scholarly journals Neuroblastoma Soft Tissue Metastasis in a 10-Month-Old Infant with a Right Thigh Mass

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Shokouh Taghipour Zahir ◽  
Fateme Salemi

Background. Neuroblastoma is a solid tumor that occurs more frequently in pediatric populations. It may originate from any part of the sympathetic nervous system, but it most commonly arises from the paraspinal sympathetic ganglia in the abdomen or mediastinum. Local lymphadenopathy and distant metastasis to the central nervous system, orbit, and liver might be detected; however, it rarely includes soft tissue or musculoskeletal involvement. Case Report. Here, we report a 10-month-old infant presented with a right thigh mass with an otherwise benign physical exam and medical history. MRI of the lower extremities suggested tumoral infiltration in the soft tissue of both thighs, predominantly on the right side. Surgical pathology of the lesion confirmed neuroblastoma. A large subhepatic mass and paraaortic lymphadenopathy in the abdominal CT scan and metaiodobenzylguanidine scan findings favored primary abdominal neuroblastoma that had spread to lower extremities. The patient has been in remission since the completion of chemotherapy. Conclusion. Neuroblastoma should be considered in infants with an abnormal mass in extremities. Due to its aggressive nature, most patients struggle with distant and local tumor spread at diagnosis. Therefore, any abnormal signs and symptoms, especially in younger pediatrics, warrant immediate evaluation to avoid tumor expansion.

2021 ◽  
Vol 13 (1) ◽  
pp. 259-266
Author(s):  
Ye-Tao Zhu ◽  
Yang Liu ◽  
Li-Gang Chen ◽  
Da-Ping Song

Solitary fibrous tumor is a very rare mesenchymal tumor that occurs mostly in the pleura, and there are few reported cases of a presence in the central nervous system, particularly in the cerebellum. In 2016, the WHO classified solitary fibrous tumors into grade I. In this article, we present a case of malignant solitary fibrous tumor recurring 8 years after surgery in a 63-year-old male. Magnetic resonance imaging showed low to intermediate mixed signal intensity on T1W1. Immunohistochemical staining positivity for Vimentin, CD99, CD34 and Bcl-2, it is consistent with the immunohistochemical characteristics of solitary fibrous tumor. We resected the patient’s tumor, and the patient was followed up for 3 months with no signs of recurrence. Solitary fibrous tumors are very rare in the central nervous system. Immunohistochemical staining positivity for CD34 and Bcl-2 is strongly expressed in most solitary fibrous tumor. Surgical resection is the preferred treatment. Due to the small number of cases, the biological behavior and prognosis of this tumor need to be further explored.


2015 ◽  
Vol 309 (10) ◽  
pp. C660-C668 ◽  
Author(s):  
Victoria L. Hodgkinson ◽  
Sha Zhu ◽  
Yanfang Wang ◽  
Erik Ladomersky ◽  
Karen Nickelson ◽  
...  

Menkes disease is a fatal neurodegenerative disorder arising from a systemic copper deficiency caused by loss-of-function mutations in a ubiquitously expressed copper transporter, ATP7A. Although this disorder reveals an essential role for copper in the developing human nervous system, the role of ATP7A in the pathogenesis of signs and symptoms in affected patients, including severe mental retardation, ataxia, and excitotoxic seizures, remains unknown. To directly examine the role of ATP7A within the central nervous system, we generated Atp7a Nes mice, in which the Atp7a gene was specifically deleted within neural and glial cell precursors without impairing systemic copper homeostasis, and compared these mice with the mottled brindle ( mo-br) mutant, a murine model of Menkes disease in which Atp7a is defective in all cells. Whereas mo-br mice displayed neurodegeneration, demyelination, and 100% mortality prior to weaning, the Atp7a Nes mice showed none of these phenotypes, exhibiting only mild sensorimotor deficits, increased anxiety, and susceptibility to NMDA-induced seizure. Our results indicate that the pathophysiology of severe neurological signs and symptoms in Menkes disease is the result of copper deficiency within the central nervous system secondary to impaired systemic copper homeostasis and does not arise from an intrinsic lack of ATP7A within the developing brain. Furthermore, the sensorimotor deficits, hypophagia, anxiety, and sensitivity to NMDA-induced seizure in the Atp7a Nes mice reveal unique autonomous requirements for ATP7A in the nervous system. Taken together, these data reveal essential roles for copper acquisition in the central nervous system in early development and suggest novel therapeutic approaches in affected patients.


PEDIATRICS ◽  
1973 ◽  
Vol 52 (3) ◽  
pp. 449-451
Author(s):  
Barry H. Rumack

The increased incidence of poisoning by overdoses of commonly used drugs with anticholinergic properties (Table I) and the general lack of knowledge concerning a specific treatment for these poisons warrants a summary of the problem at this time. Some plants containing anticholinergic alkaloids are also included in this group as they may also be taken intentionally or accidentally. Drugs with anticholinergic properties primanly antagonize acetylcholine competitively at the neuroreceptor site. Cardiac muscle, exocrine glands, and smooth muscle are most markedly affected.1 Action of the inhibitors is overcome by increasing the level of acetylcholine naturally generated in the body through inhibiting the enzyme (choline esterase) which normally prevents accumulation of excess acetylcholine. It does this by hydrolyzing that compound to inactive acetic acid and choline. Agents which inhibit this enzyme, so that acetylcholine accumulates at the neuroreceptor sites, are called anticholine esterases. Physostigmine, one of the anticholine esterases which is a tertiary amine, crosses into the central nervous system and can reverse both central and peripheral anticholinergic actions2. Neostigmine and pyridostigmine are also anticholine esterases but they are quaternary amines and are capable of acting only outside the central nervous system because of solubility and ionization characteristics. The anticholinergic syndrome has both central and peripheral signs and symptoms. Central toxic effects include anxiety, delirium, disorientation, hallucinations, hyperactivity, and seizures.2 Severe poisoning may produce coma, medullary paralysis, and death. Peripheral taxicity is characterized by tachycardia, hyperpyrexia, mydriasis, vasodilatation, urinary retention, diminution of gastrointestinal motility, decrease of secretion in salivary and sweat glands, and loss of secretions in the pharynx, bronchi, and nasal passages.


1945 ◽  
Vol 22 (1-2) ◽  
pp. 63-74
Author(s):  
JOSEPH BARCROFT ◽  
D. H. BARRON

1. A method (the needle method) is described for the measurement of the pressure in the stream going through a vessel. 2. In the foetal sheep the needle method applied to the umbilical artery gives substantially the same results as the mercurial manometer applied to the carotid, until about half-way through the gestation period. 3. As gestation proceeds the needle method applied at the first moment at which it can be applied to the umbilical artery (or a branch) gives readings substantially lower, and increasingly lower as gestation proceeds, than does the mercurial manometer read at the first moment at which it can be read. 4. The discrepancy is due to the sum of a number of causes which are discussed, but of these the most important is an actual rise of pressure between the time of delivery and the completion of the dissections contingent on the use of the mercurial manometer. 5. The cause of this is not at present demonstrated, but either or both of two factors may be concerned: (a) a dulling of the central nervous system which weakens the depressor reflex; (b) the establishment of a greater degree of vasomotor tone consequent on the bombardment of the central nervous system with sensory stimuli. 6. The pulse rates in utero and just after delivery of the foetus into a saline bath at 39-40°C. (the umbilical circulation being unimpaired) are not significantly different. 7. The pulse rate quickens up to the 70th-80th day, after which it becomes slower as gestation proceeds. 8. If both vagi be severed, the pulse rate te to quicken throughout gestation. The pulse, therefore, comes increasingly under vagus inhibition from the 80th-90th day onwards. 9. Even after the vagi have been cut after the 120th day (it has not been tried before) adrenalin in sufficient quantity will cause a further quickening of the pulse. 10. The earliest date at which stimulation of the peripheral end of the right vagus was observed to slow the heart was the 77th day. On the 85th day peripheral stimulation of the left vagus also failed, but succeeded on the 101st day. 11. Central stimulation of the left vagus, with the right vagus intact, produced slowing on the 77th day. 12. Slowing of the heart synchronous with rise of arterial pressure has been observed on the 111th day. 13. Slowing of the heart which bears evidence of being reflex has been obtained by raising the blood pressure (clamping the cord) on the 121st day and by injection of adrenalin on the 118th day. 14. Approaching term both the carotid sinus and cardiac depressor mechanisms are functional. 15. Lowering of the blood pressure as the result of stimulation of the central end of the vagus and with both vagi severed can be demonstrated late in gestation.


Neurosurgery ◽  
1981 ◽  
Vol 8 (3) ◽  
pp. 391-396 ◽  
Author(s):  
Nelson G. Ordóñez ◽  
Alberto G. Ayala ◽  
Milam E. Leavens

Abstract The case of a 33-year-old woman with oligodendroglioma with extraneural metastases involving the masseter muscle, the cervical lymph nodes, the lumbar vertebrae, and the right iliac bone is described. Oligodendroglioma with metastases outside the central nervous system is extremely rare, and only a very few cases have been reported.


1998 ◽  
Vol 1 (4) ◽  
pp. 314-318 ◽  
Author(s):  
Calvin E. Oyer ◽  
Nina G. Tatevosyants ◽  
Selina C. Cortez ◽  
Abby Hornstein ◽  
Michael Wallach

Cleidocranial dysplasia (CCD), an uncommon disorder involving membranous bones, is rarely lethal in early life. The calvaria is defective and wormian bones are present. Abnormalities of the clavicles vary in severity from a minor unilateral defect to bilateral absence. This report concerns pre- and postmortem anatomical and radiological findings in a 15-day-old female neonate with CCD. Her postnatal course was characterized by seizures and recognition of hydrocephalus during the first day of life. The calvaria was hypoplastic with numerous wormian bones. A pseudofracture of the right clavicle was present. Hydrocephalus was present in the brachycephalic brain which had a severely thinned cerebral cortex. Hemosiderin in the ventricular lining and marked subependymal gliosis were interpreted as evidence of old intraventricular hemorrhage that had occurred in utero. A CCD-related condition, Yunis-Varon syndrome (YVS), is noted for early lethality and for developmental and secondary abnormalities of the central nervous system. The present case only partially matches the phenotype of YVS and might represent a part of a spectrum of phenotypic variants ranging from viable CCD to lethal YVS.


2021 ◽  
Vol 17 (3) ◽  
pp. 65-77
Author(s):  
N. V. Tsygan ◽  
A. P. Trashkov ◽  
A. V. Ryabtsev ◽  
V. A. Yakovleva ◽  
A. L. Konevega ◽  
...  

Detailed clinical assessment of the central nervous system involvement in SARS-CoV-2 infection is relevant due to the low specificity of neurological manifestations, the complexity of evaluation of patient complaints, reduced awareness of the existing spectrum of neurological manifestations of COVID-19, as well as low yield of the neurological imaging.The aim. To reveal the patterns of central nervous system involvement in COVID-19 and its pathogenesis based on clinical data.Among more than 200 primary literature sources from various databases (Scopus, Web of Science, RSCI, etc.), 80 sources were selected for evaluation, of them 72 were published in the recent years (2016-2020). The criteria for exclusion of sources were low relevance and outdated information.The clinical manifestations of central nervous system involvement in COVID-19 include smell (5-98% of cases) and taste disorders (6-89%), dysphonia (28%), dysphagia (19%), consciousness disorders (3-53%), headache (0-70%), dizziness (0-20%), and, in less than 3% of cases, visual impairment, hearing impairment, ataxia, seizures, stroke. Analysis of the literature data revealed the following significant mechanisms of the effects of highly contagious coronaviruses (including SARS-CoV-2) on the central nervous system: neurodegeneration (including cytokine- induced); cerebral thrombosis and thromboembolism; damage to the neurovascular unit; immune-mediated damage of nervous tissue, resulting in infection and allergy-induced demyelination.The neurological signs and symptoms seen in COVID-19 such as headache, dizziness, impaired smell and taste, altered level of consciousness, bulbar disorders (dysphagia, dysphonia) have been examined. Accordingly, we discussed the possible routes of SARS-CoV-2 entry into the central nervous system and the mechanisms of nervous tissue damage.Based on the literature analysis, a high frequency and variability of central nervous system manifestations of COVID-19 were revealed, and an important role of vascular brain damage and neurodegeneration in the pathogenesis of COVID-19 was highlighted.


2020 ◽  
Vol 20 (S1) ◽  
pp. 128-135
Author(s):  
O Litovchenko ◽  
A Maximova

Aim. The paper aims to reveal the features of the central nervous system and interhemispheric asymmetry in volleyball players with hearing impairment. Materials and methods. The study involved 14 athletes of various sports experience with varying degrees of hearing impairment aged from 20 to 35 years (average age 29.20 ± 1.3 years). Simple visual reaction was studied in terms of the reaction time, reaction stability, and functional capabilities of the nervous system. To establish the manual asymmetry, the M. Annette questionnaire and motor tests (dynamometry, tests for intertwined fingers, the applause test) were used, the coefficient of the right hand was calculated. When detecting visual asymmetry, the Rosenbach test and the telescope test were used. The coefficient of the right eye was also calculated. Results. It was established that a third of the examined athletes had a symmetric distribution of signs in both the visual and manual spheres. The reliable dependence between symmetric distribution and sports titles was not found. The reaction time in volleyball players with hearing impairment was reduced. The reaction stability and the level of functional capabilities were within the normal range and had a significant dependence on the sports rank and category. Conclusion. It was revealed that volleyball players with hearing impairment formed a special type of interhemispheric interaction characterized by a more uniform distribution between the right and left hemispheres, which resulted in low values of manual and visual asymmetry.


Author(s):  
Veronica Murta ◽  
Alejandro Villarreal ◽  
Alberto Javier Ramos

With confirmed COVID-19 cases surpassing the 8.5 million mark around the globe, there is an imperative need to deepen the efforts from the international scientific community to gain comprehensive understanding of SARS-CoV-2. Although the main clinical manifestations are associated with respiratory or intestinal symptoms, reports of specific and non-specific neurological signs and symptoms, both at presentation or during the course of the acute phase, are increasing. Approximately 25-40% of the patients present neurological symptoms. The etiology of these neurological manifestations remains obscure, and probably involves several direct pathways, not excluding the direct entry of the virus to the Central Nervous System (CNS) through the olfactory epithelium, circumventricular organs, or disrupted blood-brain barrier (BBB). Furthermore, neuroinflammation might occur in response to the strong systemic cytokine storm described for COVID-19, or due to dysregulation of the CNS angiotensin system. Descriptions of neurological manifestations in patients in the previous coronavirus (CoV) outbreaks have been numerous for the SARS-CoV and lesser for MERS-CoV. Strong evidence from patients and experimental models suggests that some human variants of CoV have the ability to reach the CNS and that neurons, astrocytes and/or microglia can be target cells for CoV. A growing body of evidence shows that astrocytes and microglia have a major role in neuroinflammation, responding to local CNS inflammation and/or to dysbalanced peripheral inflammation. This is another potential mechanism for SARS-CoV-2 damage to the CNS. In this work we will summarize the known neurological manifestations of SARS-CoV-2, SARS-CoV and MERS-CoV, explore the potential role for astrocytes and microglia in the infection and neuroinflammation, and compare them with the previously described human and animal CoV that showed neurotropism. We also propose possible underlying mechanisms by focusing on our knowledge of glia, neurons, and their dynamic intricate communication with the immune system.


2018 ◽  
Vol 4 (02) ◽  
pp. 088-090
Author(s):  
Sohael Khan ◽  
Suvarn Gupta ◽  
Shashank Jain ◽  
Shraddha Singhania

AbstractTuberculomas of the central nervous system are common. However, the incidence of intraspinal intramedullary tuberculomas is very low, and a combination of intramedullary, intracranial, and pulmonary tuberculomas is extremely rare. We report a case of tuberculoma involving the brain and spine with pulmonary tuberculosis in a 46-year-old man initially presenting with back pain and weakness of both lower extremities. Conservative treatment with antituberculous medications resulted in complete resolution of his symptoms.


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