Een zeldzame oorzaak van dementie: de bestralingsencephalopathie

2001 ◽  
Vol 13 (3) ◽  
pp. 76-78
Author(s):  
N. van de Weg ◽  
F.R.J. Verhey ◽  
P J.M. Raedts ◽  
F W. Vreeling

SUMMARYWe describe the clinical history of a 49-year old woman, who demonstrated progressive personality changes more than twenty years after radiation of a pituitary adenoma (prolactinoma), with apathy, loss of initiative, memory deficits, postural instability, dysarthria and faecal incontinence. Neuropsychological assessment showed impulsivity, loss of overview, desinhibition, fluctuating deficits of attention, and memory disturbances. MRI-scanning of the brain revealed a cystic lesion along the right ventricle. The clinical picture and the findings of the other investigations are typical for dementia due to radiation encephalopathy. Such a long period between radiation and cognitive deterioration is rare, although it has been described before.

Author(s):  
Edward Kachur ◽  
Lee-Cyn Ang ◽  
Joseph F. Megyesi

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.


2016 ◽  
Vol 98 (7) ◽  
pp. e141-e142 ◽  
Author(s):  
SH Rossi ◽  
E Coveney

A de Garengeot hernia is defined as an incarcerated femoral hernia containing the vermiform appendix. We describe the case of a patient with a type 4 appendiceal diverticulum within a de Garengeot hernia and delineate valuable learning points.A 76-year-old woman presented with a 2-week history of a non-reducible painless femoral mass. Outpatient ultrasonography demonstrated a 36mm × 20mm smooth walled, multiloculated, partially cystic lesion anterior to the right inguinal ligament in keeping with an incarcerated femoral hernia. Intraoperatively, the appendix was found to be incarcerated in the sac of the femoral hernia and appendicectomy was performed. Histopathology demonstrated no evidence of inflammation in the appendix. However, an incidental appendiceal diverticulum was identified.It is widely recognised that a de Garengeot hernia may present with concomitant appendicitis, secondary to raised intraluminal pressure in the incarcerated appendix. Appendiceal diverticulosis is also believed to develop in response to raised pressure in the appendix and may therefore develop secondary to incarceration in a de Garengeot hernia. To our knowledge, only one such case has been described in the literature. A de Garengeot hernia is a rare entity, which poses significant diagnostic challenges. A high index of clinical suspicion is necessary as these hernias are at particularly high risk of perforation and so prompt surgical management is paramount.


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.


2019 ◽  
Author(s):  
András Folyovich ◽  
Angéla Majoros ◽  
Tamás Jarecsny ◽  
Gitta Pánczél ◽  
Zsuzsanna Pápai ◽  
...  

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.


2012 ◽  
Vol 10 (1) ◽  
pp. 71-74 ◽  
Author(s):  
Sumit Thakar ◽  
Yasha T. Chickabasaviah ◽  
Alangar S. Hegde

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.


2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S60-S60
Author(s):  
R Ramanarasimhaiah ◽  
B Chae ◽  
M Toprak ◽  
L M Opitz ◽  
H Wu

Abstract Introduction/Objective Filiform polyposis (FP), also referred as inflammatory polyposis or pseudopolyposis, is an uncommon benign entity usually diagnosed in patients with history of Crohn’s disease, ulcerative colitis, interstitial tuberculosis or histiocytosis X. It is characterized by multiple finger-like mucosal projections/pseudopolyps seen more commonly in transverse and descending colon. Pathogenesis of FP is uncertain but it is hypothesized to be reactive process to chronic inflammation leading to formation of fingerlike projections. The term filiform polyposis was coined in 1974 by Appleman HD and his collegues to describe numerous long slender worm-like (filiform) colonic lesions seen in radiographs. Herein, we are going to present a case of FP diagnosed in a 20-year-old male with history of medically refractive ulcerative colitis. Methods/Case Report The patient is a 20-year-old male, with a history of medically refractive ulcerative colitis on systemic steroids and vedolizumab. He underwent sigmoidoscopy and colonoscopy which reveals multiple pedunculated, polypoid lesions, ranging from 0.5 to 3 cm in length with white tips (cap of purulent mucous). Patient underwent total abdominal colectomy with end ileostomy. The specimen received by our deparment consist of a segment of right colon (63 cm in length, 3.5 cm in diameter) with attached ileum (3 cm in length, 2 cm in diameter) and appendix (6 cm in length, 0.8 cm in diameter). The part of the right colon is constricted/strictured measuring 24 cm in length, 2 cm in diameter. Opening reveals numerous (>100) pedunculated, finger-like polypoid lesions measuring from 0.3 cm to 2 cm in length. The lesions involve the entire right colon including distal colonic surgical margin. Appendix and ileum are not involved by the lesion and grossly unremarkable. Microscopic evaluation of the lesions reveal acute and chronic inflammatory cells, granulation tissue, areas of necrosis and reactive changes with focal mucosal atrophy consistent with chronic ulcerative colitis. Results (if a Case Study enter NA) NA Conclusion Filiform polyposis (FP) is an uncommon benign entity commonly seen in association with Inflammatory Bowel Diseases. Diagnosing FP requires comprehensive gross and microscopic examination as well as review of clinical history and imaginig studies. Because of its uncommon nature, we recommend to report this entity to raise awareness and to educate the pathologists who have not diagnosed it before.


2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
András Folyovich ◽  
Angéla Majoros ◽  
Tamás Jarecsny ◽  
Gitta Pánczél ◽  
Zsuzsanna Pápai ◽  
...  

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.


2003 ◽  
Vol 33 (1) ◽  
pp. 53-54 ◽  
Author(s):  
Bello B Shehu ◽  
Nasiru J Ismail

A 37-year-old woman, Para 5+0 presented with a 1 year history of recurrent convulsions and progressive weakness of the right side of the body. She had been treated for postpartum eclampsia in her last delivery but symptoms recurred 3 months later. Evaluation including computerized tomography scan of the brain suggested a parieto-temporal meningioma, which was completely excised at craniotomy. Histology confirmed this to be a meningioma. The patient was well at 8 months of follow up. The growth of meningiomas may increase during pregnancy due to presence of receptors for progestational hormones in the tumour and the meningioma may become symptomatic in pregnancy, presenting as eclampsia. Close follow up of patients with eclampsia is necessary to identify neurological features that may lead to a diagnosis of meningioma. Early diagnosis is essential if a good outcome is to be ensured.


2015 ◽  
Vol 53 (8) ◽  
pp. 2674-2685 ◽  
Author(s):  
Conceição M. P. S. de Azevedo ◽  
Renata R. Gomes ◽  
Vania A. Vicente ◽  
Daniel W. C. L. Santos ◽  
Sirlei G. Marques ◽  
...  

We report a fatal case of a chromoblastomycosis-like infection caused by a novel species ofFonsecaeain a 52-year-old immunocompetent Caucasian male from an area of chromoblastomycosis endemicity in Brazil. The patient had a 30-year history of slowly evolving, verrucous lesions on the right upper arm which gradually affected the entire arm, the left hemifacial area, and the nose. Subsequent dissemination to the brain was observed, which led to death of the patient. The internal transcribed spacer (ITS) and partial large subunit (LSU),BT2, andCDC42genes of the isolates recovered from skin and brain were sequenced, confirming the novelty of the species. The species is clinically unique in causing brain abscesses secondary to chromoblastomycosis lesions despite the apparent intact immunity of the patient. Histopathologic appearances were very different, showing muriform cells in skin and hyphae in brain.


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.


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