[Nasopharyngeal carcinoma and hereditary angioneurotic edema].
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Adolescent↗
Biomedical subjects
C Raybaud
Publications and source records attributed to C Raybaud.
M4 protocol for cerebellar medulloblastoma: supratentorial radiotherapy may not be avoided.
The main goal of the M4 protocol was to evaluate the efficacy of treatment excluding supratentorial radiation in patients with newly diagnosed medulloblastoma. All patients underwent surgical resection and received postoperative chemotherapy. Chemotherapy was adapted to the initial staging and prognostic factors (Group A: good-risk; Group B: poor-risk). Chemotherapy was started early after surgery, and consisted of two courses of the "eight drug in one day" regimen and two courses of high dose methotrexate. Radiotherapy was delayed until 5 (Group B) to 7 (Group A) weeks after the first course of chemotherapy. Radiotherapy was administered only to the posterior fossa and the spinal axis. Only 3/16 patients (18%) are alive and disease-free with a mean follow up of 6 years. The site of progression was supratentorial in 9 out of 13 patients and three patients had spinal and/or cerebrospinal fluid relapses. Only one patient had isolated posterior fossa relapse. The mean time to relapse was 484 days. We conclude that the chemotherapy regimens used in the M4 protocol do not allow the reduction of irradiation fields in patients with cerebellar medulloblastoma. In spite of long-term side effects on neurocognitive functions, supratentorial radiotherapy should remain a major component of medulloblastoma treatment.
Adolescent↗
Neurological findings and seizure outcome in children with bilateral opercular macrogyric-like changes detected by MRI.
The authors studied 10 patients aged between six and 23 years (mean age 14 years 5 months) with magnetic resonance imaging, which detected bilateral 'macrogyric-like' maldevelopment of the insulo-opercular regions. The data confirm that biopercular gyral anomaly, associated with mental retardation, pseudobulbar palsy (cortical or central) and epilepsy, represents an anatomo-clinical syndrome. Nevertheless, a wide clinical spectrum was found varying from pictures correlating with the topography and extent of the MRI-detected anomaly to conditions indicating wider cerebral involvement. Epilepsy, varying greatly in age at onset and severity, consistently influenced the prognosis for five patients with diffuse EEG abnormalities and intractable seizures with falls. Anterior callosotomy relieved such seizures in one case.
Adolescent↗
Epilepsy and focal gyral anomalies detected by MRI: electroclinico-morphological correlations and follow-up.
The authors studied 10 patients (mean age 15 years 6 months) with localized developmental gyral disorder detected by MRI. There were two groups of major malformations. Seven patients (group 1) had unilateral 'macrogyric-like' insulo-opercular changes, one of whom died early in life and had extensive microgyria. The six others had mental retardation and epilepsy, three of whom had focal neurological signs. Age at onset of epilepsy varied greatly. Clinical and EEG data suggested a wider cerebral involvement than recognized on MRI. The remaining three patients (group 2) had abnormal gyri of variable topography and extension, with bulging grey matter and ventricular deformity. One had mental retardation, another had neurological signs. All had intractable complex partial seizures and focal EEG anomalies correlating with the MRI lesion site, pointing to a well-defined epileptogenic zone. No clinical or EEG evidence of significant malformation in the remaining brain tissue was observed. Ablative surgery was beneficial for one patient; focal cortical dysplasia was the pathological substrate.
Adolescent↗
[MRI of the spinal epidural fat in pathology].
The authors report about their experience with the merits of the normal systematization of epidural fat with MRI. On sagittal sections, this fat has a variable appearance, but its location along the spinal canal is constant. On axial sections, its morphology is suggestive of the level of section: cervical, upper or lower thoracic, lumbar. Some changes in this fat are precious data to explain local hypertrophy (scoliosis) or to locate an intra- or extradural process.
Adipose Tissue↗
[Apropos of the tumors of the neck. Diagnostic considerations].
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Adenoma↗
[Ewing's tumor: current knowledge and ignorance].
The diagnosis of Ewing's malignant tumor in the young still raises major problems, either from a clinical point of view because of its rarity, its pluri-potentiality and various symptoms, or on imaging because of its numerous pitfalls. Accordingly the disease is often misdiagnosed as osteomyelitis. Only a high quality biopsy can determine histological diagnosis of undifferentiated small round cell tumor. The chromosomic study shows a specific (11;22)(q24;q12) translocation, and immunocytochemistry and molecular biology show the tumor's neuroectodermal origin. For 20 years, therapy tended toward first line tumor chemoreduction, followed, when appropriate, by complete resection and orthopedic reconstruction of the bone. Radiotherapy, which is responsible for long-term sequelae is now increasingly restricted to inaccessible or incompletely excised tumors. More intensive chemotherapy is being examined in patients with poor prognosis factors such as a negative response to induction chemotherapy, a significant mass or metastases. As a result of new strategies, disease-free survival rate is now between 60-70%. The management of this disease is highly multidisciplinary and patients will now be included in multicentric controlled therapeutic trials. Long term follow-up has to be carried out following completion of treatment.
Adolescent↗
[Drash syndrome].
The authors report one case of Drash syndrome: association of Wilms' tumor, nephropathy and genital abnormalities. The definition, prognosis and origin of this triad are discussed.
Cadaver↗
[Evaluation of nuclear magnetic resonance imaging in tridimensional acquisition in the investigation of prolactin microadenoma].
Twenty one patients with suspected prolactin-secreting microadenoma were evaluated with MRI. MRI is the most sensitive means for detecting focal microadenomas. In these patients who were clinically and endocrinologically considered to harbor a microadenoma, MR detected a focal pituitary signal abnormality in 100% when the patients had not previously taken bromocriptine therapy. On the other hand MR demonstrated a focal abnormality in only 30% of cases when the patients had been on dopamine agonist therapy: the MRI findings in the group of bromocriptine treated patients are not affected by neither the duration and dosage of the therapy or the delay between MR examination and the bromocriptine therapy discontinuation. Among the 9 cases in which MRI did not demonstrate a focal abnormality, MRI was strictly normal in 3 cases only; MR showed a localized expansion of the subarachnoid space in two cases; the pituitary gland was large and round, and had a homogeneous signal in one case; the pituitary gland had a heterogeneous signal in 3 cases. In our study the microadenoma had a high signal intensity on the precontrast T1 weighted sequence in 5 cases. The focal abnormality was not seen on the precontrast MR images in 2 cases. The microadenoma enhanced in 3 cases on the postcontrast three dimensional MR images. This MR technique allows thin section slices (1 mm) and therefore the detection of small focal abnormality of the pituitary gland (2.5 x 3 mm). Thus three points have to the emphasized: a) MRI always detected a microadenoma when the patients had never received a bromocriptine therapy.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
[Ataxic hemiparesis and simultaneous supra- and subtentorial hematomas].
In a chronically hypertensive woman with a rapidly developing right ataxic hemiparesis syndrome, computed tomography and magnetic resonance imaging (MRI) revealed two small unrelated hemorrhages: one in the posterior limb of the left internal capsule, the other in the right cerebellar hemisphere. In this patient, the ataxic hemiparesis syndrome might have resulted either from the association of the two lesions or from the capsular hemorrhage alone. The rarity of simultaneous hypertensive hematomas and the value of MRI in the diagnosis are underlined.
Aged↗
MRI study of brain myelination.
Brain development in infants is characterized by growth and myelination. Myelin is a cell membrane devoid of MRI signal; the MRI images obtained at different stages of myelination result from changes in brain tissue water content, from the multiplication of glial cells which precedes myelination (the so-called myelination gliosis), and from the accumulation of lipid myelin precursors contained in cells. T1-weighted sequences are used for the "premyelination" process and T2-weighted sequences for myelination proper. The development of myelination in the white matter is sequential, precisely determined, identical in all individuals, and it has been well studied by histologists. In vivo, myelination in infants is shown at MRI as the same precise sequence but with some changes in time towards the end. The myelination process takes place at different times and different speeds in different brain regions, and for any given structure the speed of myelination varies in relation to time.
Age Factors↗
Use and interpretation of MRI in radio-anatomical evaluation of metabolic or toxic brain stem lesions.
Children with metabolic or toxic diseases affecting the brain stem were examined by MRI in an attempt to obtain images of brain stem systematization fibres and to determine their origin. A toxic cause may be envisaged in hazy, ill-systematized lesions, and a metabolic cause in well-systematized lesions.
Antineoplastic Agents↗
[Planning the care of the sick child].
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Child↗
Magnetic resonance imaging in gangliogliomas and gangliocytomas of the nervous system.
Gangliogliomas and gangliocytomas are rare and benign neuronal tumors which affect young subjects. This study concerns 3 cases of ganglioglioma and 3 cases of gangliocytoma explored by CT and MRI and confirmed by pathological examination. The most typical CT image was that of an often calcified contrast-enhanced cystic tumor. At MRI, each tumor emitted a low-intensity signal on T1-weighted sequences and a high-intensity signal on T2-weighted sequences. Contrast enhancement after gadolinium injection was frequent. Neuroradiology is not specific, and these tumors are usually diagnosed at pathology. However, the diagnosis may be considered in young patients with a history of old, drug-resistant partial epilepsy and having a contrast-enhanced, calcified cystic lesion in the temporal lobe or the cerebellum.
Adolescent↗
[Medulloblastoma in children. Current treatments and future perspectives].
The various treatments currently available for medulloblastoma are reviewed and modifications which have occurred over the last ten years are analyzed. Surgery is the first step of the treatment of a medulloblastoma. Recent advances in neurosurgical anesthesia and intensive care have substantially improved the quality and extent of exeresis and markedly reduced operative mortality (currently under 2%). Radiotherapy of the entire central nervous system should follow surgery in every case. Use of high energy radiations (together with improvements in surgical treatment) have increased survival rates to 50-60%. However, side effects are frequent. The modalities and timing of chemotherapy have also changed considerably over the last ten years; the different modalities currently used are reviewed, as well as the strategies available for reducing toxic effects and hematologic complications. Currently, chemotherapy is "sandwiched" between surgery and radiotherapy. Treatments under evaluation (monoclonal antibodies, immunotoxins, and immunomodulation) are discussed. The purpose of this therapeutic strategy for medulloblastomas is not only to increase survival rates but, above all, to improve the quality of life in survivors. Close cooperation between health care professionals of the different specialties involved and long term multicenter studies are indispensable if this goal is to be achieved.
Adolescent↗
Phase II study of cytarabine and etoposide in children with refractory or relapsed non-Hodgkin's lymphoma: a study of the French Society of Pediatric Oncology.
Twenty-five children or adolescents with relapsed or refractory non-Hodgkin's lymphoma (NHL) were included in this phase II study of the combination of cytarabine (ARA-C) 50 mg/m2/d by 12 hours continuous infusion day 1 to day 5, ARA-C 3 g/m2/d in 3 hours day 1 to day 4, and etoposide (VP 16) 200 mg/m2 daily from day 1 to day 4. Twelve patients had B-cell, 12 T-cell, and one non-T, non-B-cell lymphoma; according to Murphy's staging system, 15 had stage III and nine stage IV disease with bone marrow involvement at diagnosis. All had previously received ARA-C by push or continuous infusion. Two patients had received epipodophyllotoxins. At the time of the study, three children had initial refractory disease, 18 were in first relapse (14 on therapy), two in first refractory relapse, and two in second relapse (on therapy). The overall response rate (RR) was 60%: eight complete responses (CRs), seven partial responses (PRs) (two became CRs after a second course). The RR was 66% (four CRs plus four PRs) in B-cell and 54% (four CRs, three PRs) in non-B-cell NHL. It was 20% (one PR per five patients) in initial or relapsed refractory disease. In four patients with measurable CNS disease, there were three CRs. Duration of response was nonassessable since all the responding patients received high-dose polychemotherapy followed by autologous bone marrow transplantation (ABMT) (five are alive with long follow-up [FU]). Toxicity was marked mostly by pancytopenia for 2 weeks, and half the patients encountered a grade-3 infection. One severe diarrhea was observed. In conclusion, high-dose ARA-C (HD-ARA-C) and VP 16 are an effective regimen in relapsed NHL, especially with CNS disease, and its toxicity is acceptable with regards to the prognosis of the disease.
Adolescent↗
[Intra-arterial thrombolytic therapy of lower limb ischemia].
Between 1984 and 1989, 35 patients with recent arterial or graft occlusions have been treated with intra-arterial infusion using sequential association of Urokinase (U.K.) and Lys-Plasminogen. Occlusion was thrombotic in 68.5% of the cases ans embolic in 31.5%, involving 28 native arteries and 7 bypass grafts. The mean duration was 16 days (2 to 90). Continuous infusion of U.K.: 84,000 U.I./H and bolus of Lys-Plasminogen 15 microKatals every 30 minutes were delivered through a catheter embedded into the clot. Intra-venous heparin was always associated. The mean duration of lytic drug infusion was 8 H. Complementary arterial reconstruction by vascular surgery of percutaneous transluminal angioplasty was performed in 23% of the patients. Patients with recent alimentary tract bleeding, hemorragic stroke in the last six months or severe high blood pressures were contra-indicated. Complete lysis was obtained in 23 cases (66%), partial lysis in 7 (20%) and no lysis in 5 (14%). The clinical result was excellent in 24 cases (68.5%), good in 3 (8.5%) and bad in 8 (23%) in which amputation was always necessary. 5 local hematoma (14%) treated by surgery or transfusion and one death (3%) due to neurological complication occurring 24 hours after the end of the procedure were observed. The literature survey has shown that the results of low doses of Streptokinase (S.K.) local infusions were not better, and that higher doses of S.K. or U.K. delivered during a shorter infusion time increased the efficacy of lysis and decreased the rate of hemorragic complications. We have proposed the local thrombolytic treatment to the limb threatening ischemic cases when the traditional medical or surgical techniques where thought to be associated to a high risk of failure or complication. The specific indications are the acute or sub-acute ischemic situation due to atheromatous artery thrombosis, distal or old embolism where the Fogarty catheter is inefficient, and graft thrombosis. Severe acute ischemia with neurologic involvement are not good indications. Local thrombolysis can be successful on arterial occlusion even after one month duration.
Adult↗
[Undifferentiated renal sarcoma or Wilm's tumor (author's transl)].
About a case in a six year old Negro boy, the existence of a special pattern among the malignant renal tumors in children is emphasized; this tumor is marked from the Wilms' tumor by its peculiar behaviour (frequently poor evolution, high incidence of osseous metastasis in contrast with the rate occurrence of pulmonary involvement) and by a sarcomatous appearance at the pathologic examination. The classification and the suitable therapeutic management are discussed.
Child↗