Idiopathic CD4+ T-cell lymphocytopenia and subacute inflammatory demyelinating polyradiculoneuropathy.
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Anemia, Hemolytic↗
Biomedical subjects
C Vital
Publications and source records attributed to C Vital.
High-grade B-cell cerebral lymphoma in a patient with anti-myelin-associated glycoprotein IgM paraproteinemic neuropathy.
A 74-year-old woman with a sensory neuropathy and IgM M-protein monoclonal gammopathy of undetermined significance developed a fatal B-cell cerebral lymphoma. CSF protein immunofixation revealed intrathecal secretion of a paraprotein of the same heavy- and light-chain isotypes as the serum monoclonal component (IgM-lambda). Reactivation of Epstein-Barr virus was present in the lymphoma cells. Different factors may be involved in the preferential malignant development of the monoclonal B-cell clone within the CNS.
Aged↗
Lymphoplasmacyte-rich meningioma in a child. Case report.
A rare case of lymphoplasmacyte-rich meningioma observed in a young girl is reported. The first clinical manifestations of the disease were seizures. Neuroradiological images favored the existence of a meningioma. Abnormalities in the patient's blood chemistry, principally including hypergammaglobulinemia and inflammatory syndrome, were associated with the disease. The tumor was histologically confirmed as meningioma with massive infiltrates of type B lymphocytes. The pathophysiology of the conspicuous lymphoplasmacyte infiltrates, responsible for peripheral blood abnormalities, has remained poorly understood. Alternative diagnostic hypotheses of masses that mimic this type of meningioma are discussed.
Brain Neoplasms↗
[Paraganglioma of the cauda equina. Clinical aspects and MRI. Apropos of a case].
The clinical and imaging features of a paraganglioma of the cauda equina are presented. A peridural anesthesia revealed the cauda equina compression syndrome in a 68 years old man who presented first with a urinary retention. Total surgical excision of a long round-shaped encapsulated tumor was uneventful. Macroscopically this lesion filled up the spinal canal and resembled to a meningioma. It was firmly attached to the filum terminale. Adjacent large arterialized vessels visible on MRI were spared. Although the diagnosis could be evoked pre-operatively on some suggestive patterns on the MRI, the pathological findings finally confirmed the diagnosis of paraganglioma. Along with the evidence of specific light microscopic features, the use of an immunocytochemical demonstration of synaptophysine in these tumors allows a confident diagnosis to be made.
Aged↗
[Supra-tentorial low-grade astrocytomas in adults. Prognostic factors and therapeutic indications. Apropos of a series of 141 patients].
An abundant literature provides informations upon the prognosis of supratentorial low grade (grade II) astrocytomas, but the series are quite heterogeneous in terms of clinical material, neuropathological evaluation and statistical methods of analysis. So, outcome, prognostic factors and therapeutical indications are poorly defined. A retrospective study of 141 adults patients suffering ordinary low grade astrocytoma diagnosed in our unit between 1978 and 1988 was conducted. A follow up of 5 years or more, since neuropathological diagnosis, was mandatory for inclusion. Endpoint of statistical analysis was duration of survival. Results were expressed after uni and multivariates analysis. Clinical and morphological features of our series were comparable to those previously reported in the literature. Median survival time was 52 months. Considering age at diagnosis, survival curve analysis showed highly significant differences (p < 0.0001) and established three prognostic classes of age (before 50, between 50 and 60, and after 60 years of age). Comparisons of survival curves showed significant statistical differences (p < 0.0001) according to pretreatment functional status. Analysis of a clinical condition using a functional scale is more powerful, from a statistical point of view, than an individual analysis of each constituting parameters. In this series a good correlation was found between functional status and age at diagnosis. Neurological deficit was more correlated to age than to tumor location. Multivariate analysis, using the Cox model, defined some parameters acting independently on duration of survival: fronto-parietal location (p < 0.0001), pretherapeutical functional status (p < 0.001), age at diagnosis (p = 0.001), deepseated or multicentric tumor, mass effect (p < 0.01), frontal location (p < 0.02), total surgical removal (p < 0.05). Non significant parameters were: radiotherapy, time before diagnosis, age of onset, sex, contrast enhancement on CT scan. Our results are in agreement with previously reported data confirmed literature data concerning prognostic factors characteristics of the host (age), characteristics of the tumor (location) and expressing the influence of the tumor on the host (functional status). Our series documented prognostic clinical forms of these tumors (function of age, of performance status, and of tumor location). Our results confirm the relative inefficacy of therapeutic weapons, considering the population as a whole, on duration of survival. Based on our results and on recently published data, a decision tree analysis was proposed for management of grade II astrocytomas.(ABSTRACT TRUNCATED AT 400 WORDS)
Adult↗
[Cerebral amyloid angiopathy].
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Alzheimer Disease↗
[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↗
[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↗
Simultaneous development of two different B-cell lymphomas in a patient with acquired immune deficiency syndrome evidenced by molecular analysis.
We report a case of a man positive for the human immunodeficiency virus who rapidly died of lymphoma with cerebral, lung, and pericardic involvement. After autopsy, histopathological study of the different tumor sites showed the same morphological feature of immunoblastic lymphoma, with large areas of necrosis. In situ hybridization showed monotypic kappa light chain mRNA within lung lymphoma cells and monotypic lambda light chain mRNA within cerebral lymphoma cells. Polymerase chain reaction analysis showed two different immunoglobulin heavy chain gene rearrangements for lung and cerebral lymphoma. Here the simultaneous association of two malignant lymphomas derived from different B-cell clones indicates that molecular analysis of different tumor sites can distinguish between dissemination of the same lymphoma and simultaneous proliferation of different malignant B-cell clones.
Acquired Immunodeficiency Syndrome↗
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↗
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↗
Expression patterns of human PNS myelin proteins in neuropathies associated with anti-myelin antibodies.
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Autoantibodies↗
[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↗
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↗
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↗
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↗
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↗