Schwannoma gigante de pelvis: Reporte de Caso / Giant pelvic Schawannoma: A case report

Introducción: Los Schwannomas son tumores derivados de las células de Schwann que se pueden presentar en cualquier lugar de la economía corporal, siendo poco usual su localización en la pelvis (1 a 3 %). Con presentación tórpida y sintomatología variada en relación con el sitio que ocupan. Su tratamiento se basa en la resección quirúrgica. Caso clínico: Presentamos el caso de un hombre joven de 21 años presentando dolor pélvico por 4 meses. Taller diagnóstico: en imágenes se evidencia una masa que ocupa toda la pelvis, esta es extirpada y revela como diagnóstico histopatológico. Neoplasia mesénquimal compatible con schwannoma. Conclusión: En conclusión, estos tumores son raros, y la localización pélvica es muy poco frecuente, su extirpación se vuelve curativa y debe realizarse de manera oportuna.

Introduction: Schwannomas are tumors derived from Schwann cells that can occur anywhere in the body economy, and their location is unusual in the pelvis (1 to 3%). With torpid presentation and varied symptoms about the site they occupy. Its treatment is based on surgical resection. Clinical case: We present the case of a 21-year-old man with pelvic pain for four months. Diagnostic workshop: Images show a mass that occupies the entire pelvis; this is removed and revealed as a histopathological diagnosis. Mesenchymal neoplasm compatible with schwannoma. Conclusion: In conclusion, these tumors are rare, and the pelvic location is very infrequent; their removal becomes curative and must be performed promptly.

Loss of Ephaptic Contacts in the Murine Thalamus during Osmotic Demyelination Syndrome.

BACKGROUND AND AIM: A murine model mimicking osmotic demyelination syndrome (ODS) revealed with histology in the relay posterolateral (VPL) and ventral posteromedial (VPM) thalamic nuclei adjoined nerve cell bodies in chronic hyponatremia, amongst the damaged 12 h and 48 h after reinstatement of osmolality. This report aims to verify and complement with ultrastructure other neurophysiology, immunohistochemistry, and molecular biochemistry data to assess the connexin-36 protein, as part of those hinted close contacts.This ODS investigation included four groups of mice: Sham (NN; n = 13), hyponatremic (HN; n = 11), those sacrificed 12 h after a fast restoration of normal natremia (ODS12h; n = 6) and mice sacrificed 48 h afterward, or ODS48 h (n = 9). Out of these, thalamic zones samples included NN (n = 2), HN (n = 2), ODS12h (n = 3) and ODS48h (n = 3). RESULTS: Ultrastructure illustrated junctions between nerve cell bodies that were immunolabeled with connexin36 (Cx36) with light microscopy and Western blots. These cell's junctions were reminiscent of low resistance junctions characterized in other regions of the CNS with electrophysiology. Contiguous neurons showed neurolemma contacts in intact and damaged tissues according to their location in the ODS zones, at 12 h and 48 h post correction along with other demyelinating alterations. Neurons and ephaptic contact measurements indicated the highest alterations, including nerve cell necrosis in the ODS epicenter and damages decreased toward the outskirts of the demyelinated zone. CONCLUSION: Ephapses contained C × 36between intact or ODS injured neurons in the thalamus appeared to be resilient beyond the core degraded tissue injuries. These could maintain intercellular ionic and metabolite exchanges between these lesser injured regions and, thus, would partake to some brain plasticity repairs.

Synergistic protection of RGCs by olfactory ensheathing cells and alpha-crystallin through regulation of the Akt/BAD Pathway.

BACKGROUND: Our recent in vivo studies have shown that olfactory ensheathing cells (OECs) and α-crystallin can promote retinal ganglion cell (RGC) survival and axonal regeneration synergistically after optic nerve injury. However, the mechanism is still unknown. OBJECTIVES: Here, we studied the synergistic effect and mechanism of OECs and α-crystallin on RGC survival after H2O2-induced oxidative damage and a crushing injury to the optic nerve in an adult rat model. METHODS: After H2O2-induced oxidative damage, RGC-5 cells were treated with OECs, α-crystallin or a combination of OECs and α-crystallin. Apoptosis of RGC-5 cells was assessed by flow cytometry. Phosphorylated Akt, BAD, and cleaved-caspase3 were detected by Western blot after optic nerve injury in vivo and H2O2-induced RGC-5 oxidative damage in vitro. RESULTS: The results showed that OECs and α-crystallin could both independently inhibit RGC-5 apoptosis (P<0.01), increase the phosphorylation of both Akt and BAD, and decrease the activation of caspase-3 (P<0.01). However, the effect of the combination of both was more significant than either alone. CONCLUSION: These findings indicate that inhibition of superoxide damage to RGCs through regulation of the Akt/BAD pathway is one of the mechanisms by which OECs and α-crystallin promote optic nerve recovery after injury.

Reprogramming cells from Gulf War veterans into neurons to study Gulf War illness.

Gulf War illness (GWI), which afflicts at least 25% of veterans who served in the 1990-1991 war in the Persian Gulf, is thought to be caused by deployment exposures to various neurotoxicants, including pesticides, anti-nerve gas pills, and low-level nerve agents including sarin/cyclosarin. GWI is a multisymptom disorder characterized by fatigue, joint pain, cognitive problems, and gastrointestinal complaints. The most prominent symptoms of GWI (memory problems, poor attention/concentration, chronic headaches, mood alterations, and impaired sleep) suggest that the disease primarily affects the CNS. Development of urgently needed treatments depends on experimental models appropriate for testing mechanistic hypotheses and for screening therapeutic compounds. Rodent models have been useful thus far, but are limited by their inability to assess the contribution of genetic or epigenetic background to the disease, and because disease-vulnerable proteins and pathways may be different in humans relative to rodents. As of yet, no postmortem tissue from the veterans has become available for research. We are moving forward with a paradigm shift in the study of GWI, which utilizes contemporary stem cell technology to convert somatic cells from Gulf War veterans into pluripotent cell lines that can be differentiated into various cell types, including neurons, glia, muscle, or other relevant cell types. Such cell lines are immortal and will be a resource for GWI researchers to pursue mechanistic hypotheses and therapeutics.

Evaluation of microtransplantation of rat brain neurolemma into Xenopus laevis oocytes as a technique to study the effect of neurotoxicants on endogenous voltage-sensitive ion channels.

Microtransplantation of mammalian brain neurolemma into the plasma membrane of Xenopus oocytes is used to study ion channels in their native form as they appear in the central nervous system. Use of microtransplanted neurolemma is advantageous for various reasons: tissue can be obtained from various sources and at different developmental stages; ion channels and receptors are present in their native configuration in their proper lipid environment along with appropriate auxiliary subunits; allowing the evaluation of numerous channelpathies caused by neurotoxicants in an ex vivo state. Here we show that Xenopus oocytes injected with post-natal day 90 (PND90) rat brain neurolemma fragments successfully express functional ion channels. Using a high throughput two electrode voltage clamp (TEVC) electrophysiological system, currents that were sensitive to tetrodotoxin, ω-conotoxin MVIIC, and tetraethylammonium were detected, indicating the presence of multiple voltage-sensitive ion channels (voltage-sensitive sodium (VSSC), calcium and potassium channels, respectively). The protein expression pattern for nine different VSSC isoforms (Nav1.1-Nav1.9) was determined in neurolemma using automated western blotting, with the predominant isoforms expressed being Nav1.2 and Nav1.6. VSSC were also successfully detected in the plasma membrane of Xenopus oocytes microtransplanted with neurolemma. Using this approach, a "proof-of-principle" experiment was conducted where a well-established structure-activity relationship between the neurotoxicant, 1,1,1-trichloro-2,2-di(4-chlorophenyl)ethane (DDT) and its non-neurotoxic metabolite, 1,1-bis-(4-chlorophenyl)-2,2-dichloroethene (DDE) was examined. A differential sensitivity of DDT and DDE on neurolemma-injected oocytes was determined where DDT elicited a concentration-dependent increase in TTX-sensitive inward sodium current upon pulse-depolarization whereas DDE resulted in no significant effect. Additionally, DDT resulted in a slowing of sodium channel inactivation kinetics whereas DDE was without effect. These results are consistent with the findings obtained using heterologous expression of single isoforms of rat brain VSSCs in Xenopus oocytes and with many other electrophysiological approaches, validating the use of the microtransplantation procedure as a toxicologically-relevant ex vivo assay. Once fully characterized, it is likely that this approach could be expanded to study the role of environmental toxicants and contaminants on various target tissues (e.g. neural, reproductive, developmental) from many species.

Schwannoma of the penis: a case report / Schwannoma del pene: presentación de un caso

Penile schwannoma is a rare tumor arising from the neurilemmal cells reported only a few in the literature. Here we report a case of penile schwannoma in a young adult treated successfully by local excision (AU)

Schwannoma del pene es un tumor poco común que surge de las células del neurilema, se han registrado solo unos pocos en la literatura. Aquí se presenta un caso de schwannoma del pene en un adulto joven tratado con éxito mediante extirpación (AU)

3-D Imaging Reveals Participation of Donor Islet Schwann Cells and Pericytes in Islet Transplantation and Graft Neurovascular Regeneration.

The primary cells that participate in islet transplantation are the endocrine cells. However, in the islet microenvironment, the endocrine cells are closely associated with the neurovascular tissues consisting of the Schwann cells and pericytes, which form sheaths/barriers at the islet exterior and interior borders. The two cell types have shown their plasticity in islet injury, but their roles in transplantation remain unclear. In this research, we applied 3-dimensional neurovascular histology with cell tracing to reveal the participation of Schwann cells and pericytes in mouse islet transplantation. Longitudinal studies of the grafts under the kidney capsule identify that the donor Schwann cells and pericytes re-associate with the engrafted islets at the peri-graft and perivascular domains, respectively, indicating their adaptability in transplantation. Based on the morphological proximity and cellular reactivity, we propose that the new islet microenvironment should include the peri-graft Schwann cell sheath and perivascular pericytes as an integral part of the new tissue.

Involvement of the Tyro3 receptor and its intracellular partner Fyn signaling in Schwann cell myelination.

During early development of the peripheral nervous system, Schwann cell precursors proliferate, migrate, and differentiate into premyelinating Schwann cells. After birth, Schwann cells envelop neuronal axons with myelin sheaths. Although some molecular mechanisms underlying myelination by Schwann cells have been identified, the whole picture remains unclear. Here we show that signaling through Tyro3 receptor tyrosine kinase and its binding partner, Fyn nonreceptor cytoplasmic tyrosine kinase, is involved in myelination by Schwann cells. Impaired formation of myelin segments is observed in Schwann cell neuronal cultures established from Tyro3-knockout mouse dorsal root ganglia (DRG). Indeed, Tyro3-knockout mice exhibit reduced myelin thickness. By affinity chromatography, Fyn was identified as the binding partner of the Tyro3 intracellular domain, and activity of Fyn is down-regulated in Tyro3-knockout mice, suggesting that Tyro3, acting through Fyn, regulates myelination. Ablating Fyn in mice results in reduced myelin thickness. Decreased myelin formation is observed in cultures established from Fyn-knockout mouse DRG. Furthermore, decreased kinase activity levels and altered expression of myelination-associated transcription factors are observed in these knockout mice. These results suggest the involvement of Tyro3 receptor and its binding partner Fyn in Schwann cell myelination. This constitutes a newly recognized receptor-linked signaling mechanism that can control Schwann cell myelination.

Intracochlear Schwannoma: Diagnosis and Management

Introduction: Schwannomas of the eighth cranial nerve are benign tumors that usually occur in the internal auditory canal or the cerebellopontine angle cistern. Rarely, these tumors may originate from the neural elements within the vestibule, cochlea, or semicircular canals and are called intralabyrinthine schwannomas. Intracochlear schwannomas (ICSs) represent a small percentage of these tumors, and their diagnosis is based on high-resolution magnetic resonance imaging (MRI). Objectives: To report the clinical and radiologic features and audiometric testing results of an ICS in a 48-year-old man after a 22-month follow-up period. Resumed Report A patient with an 8-year history of persistent tinnitus in his right ear, combined with ipsilateral progressive hearing loss and aural fullness. Audiometry revealed normal hearing in the left ear and a moderate to severe sensorineural hearing loss in the right ear, with decreased speech reception threshold and word recognition score, compared with the exam performed 5 years previously. MRI showed a small intracochlear nodular lesion in the modiolus, isointense on T1 with a high contrast enhancement on T1 postgadolinium images. During the follow-up period, there were no radiologic changes on imaging studies. Thus, a wait-and-scan policy was chosen as the lesion remained stable with no considerable growth and the patient still presents with residual hearing. Conclusions: Once diagnosed, not all ICS patients require surgery. Treatment options for ICS include stereotactic radiotherapy and rescanning policy, depending on the tumor's size, evidence of the tumor's growth, degree of hearing loss, intractable vestibular symptoms, concern about the pathologic diagnosis, and the patient's other medical conditions...

Schwannoma of the Ulnar Nerve in the Elbow: A Case Report

Schwannomas are soft tissue sarcomas arising from neurilemma of Schwann cells in peripheral nerves, and is the most frequent type of benign tumor found in these nerves. We report a case of a Schwannoma of the ulnar nerve in the elbow joint, and along this report, give a review of the literature. A 46-year-old male patient was hospitalized with complaints of swelling and pain in the left elbow and a tingling sensation and hypoesthesia of the fourth and fifth fingers. Physical examination of the patient showed he was positive for Tinel's sign, and magnetic resonance imaging results demonstrated the presence of a Schwannoma. Subsequent biopsy and excision of the Schwannoma was carried out. The suspected mass, which had a clear margin separating it from the healthy nerve of the medial left elbow, was removed along with its 2 x 2 x 3 cm capsule after a histological diagnosis of a Schwannoma was made. Pathophysiological results confirmed the excised mass as a Schwannoma. Schwannoma of the ulnar nerve within the elbow joint is rare and differential diagnosis is difficult. Therefore, treatment can only proceed after the presence of Schwannoma has been confirmed by physical and radiological examinations.

Schwannoma of the Ulnar Nerve in the Elbow: A Case Report

Schwannomas are soft tissue sarcomas arising from neurilemma of Schwann cells in peripheral nerves, and is the most frequent type of benign tumor found in these nerves. We report a case of a Schwannoma of the ulnar nerve in the elbow joint, and along this report, give a review of the literature. A 46-year-old male patient was hospitalized with complaints of swelling and pain in the left elbow and a tingling sensation and hypoesthesia of the fourth and fifth fingers. Physical examination of the patient showed he was positive for Tinel's sign, and magnetic resonance imaging results demonstrated the presence of a Schwannoma. Subsequent biopsy and excision of the Schwannoma was carried out. The suspected mass, which had a clear margin separating it from the healthy nerve of the medial left elbow, was removed along with its 2 x 2 x 3 cm capsule after a histological diagnosis of a Schwannoma was made. Pathophysiological results confirmed the excised mass as a Schwannoma. Schwannoma of the ulnar nerve within the elbow joint is rare and differential diagnosis is difficult. Therefore, treatment can only proceed after the presence of Schwannoma has been confirmed by physical and radiological examinations.

Dependence of paranodal junctional gap width on transverse bands.

Mouse mutants with paranodal junctional (PNJ) defects display variable degrees of neurological impairment. In this study we compare control paranodes with those from three mouse mutants that differ with respect to a conspicuous PNJ component, the transverse bands (TBs). We hypothesize that TBs link the apposed junctional membranes together at a fixed distance and thereby determine the width of the junctional gap, which may in turn determine the extent to which nodal action currents can be short-circuited underneath the myelin sheath. Electron micrographs of aldehyde-fixed control PNJs, in which TBs are abundant, show a consistent junctional gap of ∼3.5 nm. In Caspr-null PNJs, which lack TBs entirely, the gap is wider (∼6-7 nm) and more variable. In CST-null PNJs, which have only occasional TBs, the mean PNJ gap width is comparable to that in Caspr-null mice. In the shaking mutant, in contrast, which has approximately 60% of the normal complement of TBs, mean PNJ gap width is not significantly different from that in controls. Correspondingly, shaking mice are much less impaired neurologically than either Caspr-null or CST-null mice. We conclude that in the absence or gross diminution of TBs, mean PNJ gap width increases significantly and suggest that this difference could underlie some of the neurological impairment seen in those mutants. Surprisingly, even in the absence of TBs, paranodes are to some extent maintained in their usual form, implying that in addition to TBs, other factors govern the formation and maintenance of overall paranodal structure.

Expression of protein kinase-C substrate mRNA in the motor cortex of adult and infant macaque monkeys.

To understand the molecular and cellular bases of plasticity in the primate motor cortex, we investigated the expression of three protein kinase-C (PKC) substrates: GAP-43, myristoylated alanine-rich C-kinase substrate (MARCKS), and neurogranin, which are key molecules regulating synaptic plasticity. Prominent signals for the three mRNAs were primarily observed in pyramidal cells. Large pyramidal cells in layer V, from which the descending motor tract originates, contained weaker hybridization signals for GAP-43 and neurogranin mRNAs than did the smaller pyramidal cells. We also performed double-label in situ hybridization showing that GAP-43 and neurogranin mRNAs were expressed in a subset of MARCKS-positive neurons. Quantitative analysis showed that the expression was different between the layers: layer VI contained the strongest and layer II the weakest signals for all three mRNAs. The expression levels of GAP-43 and MARCKS mRNA in layer V were higher than in layer III, while the expression level of neurogranin mRNA in layer V was almost the same as in layer III. Developmental analysis from the newborn to adult indicated that the expression levels of the three mRNAs were higher in the infant motor cortex than in the adult. The expression of both GAP-43 and neurogranin mRNAs transiently increased over several months postnatally. The present study showed that the expression of the three PKC substrates was specific to cell types, cortical layers, and postnatal developmental stage. The specific expression may reflect functional specialization for plasticity in the motor cortex of both infants and adults.

Immunohistochemical characterization of capsular cells in neuromuscular spindles of the neck.

BACKGROUND: To identify capsular components of neuromuscular spindles in man by means of immunohistochemistry. METHODS: Investigation of histologically observed neuromuscular spindles in surgical specimens with the use of markers for sheath cells and basement membranes. RESULTS: Epithelial membrane antigen and CD34 immunoreactivities were found in the outer and inner capsular layers, respectively. S-100 protein was not expressed in the capsules and there was more collagen type IV than laminin. CONCLUSIONS: Cells resembling perineurial cells and endoneurial fibroblasts, and basement membrane rich in collagen type IV comprise the capsules of neuromuscular spindles.

¿Nódulos axonales?. Réplica / Axonal nodes? Reply

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The optic nerve sheath on MRI in acute optic neuritis.

Optic nerve sheath dilatation or gadolinium-enhancement on magnetic resonance imaging in acute optic neuritis have been previously reported but have been thought to be rare occurrences. This study recruited 33 patients with acute unilateral optic neuritis. All had their optic nerves imaged with fat-saturated fast spin-echo (FSE) imaging, and 28 had imaging before and after triple-dose gadolinium-enhanced fat-saturated T(1)-weighted imaging. Follow-up imaging was performed on 20 patients (15 following gadolinium). A dilated subarachnoid space at the anterior end of the symptomatic optic nerve on FSE imaging was seen in 15/33 cases. In three of these cases, dilatation was visible on short-term follow-up. Optic nerve sheath enhancement was seen in 21/28 cases acutely: seven at the anterior end of the lesion only, five at the posterior end only and nine at both ends. Optic sheath enhancement was seen in 13 patients on follow-up. This study suggests that optic nerve sheath dilatation on FSE images and optic nerve sheath enhancement on triple-dose gadolinium-enhanced images are common findings in acute optic neuritis. Optic nerve sheath dilatation may be due to inflammation of the optic nerve, with its associated swelling, interrupting the communication between the subarachnoid space of the diseased optic nerve and the chiasmal cistern. Optic nerve sheath enhancement suggests that meningeal inflammation occurs in optic neuritis, in agreement with pathological studies of both optic neuritis and multiple sclerosis.

[Metallophilia in the Ranvier's nodes of nerve fibers in rats of different strains (histochemical study)]. / Metallofiliia uzlovykh perekhvatov nervnykh volokon u krys razlichnykh linii (gistokhimicheskoe issledovanie)

Myelinated nerve fibers formed by the processes of LV spinal ganglion neurons were studied in two lines of rats selected according to high and low thresholds of nerve system excitability to electric current. Before ultrastructural study the fibres have been treated with potassium pyroantimonate. It was demonstrated that specific dense precipitate was deposited in the nodes of Ranvier of the nerve fibers of rats with low excitability thresholds; this precipitate was not found in the fibers of rats with high thresholds. It is suggested that deposition of precipitate is indicative of a high density of sodium channels in neurilemma, i.e. of high functional activity of nerve fibers.

Growth characteristics of ameloblastoma involving the inferior alveolar nerve: a clinical and histopathologic study.

OBJECTIVE: Growth characteristics of ameloblastomas involving the inferior alveolar nerve were examined to determine the most appropriate surgical management of the nerve at the time of the surgical procedure. STUDY DESIGN: Clinical and histopathologic examinations were performed on 22 resected mandibles in which the inferior alveolar nerve was lying adjacent to, or contained within, the tumor. RESULTS: Patterns of tumor involvement of the nerve bundle were evaluated with respect to the presence of bone (11 patients) or connective tissue wall (7 patients) between the tumor and the nerve bundle, and tumor infiltration of perineural connective tissue (4 patients). Neither invasion into the nerve sheath nor invasion into the nerve itself by the ameloblastoma was detected. Tumor infiltration of the tissue surrounding the nerve was identified for the multicystic and solid types but not for the unicystic type. Presence of bone or connective tissue wall between the tumor and the nerve bundle was dominant in the unicystic and plexiform ameloblastomas, whereas tumor infiltration of the perineural tissue was frequently observed in ameloblastomas with the follicular pattern. CONCLUSION: The preservation of the inferior alveolar nerve may be possible in the management of the unicystic type of ameloblastoma. However, a more radical approach is necessary for treatment of multicystic or solid tumors, especially those exhibiting a follicular pattern.

Optic nerve sheath fenestration for a reversible optic neuropathy in radiation oncology.

To the authors' knowledge, there is a paucity of published accounts of management of radiation-induced optic neuropathy (RION) by optic nerve sheath fenestration (ONSF) in the conventional medical literature. With higher doses of radiation being given by using conformal techniques, more radiation-induced optic neuritis and neuropathy will be identified. We report here the successful use of ONSF to restore vision to three consecutive patients with pending anterior RION, and the importance of early identification and intervention in these potentially reversible cases.