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J Goasguen

Publications and source records attributed to J Goasguen.

At least 19 recordsLinked to original sources
PubMed1991

Gingival biopsy in diagnosis of inborn storage diseases: a case of aspartylglycosaminuria.

The case described is that of a 9-yr-old girl presenting with aspartylglycosaminuria. Diagnosis of this lysosomal storage disease was guided by histologic study of gingival specimen sampled in the course of dental care. Transmitted electron microscopy (TEM) revealed many vesicles and cellular inclusions, altered collagenic formations, associated with abnormal extracellular matrix. Gingival biopsy is easily performed, is noniatrogenic, leaves nor scar, and could be properly used to help diagnose metabolic diseases in children.

Acetylglucosamine↗
PubMed1991

[Autoimmune thrombocytopenic purpura and pregnancy: significance of fetal blood punction].

The authors report three cases of percutaneous fetal blood sampling at 38 weeks of pregnancies complicated by immunologic thrombocytopenia. In each case, the purity was perfect which is essential for using the examination with a view to the obstetrical management. The birth is programmed 24 or 48 hours later. In two cases, the neonatal control is in agree with the fetal blood sampling. In the third observation, the fetal platelet count in 142,000/mm3 and the newborn control 55,000/mm3: the only explanation is a very active process as usually state with newborns. There was no accident, and these samplings led to natural births. This approach seems to be the more effective for the evaluation of fetal platelet count and allow the choice of birth management. A cesarean section is decided in case of fetal thrombopenia lower than 50,000/mm3.

Adult↗
PubMed1990

[Peripheral neurofibromatosis and involvement of lumbosacral nerves. Value of imaging].

We report a case of giant plexiform neurofibromatosis revealed by a sensori-motor, axo-myelinic polyneuropathy. Only the lumbar and sacral spinal nerves were involved in this peripheral form of Von Recklinghausen's disease. The polyneuropathy was due to compression of the nervous fibers by the neurofibromatous tumors. Ultrasonography DTPA 99 m Technetium scintigraphy, CT and MRI were the diagnostic procedures. MRI proved superior to CT in demonstrating the extent of the lesions. Biopsy confirmed the diagnosis. This form of neurofibromatosis did not involve the central nervous system, cranial nerves of cervicothoracic spinal nerves and roots; nor was there any visceral involvement, particularly endocrine neoplasia.

Adult↗
PubMed1990

[Complex temporal partial status epilepticus].

The cases of non convulsive, complex, partial ailment are a cause of a confusional state. Such a case on an adult is here reported, and its clinical presentation was a unvarying forgetfulness together with elements of a frontal syndrome. The aetiology was most probably a viral meningo-encephalitis. Clinical semiology of these "EMPC" is variable, either made of partial, recurrent attacks, sometimes with automatisms, or made of a continuous, possibly fluctuating, confusional state. Attacks shown on the EEG are partial or generalized with a variable start, sometimes bilateral, even continuous or discontinuous. The onset is most often temporal or frontal. The cause is very rarely found out. The evolution is usually good, but extended deficiencies of memory are described, linked to the duration (more than 12 hours) of EMPC. Therefore, the treatment must be precocious using diazepam or phenytoin.

Encephalitis, Arbovirus↗
PubMed1990

[Cryptococcosis in Bujumbura, Burundi. Apropos of 80 observed cases in 42 months].

Cryptococcosis is a systemic fungal disease and meningeal or meningoencephalitis involvement is the most serious complication. This is a retrospective analysis of 80 patients admitted from December 1983 to October 1985 (30 cases) and June 1987 to December 1988 (50 cases) in hospital of Bujumbura, Burundi, Central Africa. All patients have an AIDS. Clinically, the meningeal and meningoencephalitis attack prevails in 87% cases. The diagnosis in our study is essentially based on the examination of the CRL. Before the new antifungals, the treatment involved the association of amphotericin B and 5-fluorocytosine, during 6 to 8 weeks. This treatment was badly tolerated and the second falls, when the therapy was stopped, were frequent. Presently, the fluconazole is the best treatment of this affection (ailment?): it enables maintenance therapy for a disease in which the risks of recurrence and reinfection by the environment are not negligible in Africa.

Acquired Immunodeficiency Syndrome↗
PubMed1989

Overview of initial clinical studies with intravenous and oral GR43175 in acute migraine.

The novel 5-HT 1-like receptor agonist GR43175 has been evaluated as a treatment for acute migraine in a series of open, dose-ranging and controlled clinical trials. Patients with severe attacks of migraine have attended special pain or headache clinics for treatment and assessment. Given intravenously as a bolus, GR43175 is capable of aborting all migraine symptoms within 10-30 min in over 90% of cases at a dose of 64 micrograms/kg. Characteristic transient and reversible side effects with such a regimen include feelings of heaviness, pressure and occasionally warmth or tingling which can be diminished by extending the duration of drug administration to a short infusion. Initial dose-ranging studies with a dispersible tablet formulation of GR43175 have revealed an efficacy of 70-85% within 2 h with doses of 70-280 mg. Furthermore, tolerability is excellent. These encouraging early results warrant larger-scale controlled studies of GR43175 in acute migraine.

Administration, Oral↗
PubMed1988

Isolation of HIV in a seronegative demented patient without symptoms of immune deficiency.

A 60-year-old male patient, originating from West Africa, developed acute and regressive neurologic symptoms associated with aphasia, apraxia, acalculia, behavioral impairments, and an epileptic phase. Eighteen months after the onset of the disease, the patient was almost normal. All along the clinical course, biological abnormality patterns were minor. We noted only a mild neutropenia in the blood. We also observed a weak lymphocytosis and elevated protein content in the cerebrospinal fluid. Electroencephalogram examination revealed slow waves which disappeared after remission. A weak ventricular dilatation was detected on CT scan. Neither vascular, nor tumoral, nor a classical infectious origin could be identified. While the patient was seronegative to HIV, a HIV-like virus was isolated twice from his peripheral blood lymphocytes during the disease. Eighteen months later, the patient remained seronegative. He developed neither AIDS nor immunodeficiency. The subtype of HIV has been isolated and characterized, and its neurotropism is being investigated.

Antibodies, Monoclonal↗
PubMed1985

[Multiple neuritis].

Explore the source record for details and available documents.

Diabetic Neuropathies↗
PubMed1979

[Quantitation of myelofibrosis in blood diseases by electronic image analysis (author's transl)].

Normal and pathologic reticulin networks colored black by silver nitrate can be automatically quantitated by electronic image analysis. By using this technique, different parameters can be obtained, such as the average density, the surface of network meshes, the thickness of the fibers, the complexity of the reticulum, and the heterogeneity of the myelofibrosis distribution. All of these parameters were obtained in 83 osteomedullar biopsies of blood diseases (primary splenomegaly, chronic myeloid leukemia, polycythermia vera, acute leukemia, and aplastic anemia). We have shown that there is no relation between the different parameters obtained and the medullary richness, hematopoietic center, or patient survival. On the other hand, the histomorphometric parameters can be used to distinguish acute leukemia and chronic myeloid leukemia myelofibrosis, while the parameters in primary splenomegaly are shown to be very heterogeneous.

Acute Disease↗