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Biomedical subjects

A Vital

Publications and source records attributed to A Vital.

At least 73 records · Page 4Linked to original sources
PubMed1995

[Necrotic purpura of the head disclosing Churg-Strauss syndrome].

INTRODUCTION: Among the cutaneous manifestations of the Churg-Strauss syndrome, palpable purpura of the extremities is the most common. Although they are not specific, sub-cutaneous nodules of the scalp are the most distinctive lesions. CASE REPORT: A 58 year-old man, with a 7-years history of asthma, developed profuse cutaneous lesions involving the face and the scalp, initially erythematosus and vesiculous, then purpuric and necrotic which led to the diagnostic of Churg-Strauss syndrome. COMMENTARY: Cutaneous lesions occur frequently during the Churg-Strauss syndrome and may reveal the disease as in our patient. However, the initial clinical presentation was uncommon with erythematosus and vesiculous lesions mimicking primary herpes infection. Cutaneous biopsy was useful leading to an early diagnosis.

Churg-Strauss Syndrome↗
PubMed1995

Lysolecithin-induced demyelination in primates: preliminary in vivo study with MR and magnetization transfer.

PURPOSE: To study bystander demyelination in multiple sclerosis with an experimental in vivo model of toxic demyelination. METHODS: Toxic demyelinating lesions were created in two monkeys by injection of lysophosphatidylcholine in the centrum semiovale. Follow-up was done clinically and with serial MR studies, including T2-weighted and gadolinium-enhanced T1-weighted images and measurement of magnetization transfer ratio, until the animals were killed at days 14 and 34, respectively. Light and electron microscopy analysis was compared with MR data. RESULTS: Interval measurement of magnetization transfer ratio during the course of the experiment revealed a maximum decrease at day 7 to day 8, associated with the greatest clinical manifestations. The lowest values of magnetization transfer ratio correlated with histopathologic findings of myelin and axon destruction. Magnetization transfer ratio measurements appear to be sensitive to macromolecular destruction and specifically to membrane disorganization. At no time was gadolinium enhancement observed in this model of toxic demyelination. CONCLUSION: Preliminary results of this study indicated that magnetization transfer is a good technique to follow in vivo matrix destruction in brain parenchyma lesions. The results suggest also that phases of toxic demyelination in multiple sclerosis might not show gadolinium enhancement. Differentiation between demyelinating activity and associated inflammation in multiple sclerosis lesions should be considered in further in vivo work.

Animals↗
PubMed1995

[Pseudo-tumoral lesions of multiple sclerosis].

Three patients underwent a cerebral biopsy for clinical and radiological features suggesting a brain tumor. In all cases, the lesion contained large pleomorphic astrocytes that might have led to the erroneous diagnosis of grade II astrocytoma. However, the presence of foamy macrophages, demyelination and the relative preservation of axons led to the definitive diagnosis of multiple sclerosis which was confirmed by the subsequent clinical course.

Adult↗
PubMed1995

[Significance of polyglucosan bodies in neuropathology. Clinico-pathologic study of seven cases].

Seven observations, having in common an accumulation of polyglucosan bodies in the nervous system, are reported. In 2 cases of adult polyglucosan body disease, characteristic polyglucosan bodies were found on the peripheral nerve biopsies, and also on the cerebral biopsy in one of these patients who was demented. The 5 other cases presented medically intractable temporal lobe epilepsy. Recovery was obtained by a temporal lobectomy in which a massive accumulation of polyglucosan bodies was discovered. These personal observations are discussed with regard to other conditions characterized by accumulation of polyglucosan bodies in the nervous system. The non specificity of these formations is emphasized.

Adult↗
PubMed1994

Glial and neuronoglial malformative lesions associated with medically intractable epilepsy.

In a large series of 116 cortical resections for treatment of medically intractable epilepsy, 10 glial hamartomas and 11 neuronoglial malformative lesions were found. Glial hamartomas were astrocytic in 3 cases, oligodendrocytic in 6 and mixed oligoastrocytic in 1. Neuronoglial lesions corresponded to "focal cortical dysplasia" in 6 patients and to "microdysgenesis" in 5 others. This study focuses on the various neuropathological presentations of these malformative epileptogenic lesions, and on correlations with neuro-imaging data.

Adolescent↗
PubMed1994

Uncompacted myelin lamellae in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome. Ultrastructural study of peripheral nerve biopsy from 22 patients.

Mechanisms of peripheral neuropathies in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome are poorly understood. A peripheral nerve biopsy was performed in 22 patients. Of these 9 had histological features of Castleman's disease on lymph node biopsies, and 19 had a monoclonal lambda light chain in their serum. Certain nerve fragments were paraffin embedded, others were frozen and studied by direct immunofluorescence, and others were fixed for ultrastructural examination. Paraffin-embedded fragments did not show any amyloid deposits, and at direct immunofluorescence there was no immunoglobulin fixation. At ultrastructural examination, features of uncompacted myelin lamellae (UML) were present in 19 patients, and their frequency varied from 1% to 16% of myelinated fibres. Up to now UML have been reported only in 7 patients with POEMS syndrome in the literature. UML have also been noticed in a few cases of inflammatory demyelinating polyradiculoneuritis and inherited tendency to pressure palsy.

Aged↗
PubMed1994

Severe axonal polyneuropathy with onset in the postpartum period.

We report two patients who presented severe polyneuropathy in the postpartum period. Electrophysiological studies evidenced an axonal process which was associated with proximal demyelination in the second patient. In both cases, a peripheral nerve biopsy showed severe axonal Wallerian-like degeneration and no feature of demyelination. The first patient had a dramatic loss of myelinated fibres, and severe disability persisted for several months. These two patients are different from cases of acute or chronic inflammatory demyelinating polyradiculoneuropathy previously reported in relation with pregnancy.

Adult↗
PubMed1994

[Histopathology of 110 cortical resections for drug-resistant epilepsy].

Histopathologic findings in 110 cortical resections performed for medically intractable epilepsy are reported. The abnormalities observed in our series fell into seven categories: vascular lesions, tumors, non-vascular malformative lesions, focal accumulation of polyglucosan bodies, non infectious inflammatory lesions, hippocampal sclerosis, and mild non-specific lesions. There was no microscopic abnormality in 14 cases. Several patients presented a double pathology, most often an hippocampal sclerosis as one part of the dual pathology.

Anticonvulsants↗
PubMed1994

Peripheral neuropathy after autologous blood stem cell transplantation for multiple myeloma.

We report a case of peripheral neuropathy occurring after autologous blood stem cell transplantation (ABSCT) for multiple myeloma. The patient, free of neurological symptoms, was transplanted in partial remission, and achieved a complete remission after transplantation. A severe peripheral, symmetric, distal sensori-motor polyneuropathy appeared at day 25 and worsened progressively until commencement of corticosteroid therapy. A peripheral nerve biopsy showed endoneurial cellular infiltrates which were predominantly composed of T cells identified by immunocytochemistry. Ultrastructural examination showed acute axonal damage. Electrophysiologic studies performed before and during the treatment were consistent with a severe axonal degeneration and showed a marked improvement, concomitant with the favorable clinical outcome. This is the first report of peripheral neuropathy after ABSCT.

Axons↗
PubMed1993

The presence of particles resembling human T-cell leukemia virus type I at ultrastructural examination of lymphomatous cells in a case of T-cell leukemia/lymphoma.

BACKGROUND: Cases of adult T-cell leukemia/lymphoma (ATLL) resulting from human T-cell leukemia virus type I (HTLV-I) have been observed mainly in the southern part of Japan. Recently, the authors performed a second examination of cutaneous, muscle, and nerve biopsy specimens from a French white woman who died of ATLL in 1979. METHODS: A 67-year-old white woman had a lymphoma diagnosed on a lymph node biopsy. She then had acute pains and a thickened skin on both legs. Blood examination showed a leukocyte count of 16,000/ml with 75% leukemia T-cells. Biopsies were performed on the antero-external surface of the right leg. She died after 2 years of illness. RESULTS: Lymphomatous infiltrates of T-cell origin were seen in the dermis, between muscle fibers, and in a peripheral nerve. The recent ultrastructural examination of a few vacuoles located in the cytoplasm of certain lymphomatous cells showed rounded structures mixed with larger virus-like formations having a central nucleoid and spike material around the envelope. Polymerase chain reaction experiments performed on deparaffinized sections demonstrated the presence of a tax sequence homologous to that of HTLV-I. Other structural genes were not detected. CONCLUSIONS: These results contrast with other ultrastructural studies in which HTLV-I was detected only after cultivation of leukemia cells from patients with ATLL. This case probably resulted from an HTLV-I variant.

Aged↗
PubMed1993

Establishment of HTLV-I-infected cell lines from French, Guianese and West Indian patients and isolation of a proviral clone producing viral particles.

Human T-cell leukemia virus (HTLV-I) induces adult T cell leukemia/lymphoma (ATL) and a chronic neurological disease named either tropical spastic paraparesis (TSP) or HTLV-I associated myelopathy (HAM). We report here the establishment and characterization of eight HTLV-I-infected lymphoid cell lines derived either from patients with TSP (5) or from asymptomatic carriers (1). Southern blot analysis of T cell beta chain gene rearrangements indicates that all cell lines are composed of clonal populations. The same type of analysis performed with HTLV-I-specific probes showed that they harbor 1 to 5 copies of full length proviruses often associated with deleted proviruses with a restriction map for BamHI, HindIII, PstI and SacI restriction enzymes resembling those of HTLV-I previously isolated from Japan and Caribbean area. One of the cell lines, 2060, derived from a TSP patient was shown to express a relative large amount of virus easily transmissible to fresh peripheral and cord blood lymphocytes. The full length proviral genome contained in this cell line was cloned and used in transient expression experiments. We showed that the cloned provirus was able to direct the synthesis of the major structural viral proteins, the protease and the tax and rex regulatory proteins. The structural viral proteins could be assembled into free particles detected in the culture medium of transfected cells. Although the infectivity of these viral particles remains to be determined, this new clone can be employed to examine the cell types in which this TSP-derived provirus directs viral protein synthesis and eventually replicates. It should also prove of value in studies on the early cellular events induced by viral products.

Animals↗
PubMed1993

Malignant infiltration of peripheral nerves in the course of acute myelomonoblastic leukaemia: neuropathological study of two cases.

Two patients suffering from acute myelomonoblastic leukaemia developed clinical evidence of peripheral nerve involvement. In both cases, a peripheral nerve biopsy revealed endoneurial cellular infiltrates which were identified as leukaemic cells by immunocytochemistry. Ultrastructural studies showed mainly axonal damage. Moreover, in one patient, a few features of active demyelination were also observed.

Action Potentials↗
PubMed1993

Isolated peripheral nerve relapse masquerading as Guillain-Barré syndrome in a patient with acute lymphoblastic leukemia.

We report a 30 year old patient with acute lymphoblastic leukemia (ALL) whose leukemic relapse presented as an isolated symmetrical peripheral neuropathy with facial diplegia. Initially, this was consistent with a Guillain-Barré syndrome but the peripheral nerve biopsy revealed leukaemic infiltration. This was followed by a systemic relapse. Reports of peripheral nerve infiltration are scarce and to the best of our knowledge this is the first documented case of peripheral polyneuropathy as a presenting manifestation of ALL relapse.

Adult↗
PubMed1993

[Polysaccharide bodies: an unusual finding in a case of temporal epilepsy. Review of the literature].

Massive occurrence of polyglucosan bodies (PB) was found in the surgically removed temporal lobe of a 34 year-old woman presenting with complex partial seizures. The term of PB was proposed in order to group Lafora bodies (LB), corpora amylacea (CA) and Bielschowsky bodies (BB) on the basis of their biochemical similitude. A rigorous histochemical differentiation between these anomalies appears to be impossible at present. LB, BB and CA are mainly made up of glucose polymers i.e. polyglocusans. The pathway(s) leading from glycogen accumulation to PB formation is still unknown. PB are a hallmark of two diseases: Lafora disease and adult polyglucosan body disease. PB have occasionally been reported in rare cases of a variety of other neurological diseases. In all cases they were located within the neurons. This site characterizes Lafora bodies. BB are intraneuronal inclusions but restricted to neurons of the external pallidum. CA occur predominantly in the astroglia during the course of ageing. The significance of these structures depends on their regional distribution. The resemblance does not imply a common etiology for all conditions in which such bodies occur; it is probably due to the sharing of the final path in their causative pathway. Our case does not correspond to any of the classical diseases in which PB have been found. In our opinion, the patient exhibited a localized form of glycogen storage disease.

Adult↗
PubMed1993

[Peripheral nerve biopsy].

This article provides technical data obtained by regular practice of peripheral nerve biopsies in the neuropathology department, and discusses their indications. Details include site of biopsy, surgical procedures, and the choice of histopathologic techniques.

Biopsy↗
PubMed1992

Occurrence of active demyelinating lesions in children with hereditary motor and sensory neuropathy (HMSN) type I.

In three children with dominant hereditary motor and sensory neuropathy type I, peripheral nerve biopsy showed the classical lesions of segmental demyelination with remyelination and onion bulb formations. In two cases, ultrastructural examination also demonstrated numerous characteristic features of active demyelination. The presence of active demyelinating lesions suggests an autoimmune attack superimposed on the course of a chronic genetic disease.

Child↗
PubMed1992

Three cases of primary cerebral lymphoma in AIDS patients: detection of Epstein-Barr virus by in situ hybridization and Southern blot technique.

Three cases of primary cerebral lymphoma in acquired immunodeficiency syndrome were studied. Tumoral fragments taken at autopsy were frozen and studied by the Southern blot technique (SBT). Other tumoral fragments were fixed in formalin, embedded in paraffin and used for in situ hybridization (ISH) with biotinylated probes for DNA of Epstein-Barr virus (EBV). ISH was positive in each case with a spotty nuclear labelling of certain tumoral cells. SBT evidenced a clonal rearrangement of the immunoglobulin heavy chain gene in each case. In addition, EBV DNA was detected in each frozen fragment with only one restriction pattern, indicating that the EBV- infected cell population was a clonal expansion of a progenitor cell.

Adult↗