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Biomedical subjects

M B Delisle

Publications and source records attributed to M B Delisle.

At least 73 records · Page 4Linked to original sources

[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↗

[Guillain-Barré syndrome associated with cytomegalovirus infection after kidney transplantation].

The Guillain-Barré syndrome associated with cytomegalovirus infection is a recognized entity of unknown pathogenesis. We observed two characteristic cases with acute polyradiculoneuropathy which occurred after renal transplantation. Both patients fulfilled the diagnostic criteria for Guillain-Barré syndrome: tetraparesis with diffuse areflexia and moderate sensorial signs, high protein level in cerebrospinal fluid without cellular reaction, slow or blocked nerve conduction and partial cure after several months delay. In two-thirds of such cases, the syndrome follows an episode of infection. Cytomegalovirus has been recognized as a possible association. The demyelinating polyradiculoneuropathy induced by cytomegalovirus has been reported essentially in immunodepressed patients infected with the human immunodeficiency virus in whom a pathogenic role for cytomegalovirus in peripheral nerves has been suggested. In our two cases, the cytomegalovirus infection occurred before onset of the acute polyradiculoneuropathy. In both cases, specific IgM antibodies and clear IgG positivization occurred before the first signs of neurological impairment even though neither the virus nor specific antibodies could be concomitantly detected in the cerebrospinal fluid. Despite the frequency of cytomegalovirus infections in transplant recipients, to our knowledge, only 9 cases of Guillain-Barré syndrome after transplantation have been reported and the exact mechanism remains to be ascertained. These two cases suggest that the onset of the Guillain-Barré syndrome after renal transplantation was closely related to cytomegalovirus infection. Adequate measures should be taken to quickly diagnose such cases in order to instore rapid treatment for the polyradiculoneuropathy. In addition, this particular situation of post-transplantation onset might help better understand the exact role of cytomegalovirus infection in the Guillain-Barré syndrome.

Cytomegalovirus Infections↗

Human beta-mannosidase deficiency associated with peripheral neuropathy.

Human beta-mannosidosis is an inherited lysosomal storage disorder described in only seven families. We present a further case in a black African 14-year-old boy with severely deficient beta-mannosidase activity, bilateral thenar and hypothenar amyotrophy, electrophysiologically demonstrable demyelinating peripheral neuropathy, and cytoplasmic vacuolation of skin fibroblasts and lymphoid cells. The clinical and biochemical features of our patient are compared to those of previously reported patients.

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↗

Papillary neoplasm of the endolymphatic sac in a patient with von Hippel-Lindau disease.

Glandular tumours involving the middle ear and the mastoid are rare, and distinguishing between adenoma and adenocarcinoma is difficult. Two distinct histopathological patterns are accepted. While their clinical presentation differs, both require primary surgical treatment and both have a high rate of local recurrence. The papillary form takes a more aggressive course and wider regional spread. This pattern occurs predominantly in women. Its microscopic appearance and clinical course have been extensively described by Heffner who considered it as "low-grade adenocarcinoma of probable endolymphatic sac origin". A few cases have been associated with von Hippel-Lindau disease. The case of a 32 year old black woman is described. It is suggested that papillary adenocarcinoma of the endolymphatic sac should be considered in the spectrum of neoplasms seen in von Hippel-Lindau disease.

Adenocarcinoma, Papillary↗

[Brain metastasis of a cutaneous neuroendocrine carcinoma. Apropos of a case].

The authors report the case of a 69-year-old woman with a brain metastasis from a cutaneous neuroendocrine carcinoma. The initial lesion was situated on the leg. No other secondary lesion was found. The cutaneous neuroendocrine carcinoma was initially considered to be a locally malignant tumor. In fact, it is frequently complicated by metastases. However, brain secondaries remain rare.

Aged↗

[Complications of the intravascular treatment of intracranial aneurysms using metal microcoils. Embolization using coils in intracranial aneurysms].

Embolization of intracranial aneurysms with coils. The authors present the results and complications of microcoils embolization of intracranial aneurysms in 28 patients. Fibers platinum microcoils were used (Target Therapeutic) 28 patients (males: 6, females: 22; mean age: 51 years) were treated, 27 suffered from sub-arachnoid hemorrhage (SAH) and one presented with a pseudotumoral syndrome (giant aneurysm). All patients were evaluated on the day of treatment, according to the World Federation of Neuro-Surgeon classification (W.F.N.S.) of SAH and after 4 months of follow-up. At a mean follow-up period of 4 months, according to Glasgow Outcome Scale (G.O.S.) there were 13 cases of good results, 5 cases of "moderate disability", 2 cases of "severe disability", 2 cases of "vegetative state" and 6 deaths. Complications were observed in 11/28 cases. Coils migration and malposition in the parent artery were linked to the procedure (mechanical detachable coil) and resulted in severe deficit (one case), transient disability (one case) or went unnoticed (4 cases). Primary or secondary complete occlusion was achieved in 8/28 patients (32%). A majority of cases (11 cases) ended with stable residual aneurysmal sac lumen while rebleeding occurred in 3 patients and was responsible for 2 deaths. In the third case a favourable outcome was obtained after balloon occlusion of the parent artery.

Adult↗

[Creutzfeldt-Jakob disease after treatment with human extracted growth hormone. A clinicopathological study].

This report describes the pathological changes observed in the brain of a 18-year-old patient who died with Creutzfeldt-Jakob disease (CJD) of the ataxic and panencephalopathic type. Clinically, the disease began at age 17 with a rapidly progressive cerebellar syndrome, associated with myoclonus and mental deterioration. Cranial CT scan and MRI showed minor abnormalities. EEG demonstrated non specific changes. The patient became progressively demented, bedridden and died 10 months after the onset. Nine years earlier, he had been operated upon for craniopharyngioma and subsequently treated with cadaver-derived human growth hormone. Post-mortem examination of the brain revealed mild diffuse atrophy. Histology showed congophilic amyloid plaques found in both gray and white matters of the cerebrum and cerebellum, associated with spongiosis, mainly in the basal ganglia and cerebellum, and gliosis. They were immunostained with antiprion protein antibody using the immunoperoxidase method on paraffin embedded sections. The clinical findings in this case were similar to those of other cases of human growth hormone-associated CJD. However, this case is unusual because of the great number of amyloid plaques, which have been only rarely found in previous reports.

Amyloid↗

[Multiple mononeuritis and cerebral meningo-arteritis. Wegener disease or Lyme disease?].

The pathogenic mechanisms underlying Lyme disease remain uncertain but an increasing number of reports suggest a vascular inflammatory process. On the other hand, the so-called systemic vasculitides, even though they remain of pathological definition, have recently been characterized by the presence, in the serum, of anti-neutrophil cytoplasmic antibodies (ANCA). We report on a patient, finally diagnosed as having neuroborreliosis, who presented initially with multiple mononeuropathy, ANCA, vascular lesions at muscle biopsy and lymphocytic meningitis. Despite antibiotherapy, he presented with two recurrent strokes, from which he completely recovered. He also developed oto-sinusitis. The disease was finally clinically and biologically controlled by immunosuppressive therapy. The relationship between angiitis and Lyme disease are discussed. It is hypothesized that Borrelia burgdorferi infection may cause a systemic vasculitis following its own course and requiring its own treatment.

Aged↗

[Inverted papilloma of the nose and paranasal sinuses. Apropos of 10 cases].

Authors report a retrospective study of 10 new cases of inverted papilloma of the nose and paranasal sinuses, observed during a 26 years period, with a 6.1 years average follow up. This group is marked by 2 cases of directly carcinomatous association, 4 cases of recurrence and 2 cases of later malignant transformation. The responsibility of different types of human papillomavirus in the origin of this tumor and its malignant transformation is suspected. Preoperative evaluation is based on clinical examination, CT scan and endoscopic method while diagnosis and prognosis depend on histopathologic finding of the whole tumor. The aim of surgical treatment is the most complete removal of the tumor with as limited after effects as possible. Lateral rhinotomy is the most favoured surgical approach versus mid facial degloving and intranasal endoscopy. No surgical patient, no removal tumor, inadequate limits, malignant papilloma and early recurrence are indications for external radiotherapy.

Adult↗

[Olfactory esthesioneuroma. Apropos of 12 cases].

The authors report a 12-case series of olfactory esthesioneuromas, in which they review the main characteristics of this rare tumor. The olfactory esthesioneuroma diagnosis should be evoked for all nasal tumors, especially for tumors originating in a high position. This diagnosis is confirmed by the difficult pathological examination, which currently benefits widely from immunohistochimy. Prognosis is poor and surgery is the main treatment, associated with varying degrees of radiotherapy. As far as chemotherapy is concerned, its role must be clarified.

Adult↗

Central neurocytomas. Critical evaluation of a small-cell neuronal tumor.

We report herein the clinical and pathological features of 20 patients with central neurocytomas. Investigations for various differentiation antigens and cell type-specific markers were performed by immunohistochemistry using paraffin-embedded tissue. In addition, the expression of L1 adhesion molecule and of the various N.CAM (neural cell adhesion molecule) isoforms were investigated by immunoblotting studies in two frozen specimens. Central neurocytomas are clinically characterized by their intraventricular localization, occurrence in young adults, and good prognosis. It rarely occurs in patients over 50, but such cases have a poor prognosis. Total surgical excision is the best treatment. Radiotherapy is appropriate if surgery is incomplete or contraindicated. Histologically, central neurocytomas display the following features: an oligo-like pattern, usually associated with large fibrillary rosettes or perivascular arrangement, and a rich endocrine-type vasculature. Central neurocytomas have a remarkably homogeneous antigenic profile. GFAP expression is only found in scattered reactive astrocytes, S100 protein in reactive astrocytes and rare tumor cells. Among the pan-neuroendocrine markers, central neurocytomas always express neuron-specific enolase; they frequently express synaptophysin but never chromogranin A. Synaptophysin is the most reliable immunohistological marker for central neurocytomas; however, immunoreactivity could be lost with long formalin fixation. In these cases, electron microscopy is used to support the neuronal nature of the tumor cells. The expression of L1 adhesion molecule and the isoform 180 of N.CAM, indicates that central neurocytomas are formed by cells committed to neuronal phenotype. Nevertheless, advanced neuronal differentiation may be absent, as suggested by the persistence of embryonic N.CAM, the nonexpression of neurofilament proteins, and the absence of mature synapses in numerous cases. Central neurocytomas and neuroblastomas share some biochemical properties, but their respective clinicopathological features and biological behavior are dramatically different.

Adolescent↗

Evaluation of 29 monoclonal and polyclonal antibodies used in the diagnosis of pituitary adenomas. A collaborative study from pathologists of the Club Français de l'Hypophyse.

Fifteen polyclonal antibodies (pAbs) and 14 monoclonal antibodies (mAbs) directed against hGH, hPRL, beta hFSH, beta hLH, beta hTSH and alpha-subunit were assessed by five different laboratories on normal and adenomatous pituitary tissues. This study aims at providing pathologists with a selected panel of antisera suitable for diagnosis, and appreciating the interest of the recently introduced mAbs. All the anti-hGH Abs proved to be specific (3 pAbs and 4 mAbs); three mAb out of four gave a few false-negative reactions. Three out of six polyclonal anti-hPRL showed cross-reactivity with hGH; anti-hPRL mAbs gave a strong staining with no false-negativity detected so far. MAbs proved to be more efficient for detecting glycoprotein hormones and alpha subunit than pAbs, which, in several cases, gave widespread cross-reactivity. This lack of specificity could explain the noticeable discrepancies reported so far in the appraisal of gonadotropic and somatoprolactinic adenomas.

Adenoma↗

[Late myopathies located at the spinal muscles: a cause of acquired lumbar kyphosis in adults].

A primary myopathy limited to the spinal muscles and of late onset was suspected in 14 patients with a mean age of 66. These patients had an anterior inflection of the trunk and were unable to rotate the lumbar spine on the pelvis. This incurvation of the trunk, starting at around age 60, was reducible in a horizontal position and increased with tiredness. The CT scan appearance of the spinal muscles of these patients was hypodense and heterogeneous, different from the atrophy found in the elderly with lumbar osteoarthrosis, comparable with the lesions described in primary myopathies. Histologically, lesions of fibro-adiposis were major, accompanied by mitochondrial abnormalities. The frequent existence of a family history would be in favour of a genetically transmitted condition.

Aged↗

[Cardiac sarcoma disclosed by hypereosinophilia].

A 48 year old woman presented peripheral eosinophilia and neurologic symptoms which were related to a right parietal hypodense lesion. Further investigation led to the discovery of a left atrial cardiac tumor which had been incompletely resected and diagnosed as sarcoma. Eosinophilia than decreased. Two months after cardiac surgery, intracranial hypertension appeared and another expansive cerebral mass was discovered on CT scan. The patient was treated by radiotherapy. Two years later, the patient presented left abdominal pain. An increase of eosinophilic rate was noted. Abdominal CT scan revealed an heterogenous mass in the spleen. Splenectomy was performed and the tumor was diagnosed as a metastasis of the cardiac sarcoma. This case illustrates a rare tumor which is distinctive by its clinical signs: peripheral eosinophilia and neurologic signs. There were no cardiac symptoms. The clinical evolution was good after more than two years from initial diagnosis. This implies that a surgical attitude is recommended in such cases.

Eosinophilia↗

Influence of host sex on the growth of a human glioblastoma line in athymic mice.

Glioblastomas are generally thought to be more common in men than in women. In order to investigate the hormone-dependence of these tumours, we established a human glioblastoma line in athymic mice. The tumour cell type was characterized using immunocytochemical methods. The influence of host sex on growth was evaluated, and hormone receptors were characterized biochemically. The histological features of the initial tumour were conserved in the hetero-transplanted tumours, which consisted of vimentin and GFAP immunoreactive astrocytes. There was a highly significant difference in tumour growth between the two sexes (P less than 0.01). In the male mice, tumours were from 2.5 to 10 times larger than in the females, the latency periods were 30% shorter, and the growth phases were characterized by periods of slow or zero growth. In addition, androgen and oestrogen receptors were detected at low levels (80-270 fmol/g tumour) in the heterotransplanted tumours especially in the males. The fact that the male tumour growth profiles resembled those of some hormone-dependent lines, and that androgen receptors were found preferentially in the male rather than in female tumours would tend to indicate that there is a hormonal influence on the growth of the heterotransplanted tumours. These results provide further evidence for an influence of sex-steroid hormones on the growth of glioblastomas.

Aged↗