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Biomedical subjects

D Figarella-Branger

Publications and source records attributed to D Figarella-Branger.

At least 145 records · Page 8Linked to original sources
PubMed1994

Ectopic basisphenoidal craniopharyngioma: case report and review of the literature.

We report the case of a patient with a nasopharyngeal craniopharyngioma. The presenting symptoms included a diminished visual acuity, a dysmorphic syndrome, and impaired endocrine function. The computed tomography and magnetic resonance imaging scans showed a partially cystic tumor that had destroyed most of the craniofacial sinus and anterior part of the cranial base. The patient was operated on through a rhinoseptal approach. We found 14 similar cases in the available neurosurgical and otorhinolaryngologic literature. The pathogenesis of this tumor and more especially its origin from Rathke's pouch remnants or pharyngeal hypophysis are discussed.

Adult↗
PubMed1994

Cavernous angiomas and arteriovenous malformations of the spinal epidural space: report of 11 cases.

We have retrospectively studied 11 cases of solitary cavernous and arteriovenous malformations of the spinal epidural space. The acute or subacute onset of symptoms was found in all cases, with a neurological deficit in five cases. A plain x-ray study revealed abnormal calcification in one case. Hyperdensity with no iodine enhancement was found on computed tomographic scans in all cases. Magnetic resonance imaging studies showed ovoid or round lesions with isosignal and gadolinium enhancement on T1-weighted images and hypersignal on T2-weighted images, except in one case with hypersignal on T1 and T2 before the administration of a contrast medium. Four lumbar cases were localized in the posterior vertebral ligament; all the thoracic cases were posterior with foraminal extension. The other cases were anterolateral. The surgical findings revealed a dark vascularized epidural lesion associated in two cases with a localized epidural hematoma. A pathological examination revealed seven cavernous angiomas and four arteriovenous malformations. These arteriovenous malformations were all lumbar, and two of four localized within the posterior ligament. The pathophysiology of these vascular malformations is discussed. In light of the good postoperative prognosis of these lesions, surgical removal is recommended for all the symptomatic cases.

Adult↗
PubMed1994

Quantitative imaging of estrogen and progesterone receptors, estrogen-regulated protein, and growth fraction: immunocytochemical assays in 52 meningiomas. Correlation with clinical and morphological data.

Quantitative imaging of estrogen receptors (ER's), progesterone receptors (PR's), estrogen-regulated protein (pS2), and growth fraction (Ki67) immunocytochemical assays were performed in 52 meningiomas. The results were correlated with clinical (age, sex, hormonal status, and tumor volume and location) and morphological (histological types and grades) data. The authors observed a lack of ER's in all meningiomas but the presence of PR's in 53% of these meningiomas. The immunoreactivity was restricted to tumor cell nuclei. The PR immunocytochemical assay was correlated with tumor location, histological type, histological grade, and pS2 immunocytochemical assay, but not with Ki67 immunocytochemical assay; high PR content was observed in cisternae, transitional, meningothelial, and low-grade meningiomas. Only 11 meningiomas showed more than 1% Ki67 immunoreactive nuclei. These meningiomas were usually located in the convexity and were of high histological grade. Estrogen-regulated protein immunoreactivity was observed in 34 meningiomas but the number of immunoreactive nuclei was low. The pS2 immunocytochemical assay was not related to clinicopathological features but was preferentially observed in PR-negative meningiomas. The results of this study are compared with those previously reported, and the function and regulation of PR's in meningiomas is discussed. The results indicate that 1) regulation of PR's and pS2 proteins in meningiomas differs from regulation in estrogen-dependent tissues such as breast or endometrium; 2) interruption of hormonal therapy in women presenting with a meningioma is not absolutely necessary; 3) meningiomas have different biological properties according to their clinicopathological features; and 4) future studies of hormonal clinical trials should be performed on well-defined meningioma subgroups.

Adolescent↗
PubMed1994

[Digital necrosis disclosing antisynthetase syndrome].

The antisynthetases syndrome associates lung disease, polymyositis, Raynaud's phenomenon and frequently polyarthritis. Anti-amioacyl-tRNA synthetase antibodies are present. The most common is anti-Jol. We report a case with severe arteritis of the finger with multiple zones of skin necrosis which responded favourably to corticosteroids given in bolus and cyclophosphamid. Finger arteritis, in addition to classical Raynaud's phenomenon, may be a new manifestation of the anti-synthetases syndrome.

Acrodermatitis↗
PubMed1994

Expression of neural cell-adhesion molecule isoforms and epithelial cadherin adhesion molecules in 47 human meningiomas: correlation with clinical and morphological data.

Meningiomas are derived from arachnoidal cells exhibiting both epithelial and mesenchymal properties. The expression of epithelial cadherin and neural cell-adhesion molecule isoforms was examined in 47 meningioma specimens and normal arachnoid villi by using immunohistochemistry and immunoblot procedures. For immunohistochemistry, the percentage of immunostained surface versus counterstained surface and the intensity of labeling were quantified by image analysis and correlated with clinical and morphological data. Neural cell-adhesion molecule, 140-kDa, was expressed by all meningiomas examined and by cap cells and the fibrous capsule forming the arachnoid villi. Neural cell-adhesion-molecule immunostaining was correlated to tumor location, histological type, and grade. The expression of polysialylated isoforms was related to tumor location and grade, with a higher expression on convexity meningiomas and high-grade tumors. Epithelial cadherin was expressed with various intensity. In arachnoid villi, its expression was restricted to cap cells, but it was found in all meningiomas, whatever their histological type or grade. However, the expression was stronger in whorls and meningiotheliomatous areas versus fibroblastic areas. No correlation was observed with the tumor location or the grade. These data suggest that neural cell-adhesion molecule and epithelial cadherin play a role in the morphogenesis and histogenesis of human meningiomas.

Adolescent↗
PubMed1994

[Isolated proximal muscular weakness disclosing myasthenic syndrome].

Three cases of chronic limb-girdle myasthenia gravis (one man and two women) are reported. The duration of the disease was three, five and 20 years respectively. None had oculobulbar weakness during this period nor fluctuating symptoms. The primary diagnosis was myopathy in two cases and myelopathy in the other one. In view of this diagnosis, several features were atypical: absence of marked wasting, preservation of tendon reflexes and normal serum creatine kinase activity. Finally, diagnosis of myasthenia gravis was established by the occurrence of a characteristic decremental muscular response to repetitive stimulation and by positive edrophonium test. It seems that the rate of positivity of acetylcholine receptor antibody is lower than in the common form of the disease. Two patients responded to acetylcholinesterase inhibitors and one to high-doses intravenous human immunoglobulins. These and previous cases described in the literature raise the problem of the definition of an unusual form of myasthenia gravis or of a new type of neuromuscular disease characterized by the presence of distinctive changes on muscular biopsies, specially tubular aggregates as in one of our cases. However, this unusual form of chronic limb-girdle weakness has to be recognized because treatments are effective in most cases.

Adult↗
PubMed1994

Significance of isolated vasculitis of the vasa vasorum on temporal artery biopsy.

OBJECTIVE: To assess the significance of an isolated vasculitis of vasa vasorum on temporal artery biopsy. METHODS: A diagnostic procedure was performed with 28 patients presenting clinical and biological inflammatory process and an isolated vasculitis of vasa vasorum on temporal artery biopsies. RESULTS: Panarteritis nodosa was diagnosed in 5 cases: polymyalgia rheumatica was evidenced in 4 cases; temporal arteritis was strongly suspected in 2 cases; various types of underlying disease were found in 12 patients; a noncharacterized systemic disease was found in 5 cases. CONCLUSION: Isolated vasculitis of vasa vasorum on temporal artery is a meaningful lesion frequently indicating a systemic vasculitic disorder.

Aged↗
PubMed1993

CD24, a signal-transducing molecule expressed on human B lymphocytes, is a marker for human regenerating muscle.

The expression of the CD24 molecule, a glycoprotein expressed at the surface of most B lymphocytes and differentiating neuroblasts, was studied in developing nerve and muscle (after 16 weeks of gestation), normal adult and various diseased human muscles using immunohistochemistry and Western blot analysis. Immunohistochemical studies demonstrated that: (1) in developing muscles, fibers did not express CD24, whereas only some mesenchymal areas, also expressing neural cell adhesion molecule (N.CAM) and vimentin, and developing nerves were positive; (2) in normal adult muscles, CD24 immunoreactivity was observed only in some unmyelinated nerve fibers--intra and extra fusal muscle fibers, satellite cells and neuromuscular junctions were negative; and (3) in all diseased muscles studied here, CD24 expression was always associated with a subpopulation of regenerative fibers. These fibers also expressed vimentin, desmin, developmental myosin heavy chain, N.CAM and its polysialylated isoforms (PSA-N.CAM). The number of CD24-positive fibers was always lower than that of PSA-N.CAM-positive fibers. Denervated fibers and vacuolated muscle fibers never expressed CD24. Western blot analysis indicated that the apparent molecular mass of CD24 antigen was different between muscle and developing nervous tissues, suggesting that CD24 glycosylation is tissue specific. Since the molecule was not expressed in developing human muscle fibers, it strongly suggests that regenerative and fetal myotubes are different with respect to the CD24 molecule expression.

Adult↗
PubMed1993

Central neurocytoma: a synopsis of clinical and histological features.

The central neurocytoma is a supratentorial, often calcified brain tumour affecting young adults and is typically located in the lateral ventricles in the region of the foramen of Monro. Clinically, the tumour causes signs of increased intracranial pressure, visual and mental disturbances and, occasionally, pyramidal or endocrine symptoms. By light microscopy, the tumour is composed of small round cells in a delicate fibrillary matrix. Tumour cells consistently show features of neuronal differentiation by electron microscopy (synapses, dense-core vesicles, presynaptic clear vesicles, specialized synaptic junctions) and immunoreactivity for synaptophysin and other neuronal marker proteins. The tumour can be totally removed in nearly half of the cases. After incomplete surgical resection neurocytomas may recur but because of their low proliferation potential, radio- or chemotherapy are not generally recommended. Postoperative recurrence-free survival times of up to 19 years have been reported. Neurocytomas constitute nearly one half of supratentorial intraventricular tumours in adults but amount to less than 1% of all tumours of the central nervous system and its coverings.

Brain Neoplasms↗
PubMed1993

Systemic amyloidosis AL with temporal artery involvement revealing lymphoplasmacytic malignancy in a man presenting as polymyalgia rheumatica.

A 68 year old man presented with a clinical and biological picture that suggested polymyalgia rheumatica. Temporal artery biopsy disclosed no inflammatory change but massive light chain amyloid deposits in the media. Further exploration showed a malignant lymphoplasmacytic haemopathy with a triclonal gammopathy and a muscular, rectal, and probable cardiac amyloidosis. Cryoglobulinaemia and high concentrations of soluble interleukin 2 receptor (sIL-2R) were also found. This is the fifth case with confirmed involvement of the temporal artery. The especially high sIL-2R concentration was thought to reflect the tumour mass rather than lymphocyte activation.

Aged↗
PubMed1993

[Weber syndrome caused by Listeria abscess].

A 63-year old diabetic man presented with left Weber's syndrome and meningitic syndrome. CSF examination showed moderate lymphocytic pleocytosis and elevated proteins with normal glucose content and sterile culture. Blood cultures yielded Listeria monocytogenes and the patient received ampicillin. While his neurological condition had partially improved, he died of heart failure. Several mesencephalic abscesses were found at autopsy.

Brain Abscess↗
PubMed1993

Expression of polysialylated neural cell adhesion molecule (PSA-N-CAM) in developing, adult and regenerating caudal spinal cord of the urodele amphibians.

The patterns of expression of polysialylated ("embryonic") form of Neural Cell Adhesion Molecule (PSA/E-N-CAM) and of all N-CAM isoforms were investigated by indirect immunofluorescence and immunoblotting during the development of the Central Nervous System (CNS) and during the regeneration of the caudal Spinal Cord (SC) of the amphibian urodeles Pleurodeles waltl (Pw) and Notophthalmus viridescens (Nv). In this study, a monoclonal antibody to group B Meningococcus (anti-Men-B) which recognizes alpha-2,8-linked sialic units of PSA-N-CAM, and polyclonal anti-total N-CAM antibodies were used. Total-N-CAM immunoreactivities were consistently detected throughout the CNS of developing and adult newts. PSA-N-CAM expression predominated in "embryonic" developing CNS and was reduced to certain CNS areas in the adult urodeles. In the case of SC, the expression level of this isoform of N-CAM dramatically decreased to become low and nearly restricted to some ependymoglial cell surfaces. Interestingly, during newt tail regeneration, PSA-N-CAM was intensely reexpressed in regenerating SC, at the surface of ependymoglial cell processes and in axonal compartments. Expression was maximal at 4 to 6 weeks following amputation, and then gradually returned to a normal adult low level in well differentiated SC. These findings strongly supported the view that the expression of PSA-N-CAM was associated with the properties of plasticity shown by the SC ependymoglial tissue in newts, during tail regeneration. On the other hand, the high level of PSA-N-CAM expression in axonal compartments of regenerating as well as developing SC suggested that these isoforms of N-CAM could be implicated in axonal outgrowth within the "tunnels" defined by the radial ependymoglial processes. This transient PSA-N-CAM expression could therefore be considered both as a negative modulator of cell-cell and cell-substrate interactions and as a permissive factor for neuron differentiation.

Aging↗
PubMed1992

Polymyalgia rheumatica and mitochondrial myopathy: clinicopathologic and biochemical studies in five cases.

PURPOSE: The coexistence of mitochondrial myopathy and polymyalgia rheumatica without giant cell arteritis is an interesting association. The frequency of this association was assessed in a prospective study. PATIENTS AND METHODS: Muscle biopsy specimens were obtained from 15 patients with polymyalgia rheumatica. When ragged red fibers (RRF) were observed, histochemical, ultrastructural, and biochemical studies were performed. RESULTS: In five cases, we found the typical appearance of mitochondrial myopathy, with the presence of numerous RRF. Histochemical and biochemical results confirmed these mitochondrial myopathies, showing miscellaneous deficiencies of mitochondrial respiratory chain enzymes. CONCLUSION: Persistence of histologic and biochemical abnormalities after steroid treatment in two patients seems to indicate that a subclinical mitochondrial myopathy preceded polymyalgia rheumatica. How a mitochondrial myopathy could induce or facilitate the emergence of a polymyalgia rheumatica remains unknown.

Aged↗
PubMed1992

Expression of cell surface and cytoskeleton developmentally regulated proteins in adult centronuclear myopathies.

In order to evaluate the developmental status of myofibers in 3 cases of adult centronuclear myopathies (CNM) with type I predominance, we searched for the expression of (a) developmentally regulated cytoskeleton proteins (myosin heavy chains (MHC), vimentin, desmin), and (b) cell surface molecules (neural cell adhesion molecules isoforms, NCAM). Desmin intermediate filaments were overexpressed in some fibers with centrally located nuclei and radially organized. Muscle fibers do not express vimentin. These findings were not observed in muscle biopsies from disease controls with numerous central nuclei. Few myofibers (less than 5%) expressed developmental MHC together with either embryonic NCAM or adult NCAM and rare fibers only expressed adult NCAM. Most of the remaining fibers neither expressed NCAM nor developmental MHC but were slow MHC positive. These features do not favor the hypothesis of a general arrest of muscle fiber maturation in adult CNM. It is more likely that fibers undergo a very slow developmental process with a long delay of innervation as shown by some fibers with NCAM expression. Nevertheless, innervation appears to be successful, as suggested by the large number of NCAM negative fibers. Moreover, the abnormal myofiber distribution could be related to this functional disturbance of innervation.

Adult↗
PubMed1992

Defects of the mitochondrial respiratory chain complexes in three pediatric cases with hypotonia and cardiac involvement.

Three children displaying hypotonia, cardiac involvement and defects of the mitochondrial respiratory chain complexes are reported. The first case showed severe neonatal hypotonia, failure to thrive, hepatomegaly, dilation of the right cardiac cavities, profound lactic acidosis and amino aciduria. The boy died at the age of 7 weeks. In the second case hypotonia, severe cardiomyopathy, cyclic neutropenia, lactic acidosis and 3-methylglutaconic aciduria occurred. The boy died at the age of 27 months. The third case presented at the age of 16 months as an acute hypokinetic hypertrophic cardiomyopathy with transient hypotonia and mild lactic acidosis. Spontaneous clinical remission occurred. In all cases muscle biopsy was performed. Morphological studies failed to show ragged-red fibers but there was lipid storage myopathy and decreased cytochrome c oxidase activity. Biochemical studies confirmed the cytochrome c oxidase deficiency in muscle in all cases. It was associated with complex I III deficiency in case 1 and with severe deficits of all respiratory chain complexes in case 2. Post-mortem studies in case 1 indicated that complex IV was reduced in the liver but not in the heart and quantitative analysis of mtDNA revealed a depletion in muscle. Cases 1 and 2 shared some clinical features with fatal infantile myopathy associated with cytochrome c oxidase deficiency, while case 3 displayed a very unusual clinical presentation. The histochemical enzyme reaction of cytochrome c oxidase is useful for the diagnosis of mitochondrial myopathy because ragged-red fibers may be lacking. Finally, biochemical measurement of the different mitochondrial respiratory chain complexes is required because multiple defects are frequent and occasionally related to mtDNA depletion.

Abnormalities, Multiple↗
PubMed1992

Expression of various NCAM isoforms in human embryonic muscles: correlation with myosin heavy chain phenotypes.

Neural cell adhesion molecules (NCAM) are known to play a pivotal role in regulating cell-cell interactions in various tissues. The diversity of NCAM is made by alternative splicing of a single gene and by post-translational modifications. The spatio-temporal expression of the various isoforms is developmentally regulated and may modulate cell interactions. We investigated the expression of NCAM isoforms, in particular polysialylated and phosphatidylinositol-anchored isoforms, in developing psoas and quadriceps human muscle from 15 weeks of gestation to term. In parallel, we examined the expression of the myosin heavy chain phenotype (another developmentally regulated system) to determine whether polysialylated-NCAM molecules (the so-called embryonic NCAM) and developmental myosin heavy chains are coexpressed. Our results showed an expression of polysialylated-NCAM and phosphatidylinositol-anchored isoforms during the early stages of myotube maturation. The expression of polysialylated-NCAM on developing myotube was always associated with the expression of developmental myosin heavy chains. However, the loss of polysialylated-NCAM from maturing myotubes was not correlated with the disappearance of the developmental myosin heavy chains, but rather with the appearance of an adult myosin heavy chain phenotype. The relationship between polysialylated-NCAM and myosin heavy chain phenotype was similar in psoas and in quadriceps muscles. We observed that maturation of quadriceps muscle takes place earlier than psoas. Biochemical analysis showed that phosphatidylinositol-anchored molecules were never polysialylated; this indicates different roles of these isoforms in muscle development.

Aging↗