Kernicterus in Rats with an Inherited Deficiency of Glucuronyal Transferase

PEDIATRICS ◽  
1959 ◽  
Vol 24 (1) ◽  
pp. 73-73
Keyword(s):  
Binding Sites ◽  
Renal Excretion ◽  
Practical Importance ◽  
Human Beings ◽  
Human Infants ◽  
Toxic Agent ◽  
History Of ◽  
The Central Nervous System ◽  
The Brain ◽  
Comprehensive Study

Aside from the great theoretic interest, the experiments described in this paper have considerable practical importance in providing a means of studying the efficacy of treatments designed to prevent kernicterus. A comprehensive study of a strain of rats (the Gunn strain) which have a hereditary deficiency of the enzyme required to conjugate bilirubin, and thus develope jaundice due to increased concentration of unconjugated bilirubin in the blood and tissues. The rats developed kernicterus which was apparently identical with that seen in human beings and is the only example of kernicterus in animals that fulfills rigid criteria outlined by the authors. Extensive data on the natural history of the bilirubinemia and development of kernicterus in the rats, as determined by chemical and pathologic techniques, are provided. Bilirubin itself is incriminated as the toxic agent producing the characteristic changes in the brain in kernicterus. Sulfonamides were found to augment the toxic effects of bilirubin, apparently because of competition between bilirubin and sulfonamides for binding sites on serum albumin. Neither infection nor hypoxia appeared to aggravate the effects of bilirubin. Administration of sodium glucuronate to jaundiced rats was followed by a decrease in bilirubin in the serum which at times exceeded 50%. This was not accompanied by any postponement of the onset of signs of damage to the central nervous system and did not prevent development of kernicterus. It appeared that the decrease in bilirubin in the serum may have resulted from an increased transferral to tissues rather than elimination through renal excretion. On the basis of the knowledge of this strain of rats, it should be possible to explore the usefulness of proposed therapeutic regimens in the experimental animal without jeopardizing the course of human infants who might be successfully treated with exchange transfusion pending the discovery of a more satisfactory therapy.

scholarly journals Intracranial And Intraorbital Rosai Dorfman Disease

2020 ◽  
Vol 11 (4) ◽  
pp. 5187-5191
Author(s):  
Sivapriya G Nair ◽  
Jina Raj ◽  
Sajesh K Menon ◽  
Suhas Udayakumaran ◽  
Roshni P R
Keyword(s):  
Histopathological Study ◽  
Disease Treatment ◽  
Mri Scan ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
History Of ◽  
The Central Nervous System ◽  
The Right ◽  
The Brain ◽  
Suprasellar Extension

Rosai Dorfman disease is a rare histiocytic disorder. It is also known as Sinus Histiocytosis. It is with massive lymphadenopathy involves an overproduction of a type of white blood cell. The disease is rarely associated with intracranial and intraorbital involvement. Intracranial Rosai-Dorfman can mimic meningioma. Other pathologies also underline its pathologies. Here, we report a nine-year-old boy with a history of proptosis of the right eye and presenting with multiple skull lesions. Histopathological study revealed Sphenopetroclival lesion, which features that of Rosai Dorfman Disease. His MRI scan of the brain was taken, which showed evidence of right optic nerve meningioma with sella and suprasellar extension, causing severe proptosis. The child underwent right frontotemporal craniotomy with petrosectomy and Transylvanian, subtemporal approach to multicompartmental Rosai-Dorfmans lesion. After four months, the patient had a recurrence of the disease on which chemotherapy and steroids were started, which also did not show much response while taking an MRI scan. A corticosteroid is a useful option in the Central Nervous System Rosai Dorfman disease treatment. But this patient showed a negative outcome to the treatment.

scholarly journals Rosai-Dorfman Disease – A Case Report

2019 ◽  
Author(s):  
Oldřich Medek ◽  
Petra Kašparová ◽  
Michael Bartoš ◽  
Petr Krůpa ◽  
Blanka Klimova ◽  
...  
Keyword(s):  
Resonance Imaging ◽  
Case Presentation ◽  
Adjacent Tissue ◽  
Organ Systems ◽  
Rosai Dorfman Disease ◽  
History Of Disease ◽  
History Of ◽  
The Central Nervous System ◽  
Significant Regression ◽  
The Brain

Abstract Background: The aim is to discuss a case of a meningeal form of Rosai-Dorfman disease, is a rare haematological disease, primarily affecting lymph nodes, but it can also affect most organ systems of the human body, including the central nervous system. So far, there have been over 650 cases of this disease described in literature. Case presentation: This report deals with a young, 40-year-old man with a negative history of disease, who observed a loss of smell and taste, without a history of anxiety or an injury. The patient was hospitalized and completely examined. Consequently, a navigational biopsy of the brain was indicated. The findings showed that the patient suffered from Rosai-Dorfman disease with reactive gliosis in adjacent tissue without evidence of amyloid. Conclusions: In January, March, and then in May 2018, the patient was checked for the magnetic resonance imaging of the brain, according to which there was a significant regression of the finding, and according to the outpatient reports of the same year, the taste and vision of the left eye are gradually being adjusted. The patient was treated with new corticosteroids, which proved to be effective.

scholarly journals Correlation of gastro-oesophageal reflux disease with psycological factors: A viscous cycle?

2021 ◽  
Vol 1 (5) ◽  
Author(s):  
Akriti Sharma ◽  
◽  
Garima Singal ◽  
Urvi Saini ◽  
◽  
...  
Keyword(s):  
Central Nervous System ◽  
Reflux Disease ◽  
Psychological Factors ◽  
Intestinal Tract ◽  
The Other ◽  
Anxiety And Depression ◽  
History Of ◽  
The Central Nervous System ◽  
Gastro Intestinal Tract ◽  
The Brain

Gastroesophageal reflux disease (GERD), a disorder of the gastro-intestinal tract is often associated with psychological factors such as anxiety and depression. Researchers across the globe are divided in their views about psychological factors being the cause or sequalae of GERD. While some researchers believe that psychiatric disorders contribute to GERD, others suggest that unresolved GERD leads to symptoms of anxiety and depression. Through this article we aim to highlight, that the brain-gut axis acts as a strong link between the central nervous system and Gastrointestinal tract and that there is a likelihood of a viscous cycle of psychological factors leading to symptoms of GERD, and unresolved GERD contributing to further worsening of psychological illnesses. In our opinion, while treating patients for either of the two disorders, history of the other one needs to be excluded and if GERD and psychological illnesses are found to co-exist, the two must be treated simultaneously as the treatment of one maybe incomplete without treatment of the other.

Acute Measles Encephalitis in Children With Immunosuppression

PEDIATRICS ◽  
1977 ◽  
Vol 59 (2) ◽  
pp. 232-239
Author(s):  
Jean Aicardi ◽  
Francoise Goutieres ◽  
Maria-Leonor Arsenio-Nunes ◽  
Pierre Lebon
Keyword(s):  
Measles Virus ◽  
Subacute Sclerosing Panencephalitis ◽  
Immunosuppressive Drugs ◽  
Prominent Feature ◽  
Epilepsia Partialis Continua ◽  
Fatal Course ◽  
Immunosuppressed Patients ◽  
History Of ◽  
The Central Nervous System ◽  
The Brain

Four cases of encephalitis occurring in children treated for lymphatic malignancies by immunosuppressive drugs are reported. Measles virus was isolated from the brain in one case and identified immunologically in another. Nucleocapsids identical to those seen in subacute sclerosing panencephalitis were demonstrated in three cases. Severe immunosuppression was evidenced in two patients by failure of rosette formation and low phytohemagglutinin tests. Pathologically, the inflammatory reaction was absent in one brain and moderate in two. Clinically, epilepsia partialis continua was a prominent feature in three patients. A history of measles or of contact was elicited in three cases, five weeks to three months before onset. All cases ran an acute fatal course. Measles virus can behave as an opportunistic invader of the central nervous system in children and the diagnosis of measles encephalitis should be considered in immunosuppressed patients.

scholarly journals Levels of neurocognitive response in the situation of organic brain damage of a child as a clinical and psychological problem

2020 ◽  
Vol LII (1) ◽  
pp. 38-42
Author(s):  
Natalya G. Turovskaya
Keyword(s):  
Statistical Analysis ◽  
Age Of Onset ◽  
Psychological Problem ◽  
Sensitive Period ◽  
Psychological Study ◽  
Mental Functions ◽  
History Of ◽  
The Central Nervous System ◽  
Diagnostic Complex ◽  
The Brain

Aim. The article discusses the results of a psychological study of the development of mental functions in children with convulsive paroxysms in the anamnesis. In order to identify common patterns of mental dysontogenesis in children with cerebral organic pathology, the data obtained are compared with the results of other studies. Methods. As experimental research methods, methods of neuropsychological diagnosis of higher mental functions in children were used (Tsvetkova, 2002), the diagnostic complex Forecast and prevention of learning problems at school (Yasyukova, 2002). Statistical analysis was performed using the SPSS statistical analysis software. Results. The results of the study showed that the early onset of seizures in children is combined with impaired auditory perception, short-term speech memory, visual linear thinking and motor awkwardness. The prolonged presence of convulsive paroxysms in the history of a preschool child is associated with the pathology of the development of kinesthetic praxis, as well as speech functions and forms of thinking associated with speech. A significant number of seizures in the history of the disease has a more destructive and total effect on the brain of the child in comparison with the age of onset and the duration of the disease. The data obtained are similar to the results of psychological studies of children with other cerebral organic pathology. Consequently, pathological cerebral processes and conditions of various nature can lead to similar disorders in the development of mental functions, presumably those that are at the time the pathological factor begins in the sensitive period of development. This allows using the concept of levels of neurocognitive response in a situation of organic damage to the brain of a child. Conclusion. The picture of the mental development disorder of children with convulsive paroxysms in the anamnesis is mediated by the age of onset, the duration and massiveness of the disease. Children with various diseases of the central nervous system, presumably, have common patterns of mental dysontogenesis.

scholarly journals Salmonella Typhimurium - associated meningoencephalomyelitis in a foal

2019 ◽  
Vol 49 (8) ◽  
Author(s):  
Jéssica Guerra de Oliveira ◽  
Carolina Pantuzza Ramos ◽  
Izabela de Assis Rocha ◽  
Sóstenes Apolo Correia Marcelino ◽  
Felipe Pierezan ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Salmonella Typhimurium ◽  
Gastrointestinal Disease ◽  
Post Mortem ◽  
History Of ◽  
The Central Nervous System ◽  
Portal Of Entry ◽  
Brain And Spinal Cord ◽  
The Brain

ABSTRACT: A 10-day old foal presented with a history of acute recumbency and generalized weakness, that progressed to seizure episodes and death. Post mortem examination revealed necrotizing and purulent omphalophlebitis and fibrinopurulent meningoencephalomyelitis. Salmonella Typhimurium was isolated from the central nervous system and determined to be the cause of the meningoencephalomyelitis. Due to the lack of evidence of gastrointestinal disease, the umbilical cord was considered the most likely portal of entry of the bacteria. The isolated S. Typhimurium was resistant to ampicillin and cephalotin, and partially resistant to enrofloxacin. These drugs are commonly used in the treatment of salmonellosis. This is the first report of S. Typhimurium affecting the brain and spinal cord of a foal. Salmonellosis should be considered a differential diagnosis in foals with neurologic signs, even in the absence of enterocolitis.

scholarly journals Acute Myeloid Leukemia Presenting as Acute Bulbar Palsy

2014 ◽  
Vol 13 (4) ◽  
pp. 481-483
Author(s):  
Kiran Kumar Singal ◽  
Ajay Mittal ◽  
Rajni Aggarwal ◽  
Saurav Aggarwal ◽  
Rao Farman Khan ◽  
...  
Keyword(s):  
Brain Stem ◽  
Myeloid Leukemia ◽  
Medical Science ◽  
Granulocytic Sarcoma ◽  
Myeloid Sarcoma ◽  
History Of ◽  
The Central Nervous System ◽  
Immature Myeloid Cells ◽  
Laboratory Investigations ◽  
The Brain

Myeloid Sarcoma1 (also termed as chloroma, granulocytic sarcoma, extra medullary myeloid tumor) is a rare extra medullary tumor composed of immature myeloid cells (myeloblast)2. It is usually associated with leukemia or other myeloproliferative disorder. Myeloid Sarcoma in the central nervous system, around the brain stem is the commonest site of presentation and require high suspicion for diagnosis. We report a forty years male patient with history of dysphagia, dysphonia for last two months. MRI showed chloromas around the brain stem. Laboratory investigations revealed the presence of AML. This is a rare case of Myeloid Sarcoma around the brain stem in a patient of AML. DOI: http://dx.doi.org/10.3329/bjms.v13i4.16056 Bangladesh Journal of Medical Science Vol.13(4) 2014 p.481-483

scholarly journals Human Rights in Islamic Sustainable Development

2020 ◽  
Vol 2 (2) ◽  
pp. 123-131
Author(s):  
Miss Robina ◽  
Altaf Ahmad Shah ◽  
Zafar Abbas
Keyword(s):  
Human Rights ◽  
International Relations ◽  
Human Development ◽  
Research Work ◽  
Human Beings ◽  
Sustainable Human Development ◽  
Global Problems ◽  
History Of ◽  
The Cold War ◽  
Comprehensive Study

Development and Human rights are interconnected and are safeguarded by the Charter of United Nations. So far as the Islamic concept of human rights is concerned Khutba Hajjatul Wada has paramount importance in the history of human rights and sustainable human development and progress. The end of the cold war brings multiple global problems and challenges to international relations and gives birth to new perception regarding human rights in the comity of nations. Religion addresses the matters of human good and especially Islam is the religion of peace and tranquility, which focuses on the success of human beings. In this research work, the researcher has conducted thematic analysis from the existing literature and construed his work with the help of a comprehensive study of all three monotheists religions and their concept of sustainable human development.

scholarly journals MULTIPLICATION AND SPREAD OF THE VIRUS OF ST. LOUIS ENCEPHALITIS IN MICE WITH SPECIAL EMPHASIS ON ITS FATE IN THE ALIMENTARY TRACT

1947 ◽  
Vol 85 (6) ◽  
pp. 647-662 ◽  
Author(s):  
John L. Peck ◽  
Albert B. Sabin
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Intestinal Tract ◽  
Antiviral Agent ◽  
Nerve Fibers ◽  
Human Beings ◽  
Poliomyelitis Virus ◽  
Virus Content ◽  
The Central Nervous System ◽  
The Brain

1. Beginning at 24 hours after intravenous injection of about 10 million intracerebral LD50 of virus there was evidence of simultaneous, progressive multiplication in the brain and intestinal tract. 2. When the virus was introduced directly into the brain or the nasal cavities and mouth, none was found in the intestinal tract until there was general centrifugal spread from the central nervous system during the last stages of the infection at 96 or 120 hours after inoculation when the virus in the entire brain had reached a concentration of about 3 billion LD50. 3. Centrifugal spread began when the virus in the brain reached a concentration of about 400 million LD50 and virus appeared in the pharynx, tongue, and adrenals before it was demonstrable in the intestinal tract, blood, or viscera such as the spleen, liver, and kidneys. 4. Despite the high concentrations of virus which developed in the intestinal tract following intravenous inoculation, it was not demonstrable in the stools, differing in this respect from Theiler's virus in mice and poliomyelitis virus in human beings and monkeys. 5. No antiviral agent was found in the stools, but the urine of normal mice having a pH of 5.6, inactivated large amounts of St. Louis encephalitis virus. 6. There was no evidence of multiplication in the nasal mucosa of mice which succumbed with encephalitis following nasal instillation of the virus, the course of events being comparable in this respect to the behavior of the M.V. poliomyelitis virus in rhesus monkeys. 7. At the terminal stage of infection the virus content per milligram of tissue was as great in the leg muscles as in the sciatic nerves. Since this was also true for the urinary bladder, heart, lungs, and tongue among other tissues, and since the amount in the blood was too negligible to account for it, it would appear that the virus either accumulated in these tissues by diffusion from the nerve fibers, along which it was spreading from the central nervous system, or that it multiplied in some constituent other than the nerve fibers.

MRI Findings in a Rottweiler with Leukoencephalomyelopathy

2013 ◽  
Vol 49 (4) ◽  
pp. 255-261 ◽  
Author(s):  
Joseph S. Eagleson ◽  
Marc Kent ◽  
Simon R. Platt ◽  
Raquel R. Rech ◽  
Elizabeth W. Howerth
Keyword(s):  
Spinal Cord ◽  
Cervical Spinal Cord ◽  
Neurologic Examination ◽  
Mri Findings ◽  
Pyramidal Tracts ◽  
Dorsal Portion ◽  
History Of ◽  
The Central Nervous System ◽  
Mri Characteristics ◽  
The Brain

A 22 mo old male rottweiler presented with a 1 mo progressive history of general proprioceptive ataxia and upper motor neuron tetraparesis. Neurologic examination was consistent with a lesion affecting the first through fifth cervical spinal cord segments. MRI disclosed bilaterally symmetric hyperintensities on T2-weighted (T2W) images in the crus cerebri and pyramidal tracts of the brain and the dorsal portion of the lateral funiculi of the cervical spinal cord. Fifty days after initial presentation, the dog was euthanized due to disease progression. Pathologic examination of the central nervous system (CNS) revealed a bilaterally symmetric chronic leukoencephalomyelopathy (LEM) consistent with previous reports of LEM in rottweilers. To the authors’ knowledge, this is the first report to describe the MRI characteristics of LEM in the rottweiler. The topography of the changes observed with MRI paralleled the pathologic changes, which were widespread loss of myelin, decreased axon numbers, and astroglial proliferation. Consequently, MRI of the CNS of affected rottweilers may aid in establishing a presumptive antemortem diagnosis of LEM.

Sign in / Sign up

Export Citation Format

Share Document