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Biomedical subjects

M B Delisle

Publications and source records attributed to M B Delisle.

At least 19 recordsLinked to original sources
PubMed2006

Comparison of extent of tau pathology in patients with frontotemporal dementia with Parkinsonism linked to chromosome 17 (FTDP-17), frontotemporal lobar degeneration with Pick bodies and early onset Alzheimer's disease.

In order to gain insight into the pathogenesis of frontotemporal lobar degeneration (FTLD), the mean tau load in frontal cortex was compared in 34 patients with frontotemporal dementia linked to chromosome 17 (FTDP-17) with 12 different mutations in the tau gene (MAPT), 11 patients with sporadic FTLD with Pick bodies and 25 patients with early onset Alzheimer's disease (EOAD). Tau load was determined, as percentage of tissue occupied by stained product, by image analysis of immunohistochemically stained sections using the phospho-dependent antibodies AT8, AT100 and AT180. With AT8 and AT180 antibodies, the amount of tau was significantly (P < 0.001 in each instance) less than that in EOAD for both FTDP-17 (8.5% and 10.0% respectively) and sporadic FTLD with Pick bodies (16.1% and 10.0% respectively). With AT100, the amount of tau detected in FTDP-17 was 54% (P < 0.001) of that detected in EOAD, but no tau was detected in sporadic FTLD with Pick bodies using this particular antibody. The amount of insoluble tau deposited within the brain in FTDP-17 did not depend in any systematic way upon where the MAPT mutation was topographically located within the gene, or on the physiological or structural change generated by the mutation, regardless of which anti-tau antibody was used. Not only does the amount of tau deposited in the brain differ between the three disorders, but the pattern of phosphorylation of tau also varies according to disease. These findings raise important questions relating to the role of aggregated tau in neurodegeneration - whether this represents an adaptive response which promotes the survival of neurones, or whether it is a detrimental change that directly, or indirectly, brings about the demize of the affected cell.

Adult↗
PubMed2005

Detection of human cytomegalovirus genome and gene products in central nervous system tumours.

Human cytomegalovirus (HCMV) genome and related proteins have been reported in a great proportion of malignant gliomas. However, these results are unexpected since HCMV is not known as an oncogenic virus. By immunohistochemistry (with an anti-IE1 monoclonal antibody) and in situ hybridisation (with biotinylated DNA probes) on tissue microarrays and frozen sections, we investigated a French series of central nervous system (CNS) tumours, including 97 glioblastomas. In 10 cases of glioblastoma, rare astrocyte-like cells, admixed with tumour cells, stained positively for HCMV and in one case a doubtful staining of rare cells was noticed. This may indicate a reactivation of the virus under local immunosuppression but none of the cases of CNS tumours (n=132) contained HCMV genomes and/or proteins in a significant proportion of tumour cells. Our results strongly suggest that HCMV is unlikely to be implicated in the development of human malignant gliomas, at least in French cases.

Adult↗
PubMed2004

Intracellular ferritin accumulation in neural and extraneural tissue characterizes a neurodegenerative disease associated with a mutation in the ferritin light polypeptide gene.

Abnormal accumulation of ferritin was found to be associated with an autosomal dominant slowly progressing neurodegenerative disease clinically characterized by tremor, cerebellar ataxia, parkinsonism and pyramidal signs, behavioral disturbances, and cognitive decline. These symptoms may appear sequentially over a period of 4 decades. Pathologically, intranuclear and intracytoplasmic bodies were found in glia and subsets of neurons in the central nervous system as well as in extraneural tissue. Biochemical analyses of these bodies isolated from the striatum and cerebellar cortex revealed that ferritin light polypeptide (FTL) and ferritin heavy polypeptide (FTH1) were the main constituents. Molecular genetic studies revealed a 2-bp insertion mutation in exon 4 of the FTL gene. The resulting mutant polypeptide is predicted to have a carboxy terminus that is altered in amino-acid sequence and length. In tissue sections, the bodies were immunolabeled by anti-ferritin and anti-ubiquitin antibodies and were stained by Perls' method for ferric iron. Synthetic peptides homologous to the altered and wild-type carboxy termini were used to raise polyclonal antibodies. These novel antibodies as well as an antibody recognizing FTH1 immunolabeled the bodies. This study of this disorder has provided additional knowledge and insights in the growing area of ferritin-related neurodegeneration.

Adult↗
PubMed2003

Cervical dystonia mimicking dropped-head syndrome after radiotherapy for laryngeal carcinoma.

We report a case of cervical dystonia mimicking dropped-head syndrome (DHS) in a 57-year-old man treated for laryngeal carcinoma by radiotherapy (74.4 Gy) 3 months before. Cervical computerized tomographic scan and magnetic resonance imaging (MRI) did not find any muscle fat changes but found a high-intensity signal on T2 weighted images in the cervical spinal cord. Clinical and electromyographic findings were consistent with cervical dystonia. A trapezius biopsy was normal. Spontaneous remission of the dystonia was observed for 1 month whereas the laryngeal carcinoma progressed. The link between cervical dystonia and radiotherapy might be acute radiation-induced damage to the cervical spinal cord.

Carcinoma, Squamous Cell↗
PubMed2002

Symptomatic macroglossia and tongue myositis in polymyositis: treatment with corticosteroids and intravenous immunoglobulin.

Symptomatic macroglossia occurs in some rare congenital muscle diseases, such as Becker's and Duchenne's dystrophies or Pompe's disease. Herein we describe a case of symptomatic macroglossia with myositis of the tongue occurring in a patient with polymyositis. Tongue myositis was evidenced by dysarthria, frequent biting during mastication, swallowing difficulties without aspiration, and noisy breathing. Magnetic resonance imaging showed homogeneous hypertrophy of the tongue, especially the mouth's floor muscles. The diagnosis of tongue myositis was established by electromyography and biopsy. No other cause for the macroglossia was found. Symptoms resolved quickly with corticosteroid and intravenous immunoglobulin treatment. To our knowledge, this is the first reported case of symptomatic tongue myositis occurring in the course of polymyositis.

Humans↗
PubMed2002

A relapsing remitting lymphocytic hypophysitis.

Lymphocytic Hypophysitis is a rare autoimmune disease of the pituitary presenting mainly with features of a mass lesion and loss of pituitary function. Its course is quite unpredictable and its treatment is still controversial as experience in the field remains scarce. We describe a 45 year-old woman with a history of recurrent fever and meningeal symptoms who was referred 3 years later to our department for pituitary insufficiency. Hormonal studies revealed an anterior pituitary deficiency and autoimmune thyroiditis. Pituitary hypertrophy as evidenced by magnetic resonance imaging showed complete regression upon hydrocortisone substitution therapy. Two years later, fever and meningeal symptoms recurred as well as pituitary hypertrophy. Cerebrospinal fluid analysis revealed an aseptic lymphocytic meningitis. Pituitary biopsy confirmed the diagnosis of LH. The patient was started on prednisone 70 mg per day. She improved clinically and radiologically and remained free of symptoms thereafter. At a recent follow up the MRI showed an empty sella turcica. Hereby we illustrate a yet unreported pattern of LH presenting as a recurrent aseptic meningitis. We also describe one of the long-term course of the disease which is still unpredictable.

Anti-Inflammatory Agents↗
PubMed2001

Activation of the JNK/p38 pathway occurs in diseases characterized by tau protein pathology and is related to tau phosphorylation but not to apoptosis.

JNK and p38, two members of the MAP kinase family, are strongly induced by various stresses including oxidative stress and have been involved in regulation of apoptosis. As both kinases phosphorylate tau protein in vitro, we have investigated their immunohistochemical localization in a group of neurodegenerative diseases characterized by intracellular deposits of hyperphosphorylated tau. Cases included Alzheimer disease, Pick disease, progressive supranuclear palsy, corticobasal degeneration, Gerstmann-Sträussler-Scheinker disease-Indiana kindred, and frontotemporal dementia with parkinsonism linked to chromosome 17. In all tissue samples, strong immunoreactivity for both MAP kinases was found in the same neuronal or glial cells that contained tau-positive deposits. By double immunohistochemistry, JNK and p38 colocalized with tau in the inclusions. Analysis of apoptosis-related changes (DNA fragmentation, activated caspase-3) showed that the expression of JNK and p38 was unrelated to activation of an apoptotic cascade. Our data indicate that phospho-JNK and phospho-p38 are associated with hyperphosphorylated tau in a variety of abnormal tau inclusions, suggesting that these kinases may play a role in the development of degenerative diseases with tau pathology.

Adult↗
PubMed2001

Intrarectal injection of glycerol induces hypersensitivity to rectal distension in healthy subjects without modifying rectal compliance.

BACKGROUND: Rectal sensory thresholds are lowered in patients with irritable bowel syndrome (IBS), reflecting visceral hyperlagesia, which might be related to subclinical inflammation. AIM: To evaluate the effects of an intraluminal injection of glycerol, a mucosal irritant, on rectal tone and perception of distension in 12 healthy subjects. METHODS: Rectal tone was evaluated with a barostat. First sensation, need to defecate and pain thresholds were evaluated during isobaric phasic distensions, before and 20 and 120 min after injection of 10 ml glycerol in the rectum. RESULTS: Baseline bag volume (97.9 +/- 56.2 ml) significantly decreased 20 min (49.7 +/- 42.2 ml; P= 0.026) and 120 min (66.5 +/- 38.3 ml; P= 0.050) after injection of glycerol, indicating its hypertonic effect. The pressure defining sensory thresholds was decreased significantly 20 min after glycerol injection: first sensation, 14.6 +/- 2.9 versus 18.3 +/- 7.2 mm Hg (P = 0.01); need to defecate, 19.6 +/- 3.7 versus 26.0 +/- 6.9 mm Hg; pain, 23.8 +/- 4.5 versus 35.6 +/- 9.5 mm Hg (P = 0.001). This effect was maintained for 120 min after injection of glycerol. Slopes of the compliance curves did not differ before and after injection of glycerol. CONCLUSIONS: Intraluminal injection of glycerol significantly increases rectal tone and sensitizes healthy volunteers to rectal distension, since they show significantly lower thresholds after glycerol. This could constitute a model of visceral hypersensitivity in healthy volunteers.

Administration, Rectal↗
PubMed2001

Vestibular schwannomas: correlations between magnetic resonance imaging and histopathologic appearance.

BACKGROUND: The indication for surgery of vestibular schwannomas (VS) remains controversial and depends on several factors. The ability to predict their patterns of growth would allow better surgical planning. This growth may depend on tumoral proliferation but also depends on dystrophic changes. OBJECTIVE: The aim of this study was to evaluate the role of magnetic resonance imaging (MRI) in predicting the evolution of VS. For this purpose, the authors attempted (1) to compare the MRI appearance of VS with its histopathologic features, (2) to correlate the MRI appearance of VS and its histopathologic features with its size, and (3) to evaluate the index of proliferation (IP) of each VS. PATIENTS AND METHODS: Thirty VS were studied with MRI before surgery. The VS were measured and classified as homogeneous, heterogeneous, and cystic. After surgery, IP was evaluated with immunohistochemical study using MIB-1 monoclonal antibody, and compared with tumor size. Pathologic studies evaluated the prevalence of Antoni type A and type B tissue, the amount of fibrosis, and the presence of siderin-loaded macrophages, xanthomatous cells, and cysts. RESULTS: The IP was low (0.2%-2.2%) and was not correlated with VS size. On MRI, 13 VS were homogeneous, 12 heterogeneous, and 5 cystic. The 13 homogeneous VS were smaller and were predominantly made of Antoni type A tissue. The 12 heterogeneous and 5 cystic VS were larger and were predominantly made of Antoni type B/mixed tissue. Heterogeneous and cystic VS showed significantly more hemosiderin deposits. There was a significant relation between the amount of hemosiderin deposits and the mean size of VS. Microscopic cysts were observed only in VS with cystic MRI appearance. Fibrosis was present in all tumors regardless of their size and MRI appearance. CONCLUSION: A heterogeneous MRI aspect (correlated with larger mean size) not only is related to the ratio of type A to type B tissue but also is caused by other pathologic changes, mainly hemosiderin deposits and cystic formation. Increasing tumor size probably depends less on IP than on dystrophic changes (hemosiderin, cysts) and/or on the presence of type B tissue.

Adolescent↗
PubMed2001

Immunohistochemical assessment of human herpesvirus 8 infection in primary central nervous system large B cell lymphomas.

BACKGROUND/AIMS: Primary central nervous system large B cell lymphomas (PCNSLs) are frequently associated with Epstein-Barr virus (EBV) in patients with AIDS and less frequently in those without AIDS. Human herpesvirus 8 (HHV-8) has been detected in these tumours by the polymerase chain reaction (PCR) at low copy number, suggesting its presence in a cell compartment other than the malignant one. The aim of this study was to use immunohistochemistry to assess HHV-8 infection in a series of PCNSLs from patients with and without AIDS. METHODS: The antibody LN53, which reacts with the latent nuclear antigen 1 (LNA1) of HHV-8, was used on tissue sections from 35 patients (17 with and 18 without AIDS) with PCNSL. In addition, DNA was available for PCR (open reading frame 26 (ORF 26), ORF 72, ORF 75) in three patients (two without AIDS, one with AIDS). RESULTS: None of the 35 cases contained either DNA sequences or LNA1 positive cells. CONCLUSIONS: These results confirm the lack of HHV-8 infection in tumour cells of PCNSL. In addition, HHV-8 infected bystander cells do not express LNA1 latent protein in this setting.

Animals↗
PubMed2001

Nasal polyp-derived superoxide anion: dose-dependent inhibition by nitric oxide and pathophysiological implications.

The epithelium of the paranasal sinuses produces nitric oxide (NO), which probably plays a major role in the nonspecific defense of these cavities through its bacteriostatic and cilia motility stimulation properties. Abundant eosinophils of nasal polyps potentially generate superoxide anion (O2-*), but NO and O2-* inactivate reciprocally. The purpose of the present work was to evaluate the relationship between NO concentrations and nasal polyp production of O2-*. Polyp fragments from 24 patients were studied using histological examination and lucigenin-enhanced chemiluminescence (to assess O2-* production). The effect of various concentrations of exogenous NO on chemiluminescent signals was assessed. Basal and phorbol ester-stimulated O2-* production varied largely among patients, but both were highly related to eosinophilic infiltration. A slow releasing NO donor DETA NONOate (DETA/NO NOC-18) dose dependently inhibited lucigenin-enhanced chemiluminescence from phorbol ester-stimulated polyp fragments, with an EC50 of 1.5 mM. The NO concentration in normal maxillary sinus was estimated about 10 ppm (i.e., 0.5 microM in aqueous phase) (Lundberg, et al. Nature Med 1995;1:370). Calculations revealed that the DETA NONOate 0.75 mM and 1.5 mM generate steady-state concentrations of NO of 0.5 microM and 2.5 microM, respectively. In conclusion, the NO concentration present in paranasal sinuses appears to partially suppress (approximately 20-40%) O2-* production from polyp eosinophils. Conversely, phagocytic-derived O2-* could contribute to decrease sinus NO concentration, further altering this natural local defense. Together, these events could participate in chronic inflammation and contribute to the pathophysiology of nasal polyps.

Acridines↗
PubMed2001

Macroporous polyester-covered stent in an experimental abdominal aortic aneurysm model.

PURPOSE: To validate a recently described animal model of abdominal aortic aneurysm (AAA) and to assess a new macroporous polyester-covered stent for endovascular AAA exclusion. METHODS: Twenty adult sheep had AAAs surgically created by replacing a segment of the infrarenal aorta with an autologous jugular venous graft. Three months later, surviving animals underwent percutaneous implantation of macroporous polyester-covered nitinol stents; 3 animals with untreated AAAs served as controls. Follow-up surveillance included spiral computed tomography at 1 month and digital subtraction angiography at 3 and 6 months. Endografted animals were sacrificed at 1, 3, and 6 months after implantation; specimens from all animals were examined grossly and microscopically. RESULTS: Seven (35%) animals died within 24 hours of causes related to the technique; 1 animal developed paraplegia and was sacrificed on day 1. Three (25%) animals died of spontaneous aneurysm rupture at <10 days, and 6 received the stent-graft at 3 months. The macroporous cover did not prevent continued perfusion of the sac early after stent-graft deployment, but all aneurysms were excluded on the 1-month CT. CONCLUSIONS: Spontaneous AAA rupture occurred earlier and was not as frequent as previously described for this model. Implantation of the covered stent was feasible, but aneurysm exclusion was not immediate.

Animals↗
PubMed2001

Value of investigation in the diagnosis of allergic fungal rhinosinusitis: results of a prospective study.

The authors report a prospective study in which the aim was to analyse the usefulness of different criteria in optimizing the diagnosis of allergic fungal rhinosinusitis. From 1995 to 1998, 165 patients were operated on for chronic rhinosinusitis. Investigations used in this study for the diagnosis of allergic Aspergillus rhinosinusitis consisted of an analysis of clinical, radiological, immuno-allergic criteria. Fourteen patients presented with allergic Aspergillus rhinosinusitis. One hundred and fifty-one patients did not present any of the necessary criteria for the diagnosis of allergic Aspergillus rhinosinusitis. The results show that the characteristic macroscopic appearance, the maxillary sinus localization, and the presence of positive specific IgE to Aspergillus fumigatus are arguments that reinforce the diagnostic certitude of allergic fungal sinusitis. No specific clinical or radiological criteria orients a diagnosis of chronic rhinosinusitis toward that of allergic fungal rhinosinusitis. The other immuno-allergic tests do not contribute to the diagnosis of allergic fungal rhinosinusitis. pathological, mycological, and

Adolescent↗
PubMed2001

[Anaplastic temporal ganglioglioma with spinal metastasis].

We report the case of a 26-year-old male who was operated on after an acute episode complicating a long history of seizures. The resected temporal lesion was an anaplastic ganglioglioma. Ganglioglioma is a tumor of children and young adults, characterized by two cell components, neuronal and glial. It is usually a benign lesion. The present case is remarkable in several aspects. Hemorrhage led to the discovery of a supra-tentorial tumour. Microscopic examination revealed anaplastic foci in an otherwise typical lesion. There was a long past medical history but outcome was rapidly unfavorable after surgical resection and associated with dissemination along the spinal cord.

Adult↗
PubMed2000

Suprasellar seeding of a benign choroid plexus papilloma of the fourth ventricle with local recurrence.

A suprasellar location of a benign choroid plexus papilloma is reported. Local recurrence within the fourth ventricle was also present, 8 years after apparently complete removal. Imaging and histological findings were similar to those of the initial lesion. At surgery, the suprasellar lesion had no connection with the ventricular system. Seeding of choroid plexus papillomas is discussed, and the pertinent literature reviewed.

Female↗
PubMed2000

Perturbation in dystrophin-associated glycoprotein complex in a boy with Becker muscular dystrophy.

We report on a boy with a BMD phenotype presenting with a deletion of exons 45-49 in the DMD gene. Immunofluorescence and Western blot analysis of a skeletal muscle sample revealed, as expected, truncated dystrophin with loss in the central rod domain, but with an unusual severe deficiency in the sarcoglycan complex, as in severe DMD. We discuss possible neighboring between dystrophin and associated proteins within their complex organization at the muscle membrane.

Child, Preschool↗