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 Intraparenchymal Supratentorial Neurenteric Cyst

2004 ◽  
Vol 31 (3) ◽  
pp. 412-416 ◽  
Author(s):  
Edward Kachur ◽  
Lee-Cyn Ang ◽  
Joseph F. Megyesi
Keyword(s):  
Cystic Lesion ◽  
Brain Parenchyma ◽  
Neurenteric Cyst ◽  
Complete Excision ◽  
First Case ◽  
History Of ◽  
The Central Nervous System ◽  
One Year ◽  
The Right ◽  
The Brain

Background:Neurenteric cysts are congenital cysts of the central nervous system that are believed to be of endodermal origin. In this report we present the unique case of a supratentorial neurenteric cyst that is contained entirely within the brain parenchyma.Methods:Apatient presented with an intraparenchymal cystic lesion that was subsequently identified as a neurenteric cyst. This lesion is reviewed in light of the available literature.Case report:A 35-year-old female presented with a one year history of progressive headaches and seizure-like episodes. Her examination revealed no deficits. Magnetic resonance imaging showed a 4 cm x 4 cm x 4 cm cystic lesion within the parenchyma of the right frontal lobe. A right frontal craniotomy and complete excision of the cystic lesion was performed. Pathologic examination confirmed that it was a neurenteric cyst. Postoperatively the patient's symptoms improved.Conclusion:Review of the literature revealed this to be the first case of a surgically excised, pathologically confirmed supratentorial neurenteric cyst, contained entirely within the brain parenchyma.

scholarly journals Idiopathic Hypertrophic Pachymeningitis Manifesting with Frontal Brain Edema

2001 ◽  
Vol 19 (3) ◽  
pp. 491-495
Author(s):  
Mário Emílio Teixeira Dourado Júnior ◽  
Ricardo Humberto de Miranda Félix ◽  
Marcos Dias Leão
Keyword(s):  
Dura Mater ◽  
Histopathological Study ◽  
Hypertrophic Pachymeningitis ◽  
Falx Cerebri ◽  
Additional Information ◽  
Frontal Brain ◽  
History Of ◽  
Cerebral Parenchyma ◽  
Magnetic Resonance Imaging Mri ◽  
The Brain

Introduction. Hypertrophic pachymeningitis (HP) is a rare inflammatory disease that results in thickening of the dura mater. Atypically, it can progress to include the involvement of the cerebral parenchyma. Method. In this paper, we report the rare case of a 31-year-old man with a three-week history of headaches, seizures, impairments of cognitive function, and changes in behavior and mood. Magnetic resonance imaging (MRI) of the brain showed a thickening and an abnormal enhancement of the dura mater over the falx cerebri with extensions into the adjacent cranial base and with brain edemas in the frontal lobes. Histopathological study of meningeal and brain biopsies showed an inflammatory process that was compatible with HP. The results of an extensive laboratory investigation were unremarkable and did not provide additional information on the cause of the meningeal disease. The patient exhibited relapses despite immunosuppressive therapy. Conclusion. This case shows the challenges associated with the management of the disease and the importance of early diagnosis to avoid worsening of the condition and cerebral damage.

scholarly journals Epileptic Seizure Provoked by Bone Metastasis of Chronic Lymphoid Leukemia and Merkel Cell Carcinoma – Case Report

2019 ◽  
Author(s):  
András Folyovich ◽  
Angéla Majoros ◽  
Tamás Jarecsny ◽  
Gitta Pánczél ◽  
Zsuzsanna Pápai ◽  
...  
Keyword(s):  
Bone Metastases ◽  
Cell Carcinoma ◽  
Merkel Cell Carcinoma ◽  
Cerebral Metastasis ◽  
Merkel Cell ◽  
Lymphoid Leukemia ◽  
Chronic Lymphoid Leukemia ◽  
History Of ◽  
The Right ◽  
The Brain

Abstract Background Merkel cell carcinoma (MCC) is a rare primary neuroendocrine cutaneous tumor, rarely metastatizing to the brain. Chronic lymphoid leukemia (CLL) is a disease predisposing to MCC. According to previous reports, headache and focal neurological deficits suggest disease progression to the brain. We present a patient with MCC whose seizure was not elicited by a cerebral metastasis, but by bone metastases compressing the brain. Case presentation The 62-year-old female patient had a history of CLL. A lesion with the appearance of an atheroma was removed from the right upper arm. Histology confirmed the diagnosis of MCC. She was admitted to the neurology department with her first GM seizure. The cranial MRI/MRA showed bone metastases in the right parietal and left frontal areas, compressing the brain. Flow cytometry of CSF did not reveal metastasis of MCC or CLL. No surgery was performed, chemotherapy was continued. Conclusions The case history of the patient was unique even among the rare cases of MCC with neurological involvement. The seizure was not elicited by a cerebral metastasis, but by bone metastases compressing the brain. In addition to patient history, clinical presentation and radiological findings enabled a suspected diagnosis preceding confirmation by laboratory methods.

Spontaneous resolution of invasive cerebral aspergillosis following partial resection in a medically untreated infant

2012 ◽  
Vol 10 (1) ◽  
pp. 71-74 ◽  
Author(s):  
Sumit Thakar ◽  
Yasha T. Chickabasaviah ◽  
Alangar S. Hegde
Keyword(s):  
Emergency Services ◽  
Mass Effect ◽  
Cerebral Hemiatrophy ◽  
Right Cerebral Hemisphere ◽  
History Of ◽  
Subcortical Regions ◽  
The Right ◽  
Mri Study ◽  
The Brain

Invasive craniocerebral aspergillosis, often encountered in an immunocompromised setting, is almost uniformly fatal despite radical surgical and medical management, and is frequently a necropsy finding. The authors report a unique, self-resolving clinical course of this aggressive infection in a 10-month-old infant. The infant was brought to the emergency services in altered sensorium with a 1-week history of left-sided hemiparesis, excessive irritability, and vomiting. An MRI study of the brain revealed multiple, heterogeneously enhancing lesions in the right cerebral hemisphere with mass effect. The largest lesion in the frontotemporal cortical and subcortical regions was decompressed on an emergent basis. Histopathological findings were suggestive of invasive aspergillosis, although there was no evidence of the infection in the lungs or paranasal sinuses. Computed tomography–guided aspiration of the remaining lesions and follow-up antifungal therapy were recommended. The parents, however, requested discharge without further treatment. The child was seen at a follow-up visit 3 years later without having received any antifungal treatment. Imaging showed resolution of the infection and features of Dyke-Davidoff-Masson syndrome (cerebral hemiatrophy). This report of invasive cerebral aspergillosis resolving without medical therapy is the first of its kind. Its clinicoradiological aspects are discussed in light of previously reported cases.

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 Epileptic Seizure Provoked by Bone Metastasis of Chronic Lymphoid Leukemia and Merkel Cell Carcinoma

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
András Folyovich ◽  
Angéla Majoros ◽  
Tamás Jarecsny ◽  
Gitta Pánczél ◽  
Zsuzsanna Pápai ◽  
...  
Keyword(s):  
Bone Metastases ◽  
Cell Carcinoma ◽  
Merkel Cell Carcinoma ◽  
Cerebral Metastasis ◽  
Merkel Cell ◽  
Lymphoid Leukemia ◽  
Chronic Lymphoid Leukemia ◽  
History Of ◽  
The Right ◽  
The Brain

Background. Merkel cell carcinoma (MCC) is a rare primary neuroendocrine cutaneous tumor, rarely metastasizing to the brain. Chronic lymphoid leukemia (CLL) is a disease predisposing to MCC. According to previous reports, headache and focal neurological deficits suggest disease progression to the brain. We present a patient with MCC whose seizure was not elicited by a cerebral metastasis, but by bone metastases compressing the brain. Case Presentation. A 62-year-old female patient had a history of CLL. A lesion with the appearance of an atheroma was removed from the right upper arm. Histology confirmed the diagnosis of MCC. She was admitted to the neurology department with her first GM seizure. The cranial MRI/MRA showed bone metastases in the right parietal and both frontal areas, compressing the brain. Flow cytometry of CSF did not reveal metastasis of MCC. Conclusions. The case history of the patient was unique even among the rare cases of MCC with neurological involvement. The seizure was not elicited by a cerebral metastasis, but by bone metastases compressing the brain. In addition to patient history, clinical presentation and radiological findings enabled a suspected diagnosis of skull metastasis of MCC compressing the brain, causing symptomatic epileptic seizures.

Lateral Sacral Artery Aneurysm: Case Report and Review of the Literature

Neurosurgery ◽  
2005 ◽  
Vol 57 (1) ◽  
pp. E197-E197 ◽  
Author(s):  
Michaël Bruneau ◽  
Pierre Goffette ◽  
Guy Cosnard ◽  
Denis Rommel ◽  
Christian Raftopoulos
Keyword(s):  
Renal Transplantation ◽  
Medical History ◽  
Iliac Artery ◽  
Cauda Equina ◽  
Artery Aneurysm ◽  
Treatment Modalities ◽  
Spinal Epidural Hematoma ◽  
Mri Scan ◽  
History Of ◽  
The Right

Abstract OBJECTIVE AND IMPORTANCE: We report the third case of an aneurysm of the lateral sacral artery (AnLSA). In all cases, because of an incorrect preoperative diagnosis, the surgeons were confronted with severe and unexpected hemorrhaging, and surgery was aborted without effective treatment. Our purpose is to present the preoperative features of AnLSA and its treatment modalities. CLINICAL PRESENTATION: A 54-year-old man had a medical history of renal transplantation on his left external iliac artery. He complained of acute lumbar pain associated with cauda equina syndrome, which resolved within a few hours. At that time, a magnetic resonance imaging (MRI) scan revealed an intracanal hematoma extending from S1 to T12. Six weeks later, a second MRI scan demonstrated an oval-shaped intracanal mass behind the vertebral body of S1 with intense gadolinium enhancement. INTERVENTION: An anterior epidural mass was found. An incision into this mass resulted in significant arterial hemorrhaging. Transparietal embolization with a cotton compress and closure of the aneurysm wall were performed. The postoperative clinical status was stable, and a delayed angiographic study suggested a diagnosis of aneurysm of the right LSA, a branch of the internal iliac artery. Its pathophysiology was explained by the development of a high-flow transpelvic shunt from the right iliac artery territory to the left, to maintain the renal graft blood flow that had initially been reduced by stenosis of the left common iliac artery. Six weeks later, a new MRI scan demonstrated that the AnLSA had increased in size. The lesion was then excluded endovascularly by injection of glue. CONCLUSION: A medical history of renal transplantation with MRI scans showing an anterior epidural mass behind S1 or a spontaneous spinal epidural hematoma are features that must evoke a diagnosis of AnLSA. Treatment is mandatory and is best achieved by embolization. Surgery based on angiographic findings is indicated if the lesion is responsible for a compressive hematoma.

scholarly journals Rosai-Dorfman disease of the colon presented as small solitary polypoid lesion

Rare Tumors ◽  
2010 ◽  
Vol 2 (1) ◽  
pp. 5-7 ◽  
Author(s):  
Munenori Ide ◽  
Takayuki Asao ◽  
Takatomo Yoshida ◽  
Junko Hirato ◽  
Tatsuo Shimura ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Gastrointestinal Tract ◽  
Microscopic Study ◽  
Polypoid Lesion ◽  
Sinus Histiocytosis ◽  
Whole Body ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
History Of ◽  
Submucosal Mass

Rosai-Dorfman disease (RDD) was formerly known as “sinus histiocytosis with massive lymphadenopathy”, and cases involving the gastrointestinal tract are rare. We present a case of pure extranodal RDD, resected as a polypoid lesion in colonoscopic study. The patient was a 62-year old woman with a history of sigmoidectomy for unexplained peritonitis. Microscopic study of the polypoid lesion showed the submucosal mass with histological and immunological features of RDD. The whole body computed tomography revealed neither lymphadenopathy nor tumor-like mass.

Sign in / Sign up

Export Citation Format

Share Document