Prognosis and long-term follow-up in patients with medulloblastoma.
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Biomedical subjects
Publications and source records attributed to M Choux.
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An unusual cerebral tumor is reported in a ten-month-old boy. The tumor was large (10 cm x 6 cm), firm and included several cysts. Light and electron microscopy showed a striking cellular pleomorphism. Typical neurosecretory ganglion cells were observed, always surrounded by Schwann cells. Amyelinic neurites, clusters of Schwann cells, fibroblasts and numerous foci of GFA positive astrocytes composed the tumor. These features were compared with the rare previous electron microscopic reports of cerebral neuronal tumor of the central nervous system. A dysembryoplastic origin from the neural plate was retained to explain the growth of such a complex tumor.
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17 children presenting with craniopharyngioma were studied from 1970 to 1980. Presenting symptoms were usually reflecting endocrine dysfunction (13 of 17 cases), such as growth retardation, weight abnormalities, diabetes insipidus and hypothyroidism. Unfortunately, these signs were most often misinterpreted (10 of 13 cases) so that when diagnosis was made dissociated hypopituitarism was present. Further impairment of endocrine function always followed surgical excision. In most children pituitary insufficiency becomes complete and permanent. These results are similar whatever the tumor site and surgical procedure. Growth goes on and puberty occurs without treatment in a few cases but in the majority of them substitutive therapy is necessary.
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This report presents the ultrastructural study of a germinoma of the third ventricle occurring in a 13 year old boy. The electron microscopic data showed similarities linking this tumor to gonadal and mediastinal germinomas and emphasized the exceptional glycogenic storage in tumor and stroma cells. Another morphological analogy was the intense macrophagic activity that led to tumor cell phagocytosis. The authors discuss the meaning of such an immune response, which is usually not observed in cerebral tumors.
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An analysis was made of 175 cases of pediatric nontumoral hydrocephalus. CT scan by itself permits a precise diagnosis in only one-third of the cases. However, when the clinical context suggests that hydrocephalus is secondary to certain conditions such as infection, bleeding, or trauma, CT scan may suffice, unless more advanced investigation permits more efficient treatment. When hydrocephalus is associated with brain malformations, however, more complete investigation is needed for a better understanding of the condition. Conventional contrast studies are quite safe, especially ventriculography which is easy to perform in this type of patient, who is usually investigated during the first 12 months of life.
The intracranial, liquid-containing cysts in children (excluding tumor and parasitic cysts) are relatively frequent in neurosurgical practice. They raise several problems about their nosology, etiology, clinical and radiological diagnosis, and treatment which are analyzed in a series of 36 cases of supratentorial cysts. The most frequent clinical feature is increased head circumference (22 cases) followed by epileptic fits (18 cases), as well as mental and motor retardation (19 cases). Fundi were found abnormal in only one case out of three. Electroencephalogram was abnormal in almost every case, showing either spikes, spikes and waves or localized slow waves, or an asymmetric depression of the electrical activity. Radiological investigation is essential for diagnosis. Plain radiographs of the skull may show an asymmetry (11 cases). Carotid angiogram and pneumoencephalography give the diagnosis of the lesion without accuracy as to the histology. Computerized axial tomography shows the position of the liquid cavity within the head. The surgical approach (simple shunting [6], direct approach [19], or both [7]) should be carefully considered according to anatomical variety and age of the child. An anatomical classification is proposed, based on the radiological, surgical and pathological findings. Three types of cysts are defined: external cysts (cortical or extracortical) which may or may not be communicating with the ventrioles or the subarachnoid space; internal cysts which again may or may not be communicating; and the corticoventricular cysts. The ultrastructural study represents further progress in the attempt to define the exact anatomical type.
The authors report their experience about 60 patients who suffered of a spondylolisthesis and who were treated by a surgical procedure. A posterior approach has been used for all the patients. After a brief review of the clinical and radiological features of the disease, the authors emphazise the advantages of the posterior approach; only this technique allows a correct access both to the bone and the nerve roots lesions. Two types of operations are proposed:--the ablation of the posterior arch of the concerned vertebra (Gill's operation).--a posterior arthrodesis by mean of an homograft, using one of the following technique: an Albee grafting a Bosworth grafting an ilio-transversary grafting. The results are considered from a clinical, and an anatomo-radiological point of view.
Cerebral tomography allows the delineation of areas modified by edema or necrosis, the visualization of calcifications undetectable by standard x-ray examination, extra- or intra-cerebral blood effusions and neoformations of the cerebrum or of its envelopes. It also reveals slight or localized cerebral atrophies that other neuro-radiologic techniques sometimes failed to detect. It appears therefore worthwhile to reconsider pediatric neurology according to this new type of investigation. Its sole difficulty in young children, is the requirement of a half hour immobility, best obtained by general anesthesia. However, the rapid improvements of this technique will probably permit to overcome this slight drawback.
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This study reports 19 cases of extradural hematomas in infants for their relative frequency in head injury of children, their clinical findings, their particular evolution and the therapeutic problems specific to this age.