosological study.

Pelizaeus-Merzbacher disease can be diagnosed on genetic and clinical criteria. These include: involvement of several males in a lineage in a manner consistent with X-linked recessive inheritance; early nystagmoid movements; precocious psychomotor deterioration; progressive pyramidal, dystonic, and cerebellar signs. We present seven cases from three families and review 148 cases in 19 families from the literature. Laryngeal stridor present in two of our patients may be a presenting feature. Neurophysiological investigations may be helpful in the diagnosis.

Child↗

[Palatal myoclonus syndrome with spontaneous recovery in a child].

In a 71/2 year-old boy clinically isolated palatal myoclonus was observed. It was continuous even during sleep. It disappeared spontaneously after 3 years of evolution. The authors distinguish this syndrome from the one observed in adults, which never regresses.

Child↗