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 ◽  
...  

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

Author(s):  
A Ghare ◽  
K Langdon ◽  
A Andrade ◽  
R Kiwan ◽  
A Ranger ◽  
...  

Background: Myeloid sarcoma (MS) is a rare solid tumour made of myeloblasts or immature myeloid cells in an extramedullary site or in bone, associated with systemic hematologic neoplasms. When they occur in the brain parenchyma, they can often be misdiagnosed. Methods: The authors report a case of a 4-year old boy 6 months out of remission from AML, presenting with a short history of headaches and vomiting, and found to have a heterogenous contrast-enhancing lesion in the right cerebellar hemisphere, with differential diagnosis of myeloid sarcoma, astrocytoma, medulloblastoma and ATRT. Preliminary diagnosis was made flow cytometry from an intraoperative biopsy. The patient had a long course of chemotherapy and radiation, but eventually died from the systemic burden of his AML. Results: The authors present a literature review on 178 published cases of CNS myeloid sarcomas, and their radiological presentation and the basis of immunohistochemical and pathological diagnosis is discussed. Conclusions: Diagnosis rests on a combination of immunohistochemistry and histopathology of biopsied tissue. Surgical resection is controversial, especially given the efficacy of chemotherapy and radiation, and prognosis remains unclear. As with all uncommon and rare clinical entities, further investigation is warranted to determine prognosis and optimal management of CNS myeloid sarcomas.


Author(s):  
Nupur Kaushik ◽  
Lalit Kumar ◽  
Pooja Agarwal ◽  
Harendra Kumar ◽  
Brijesh Sharma

Myeloid sarcoma, also known as chloroma, granulocytic sarcoma, extramedullary Acute Myeloid Leukaemia (AML), myeloblastoma or extramedullary myeloid tumour, is a rare manifestation, characterised by the proliferation of immature myeloid cells, myeloblasts or monoblasts occurring as one or more tumour at an extramedullary site. It is associated with disruption of normal architecture of tissue in which it is found. Monoblastic sarcoma is a rare variant of myeloid sarcoma. Hereby, the authors report a rare case of primary monoblastic sarcoma in a 64-year-old male patient presented with complaint of swelling over right side lower cervical region. He had no history of AML. On physical examination, the swelling was over medial one-third of right clavicle measuring 6×5.8×3 cm. The swelling was excised and sent for histopathological examination, which was diagnosed as monoblastic sarcoma, and confirmed on immunohistochemistry.


2019 ◽  
Vol 16 (1) ◽  
Author(s):  
Włodzisław Duch ◽  
Dariusz Mikołajewski

Abstract Despite great progress in understanding the functions and structures of the central nervous system (CNS) the brain stem remains one of the least understood systems. We know that the brain stem acts as a decision station preparing the organism to act in a specific way, but such functions are rather difficult to model with sufficient precision to replicate experimental data due to the scarcity of data and complexity of large-scale simulations of brain stem structures. The approach proposed in this article retains some ideas of previous models, and provides more precise computational realization that enables qualitative interpretation of the functions played by different network states. Simulations are aimed primarily at the investigation of general switching mechanisms which may be executed in brain stem neural networks, as far as studying how the aforementioned mechanisms depend on basic neural network features: basic ionic channels, accommodation, and the influence of noise.


2020 ◽  
Vol 11 (4) ◽  
pp. 5187-5191
Author(s):  
Sivapriya G Nair ◽  
Jina Raj ◽  
Sajesh K Menon ◽  
Suhas Udayakumaran ◽  
Roshni P R

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.


2020 ◽  
Vol 19 (1-2) ◽  
pp. 55-61
Author(s):  
Andrej Nikolovski ◽  
Dragoslav Mladenovikj ◽  
Aleksandra Veljanovska ◽  
Gordana Petrusevka

Myeloid sarcoma (extramedullary myeloblastoma, granulocytic sarcoma, chloroma) is an extramedullary isolated malignant tumor of myeloblasts and immature myelocytes. It can occur anywhere in the body as a solitary tumor or can be accompanied with acute myeloid leukemia. We are presenting a case of a young male patient that presented with sings of a small bowel obstruction and a palpable tumor mass in the abdomen. After uneventful postoperative period, the immunohistochemistry analysis reported an extramedullary myeloid sarcoma since a normal bone marrow biopsy was revealed.


PEDIATRICS ◽  
1959 ◽  
Vol 24 (1) ◽  
pp. 73-73

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.


PEDIATRICS ◽  
1957 ◽  
Vol 19 (5) ◽  
pp. 949-957
Author(s):  
William A. Hawke ◽  
John S. Prichard

THE SEMINAR was conducted in four 3-hour sessions and aimed to cover the more important features of pediatric neurology. DEVELOPMENT Dr. Hawke reviewed the normal development of the central nervous system in the infant and child which is so important in the assessment of neurologic disorders in this age group. It was noted that the nervous system was particularly immature and changing rapidly in the first 2 years of life. Development was related to myelination and it was emphasized that this was not a steady process but a pattern of sequences of rapid and slow growth. Motor and sensory development appeared to develop from above and to proceed downward, so that eye-control develops before hand- and legcontrol. Development was related to three functioning levels of the central nervous system—the brain stem, the archipallium, and the neopallium. It was observed that the newborn baby functioned at the brain stem level, and to illustrate this an example was given of the hydranencephalic baby which behaves perfectly normally for the first few weeks of life. The anchipallium, which includes part of the temporal lobe, the cingulate gyrus and basal ganglia, supervenes on the brain stem and may be considered responsible for the basic emotions and some primitive motor and sensory control. The neopallium, which includes most of the cerebral hemisphere, becomes dominant in primates. Its function is intellectual rather than emotional and is responsible for skills, discrimination and fine movements. The clinical application of these developmental patterns are innumerable but illustrations were given of changes in physical signs in static brain lesions.


2019 ◽  
pp. 12-31
Author(s):  
Alan J. McComas

This chapter outlines the history of research meetings dealing with consciousness, beginning with that hosted by Herbert Jasper in the Laurentian mountains of Quebec in 1953. It starts, however, with a brief discussion on ancient scientific approaches to medicine, which was jump-started by the Greek physician, Hippocrates. Afterward, the chapter skips forward two millennia to major figures who made breakthroughs in the field of brain science. It also touches on a central debate that reached its climax a little later, as to which part of the brain was responsible for consciousness. The chapter considers whether it was the cerebral cortex, as had been the prevailing assumption, or if it was the brain stem.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Bahaa Razem ◽  
Mohamed Raiteb ◽  
Sanaa El Mrini ◽  
Faiçal Slimani

Abstract Background Myeloid sarcoma is a solid tumor that consists of immature myeloid cells occurring at an extramedullary site. It can present before, concurrent with, or after the diagnosis of acute myeloid leukemia or other myeloproliferative diseases, and a proportion of patients never develop bone marrow infiltration. Only a few isolated cases of pediatric orbital myeloid sarcoma have been reported, and they are often associated with a high misdiagnosis rate. Case report We report a rare case of pediatric orbital myeloid sarcoma associated with blunt trauma in a 3-year-old Caucasian male patient, which was clinically and radiologically misdiagnosed for orbital subperiostal hematoma. The patient underwent a surgical intervention to drain the hematoma when an orbital mass was found. The microscopic, immunologic, and genetic features of the tumor and the myelogram were in favor of LAM2, and the patient was started with chemotherapy with a favorable evolution within 18 months follow-up. Conclusion Orbital myeloid sarcoma usually exhibits clinical and radiological features that can be easily misleading, especially if it happens de novo or as the first manifestation of acute myeloid leukemia. Only a few isolated cases have reported and proposed trauma as a trigger event of the onset of this type of tumor proliferation, but further investigations and evidence are needed to support this hypothesis.


1980 ◽  
Vol 17 (5) ◽  
pp. 544-552 ◽  
Author(s):  
W. D. Sheffield ◽  
O. Narayan ◽  
J. D. Strandberg ◽  
R. J. Adams

A visna-maedi-like disease was found in a Corriedale sheep from which a retrovirus sharing the group antigen of visna-progressive pneumonia virus was isolated from lung, brain, and spleen. Clinically, the sheep had acute neurologic signs and dyspnea. Pathologic examination showed lesions similar to both visna and maedi. In the lung, there was a patchy interstitial pneumonia with marked lymphoid hyperplasia. Changes in the central nervous system were necrotizing nonsuppurative encephalitis of the brain stem, poliomyelitis of the cervical cord, and ependymitis and subependymal gliosis of the ventricles. Histologically, the central nervous system lesions seemed to have arisen sequentially, perhaps in response to bursts of virus replication as the agent underwent possible antigenic mutation. The severe lesions in both the central nervous system and lungs suggested a virus strain with dual tropism.


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