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Biomedical subjects

G Ponsot

Publications and source records attributed to G Ponsot.

At least 127 records · Page 7Linked to original sources
PubMed1984

[Pediatric dermatomyositis. Apropos of 28 cases].

The authors report 28 cases of juvenile dermatomyositis. Bohan and Peter criteria were fulfilled in all cases. All children received a treatment with prednisone as soon as diagnosis was established. Five patients (18%) died during this treatment. The remaining children were followed for at least 2 years after prednisone treatment was discontinued. An acute onset was found in 4 of the 5 children who died and in 6 of 23 children still alive. Swallowing disorders were present in all children who died and in 11 of the children still alive. Ten children recovered fully and 13 presented with sequellae. The average interval between clinical onset and diagnosis was 2 months 19 days for the children who recovered and 5 months 21 days for those presenting with sequellae. The average duration of the period of activity of dermatomyositis was 6 months for the children who recovered and 3 years 4 months for those with sequellae.

Adolescent↗
PubMed1984

[Calcinosis of childhood dermatomyositis. Apropos of 10 cases].

Twenty-eight children were diagnosed as having dermatomyositis (DM) on the basis of the criteria of Bohan and Peter. Ten of the 28 children developed calcinosis. Calcium deposits mainly occurred in DM with gradual onset of symptoms and with a chronic course. Calcinosis always appeared insidiously and early during the active stage of the disease. It persisted for a long time beyond the remission of the active muscle symptoms without tendency to spontaneous resolution. Long-term follow-up data substantiate calcinosis as the most frequent and the most serious sequela in this series of childhood DM.

Calcinosis↗
PubMed1983

[Emergency indications for computed tomography of the skull in pediatrics].

Indications of CT examinations should be carefully established. Undisputed emergency indications in pediatric neurology are specified. Acute intracranial hypertension, whether isolated or associated with neurological signs or fever, is the principal emergency indication. Its main causes are posterior fossa tumors, but other space-occupying lesions may be found. The main radiological features in each etiology are described. Hemorrhage and trauma are the other essential emergency indications. Emergency CT examination can also be performed in unexplained coma. The indications of CT and ultrasonography in neonates are discussed.

Brain↗
PubMed1983

Hamartomas of the tuber cinereum.

The hamartoma of the tuber cinereum, a midline dysraphic syndrome, corresponds to a mass of normal neuronal tissue implanted in the area of the mamillary bodies. It has no evolutionary potential, but association to other cerebral malformations has been observed. On the basis of 18 personal cases and a review of the literature, the authors evaluate the actual diagnostic criteria and the incidence of different clinical signs such as precocious isosexual puberty, seizures, and mental impairment.

Adolescent↗
PubMed1983

[Hypothalamic hamartomas and laughing seizures].

The authors report 7 new cases of children with laughing seizures associated with hamartoma of the hypothalamus. Precocious puberty is present in 4 cases and seizures of another type (clonic or tonic) in 5 cases. The diagnosis is confirmed by histology (2 cases) or by CT scan (5 cases). The interictal EEG abnormalities are detailed as well as those found during the laughing seizures which were registered (3 cases). The literature was reviewed and 18 cases were found with posterior hypothalamic lesions and laughing seizures: they have been studied and compared with ours. The role of hamartoma is discussed in the production of these seizures, either isolated laughing seizures or else laughing seizures accompanied with other symptoms.

Adolescent↗
PubMed1983

[Nosological aspects of epilepsia partialis continua in children].

Among 26 patients suffering from Epilepsia Partialis Continua, 2 major groups were observed. The first, resulting from a fixed lesion of the rolandic area, showed electro-clinical correlation of seizures; the latter disappeared during sleep; clinical and radiological follow-up failed to disclose any worsening of the cerebral lesion. The second group was characterized by progressive mental and motor deterioration, lack of electro-clinical correlation of fits, persistence of the latter during sleep and frank increase of cerebral atrophy observed on serial neuroradiological examinations. This easily recognized group seems to result from a progressive inflammatory disease of unknown cause.

Child↗
PubMed1983

[Cerebral ischemic accident and hereditary spherocytosis].

A cerebral ischemic accident is reported in a girl presenting with hereditary spherocytosis. The relationship between congenital hemolytic anemia and acute vascular accidents are discussed from data concerning other types of chronic hemolytic anemias. The possible predisposing role of splenectomy in this child is underlined and the risk of ischemic accidents in all patients undergoing splenectomy is emphasized.

Child↗
PubMed1983

[Herpetic encephalitis in infants and children. Methods of diagnosis].

Thirteen infants and 2 children with Herpes simplex encephalitis are reported and the authors emphasize the diagnostic value of several investigations: the neurological examination (fits followed by early motor deficit on the same side and coma), the EEG (periodicity and asymmetry of the trace), the CT scan (hypodensity in the frontotemporal areas), the level of the Interferon alpha in blood and cerebrospinal fluid, the electrophoretic pattern of cerebrospinal fluid proteins and the comparative study of cerebrospinal fluid/serum antibodies towards several viral antigens.

Adolescent↗
PubMed1983

[Hypothalamic hamartoma and gelastic crises. Apropos of 7 cases].

Seven cases of hypothalamic hamartomas with gelastic seizures are reported. A precocious puberty was found in 4 cases. The normal neurologic examination and lack of sign of intracranial hypertension were in contrast with the severity of the epileptic seizures, of the mental impairment and of the behavioral disorders. The fact that the presenting symptom may be gelastic seizures is stressed. CT scan is the best means to assess the diagnosis and to follow the evolution of these tumors. Except for the management of the precocious puberty, the treatment is disappointing and neurosurgical indications are quite exceptional.

Adolescent↗
PubMed1982

[Thalamic tumors in children. A study of 38 cases (author's transl)].

The main clinical, evolutive and therapeutic features of thalamic tumors are reviewed in 38 children. Signs of increased intracranial pressure (76% of the cases) and of controlateral hemiparesis of varying degree (76% of the cases) were the main clinical symptoms. CT-scan is the best means of investigation. Therapeutic protocols that seem to obtain the best results consist of radiotherapy (between 45 and 55 grays) associated with shunting, when intracranial hypertension results from obstruction of an interventricular foramen by the tumor. Surgery is contra-indicated in most tumors in this area.

Adolescent↗
PubMed1982

[Paraplegia complicating surgery for coarctation of the aorta].

A case of paraplegia occurring during surgery for coarctation of the aorta in a 28 month-old child is reported. Paraplegia was due to medullary ischemia, probably related to insufficient collateral circulation. Such ischemic accidents generally remain unforeseeable and no good prevention is available. This rare operative complication should not alter the present management of coarctation of the aorta.

Aortic Coarctation↗
PubMed1981

[Progressive bulbar paralysis. Report of a juvenile case (author's transl)].

The authors report the case of a 16 year-old girl with the following features: clinically, a progressive bulbar paralysis, a weakness and wasting of muscles predominantly in the upper limbs; pathologically, a severe neuronal loss in the motor nuclei of the VIIth, IXth, XIIth cranial nerves and in the anterior horns of the cervical and thoracic spinal cord, a demyelinisation of the corticospinal tracts. A classification of progressive bulbar paralysies in infants and children is proposed. In the first group, the peripheral motoneuron is the only involved. Such cases are often called Fazio-Londe disease and can be related to those cases of infantile spinal amyotrophy either of the Werdnig-Hoffmann type or, most often, of the Wolfhardt-Kugelberg type. In the second group, the corticospinal tract is also involved. Some of these cases can be included in the spinocerebellar degenerations but others, such as the case reported here, are strongly reminiscent of the adult amyotrophic lateral sclerosis.

Adolescent↗
PubMed1981

[The pattern of cerebrospinal fluid proteins in infants and children. Determination of normal values (author's transl)].

Cerebrospinal fluid (CSF) protein values were measured in 652 children between the ages of 1 day and 17 years, allowing the authors to define the dynamics of the blood-brain barrier under normal conditions and during inflammation of the nervous system. The ratio of CSF albumin/serum albumin (whose upper limit was 0.65 in the study) was the best sign of alteration of blood-brain barrier permeability. The CSF IgG level, whose upper limit was 0.85 (for serum IgG between 10 and 14 g/l) is the most useful criterion for detecting an intra-thecal synthesis of IgG. Six patterns of CSF proteins are defined on the basis of immunochemical and electrophoretic studies. The ratio of CSF albumin/serum albumin and the CSF IgG level must be compared to the electrophoretic pattern of CSF proteins in order to better characterize one aspect of the blood-brain barrier under normal and pathologic conditions of the central nervous system.

Adolescent↗
PubMed1981

[Optic neuritis in children. Clinical aspects and evolution in 14 patients (author's transl)].

Fourteen children presenting with optic neuritis are reported. Bilateral ocular lesions and papilledema were present in 80% of cases. Biological investigations did not show any local production of IgG or anti-measles antibodies, contrary to what is observed in adults. CAT scan most always rules out a tumoral compression that could be responsible for a sudden decrease in visual acuity. Prognostic factors helpful in predicting evolution towards multiple sclerosis are discussed.

Adrenal Cortex Hormones↗