scholarly journals P.029 Facial onset sensorimotor neuronopathy syndrome (FOSMN) associated with Non-Hodgkin Lymphoma (NHL)

2019 ◽  
Vol 46 (s1) ◽  
pp. S21
Author(s):  
DA Vargas ◽  
S Botez ◽  
R Massie
Keyword(s):  
Clinical Presentation ◽  
Motor Deficits ◽  
Sensory Abnormalities ◽  
Non Hodgkin Lymphoma ◽  
First Case ◽  
Initial Description ◽  
The Right ◽  
Paraneoplastic Disorder ◽  
Work Up ◽  
Slow Onset

Background: FOSMN is a recently describe neurological syndrome, characterizes by slow onset of facial sensory abnormalities and motor deficits. The initial description showed a very uniform clinical presentation. Since the initial description there are clinical cases describe in literature with subtle phenotype variations. Methods: We describe a clinical case associated with NHL. We will report clinical data, laboratory and neurophysiological findings. Results: Patient initiated with left perioral and mental sensory symptoms on her left side. It spread up to include left V2 area and spread to the right side. After 2 years she developed sensory symptoms on her right hand. Progressed to weakness and atrophy on the right upper limb. Also developed dysarthria, dysphonia, dysphagia, as well as photophobia, anisocoria and double vision. Had thorough work-up and everything unrevealing. Except for Spep that showed increased free kappa. Bone marrow biopsy showed evidence of a clonal cell expansion consistent with indolent lymphoma Conclusions: This case provides evidence of FOSMN associated with NHL. To our knowledge this is a first case describe with NHL. There had been reports with motor neuro diseases phenotype with lymphoma that may represent a paraneoplastic disorder. Our patient expands the clinical presentation. This finding should not lessen the diagnosis of FOSMN.

Xanthogranuloma of the Sciatic Nerve: Case Report

Neurosurgery ◽  
2004 ◽  
Vol 55 (6) ◽  
pp. E1453-E1458 ◽  
Author(s):  
Parag G. Patil ◽  
Thomas J. Cummings ◽  
James A. Nunley ◽  
Allan H. Friedman
Keyword(s):  
Nervous System ◽  
Sciatic Nerve ◽  
Peripheral Nervous System ◽  
Groin Pain ◽  
Clinical Presentation ◽  
Resonance Imaging ◽  
Imaging Studies ◽  
Pathological Characteristics ◽  
First Case ◽  
The Right

Abstract OBJECTIVE AND IMPORTANCE: Xanthogranulomas involving the central or peripheral nervous system are extraordinarily rare. None have been reported in the lower extremity. Here, we report and characterize the first case of xanthogranuloma of the sciatic nerve. CLINICAL PRESENTATION: A 58-year-old woman presented with poorly localized right back and groin pain. Magnetic resonance imaging studies revealed an enhancing lesion of the sciatic nerve in the right gluteal region. INTERVENTION: The sciatic nerve xanthogranuloma was resected without complication. CONCLUSION: Our report indicates that xanthogranuloma, although extremely rare, may occur throughout the nervous system. Our evaluation demonstrates that such lesions involving the peripheral nervous system have similar pathological characteristics to xanthogranulomas that more commonly occur in the skin.

scholarly journals Role of early surgical intervention in outcome and prognosis of spontaneous cerebellar hemorrhage with intraventricular extension

2021 ◽  
Vol 3 (01) ◽  
pp. 28-32
Author(s):  
Jemesh Singh Maharjan
Keyword(s):  
Clinical Presentation ◽  
Surgical Intervention ◽  
Sudden Onset ◽  
Cerebellar Hemorrhage ◽  
First Case ◽  
Spontaneous Intracranial Hemorrhage ◽  
Poor Outcomes ◽  
The Right ◽  
Early Surgical Intervention

Spontaneous cerebellar hematomas represent 5 to 13% of all cases of spontaneous intracranial hemorrhage. The main controversy involves deciding which cases require surgical evacuation of the hematoma versus other options, such as ventricular drainage only or conservative treatment. Furthermore, because the clinical course is variable in some cases, timing of such treatment should be carefully considered. The duration from the onset of hemorrhage also plays an important role in prognosis and recovery of the patient. Both the clinical presentation and subsequent course vary among cases. Unpredictable rapid deterioration in consciousness levels has been recognized. The majority of patients with such decline in consciousness experience the deterioration primarily within 72 hrs after onset⁠. Acute presentation was observed to be correlated with poor outcomes. In our report, the first case presented with sudden onset of headache in the right frontal region of head with vertigo. He came to hospital within 6 hours of onset. However, the second case had an onset of symptoms around 72 hours before the presentation.

Cerebellar Malignant Fibrous Histiocytoma: Case Report and Literature Review

Neurosurgery ◽  
2004 ◽  
Vol 54 (3) ◽  
pp. 745-752 ◽  
Author(s):  
Abderrahmane Hamlat ◽  
Mahmoudreza Adn ◽  
Sylvie Caulet-Maugendre ◽  
Yvon Guegan
Keyword(s):  
Malignant Fibrous Histiocytoma ◽  
Clinical Presentation ◽  
Treatment Approach ◽  
Fibrous Histiocytoma ◽  
Cerebellar Hemisphere ◽  
Cerebellar Syndrome ◽  
Computed Tomographic ◽  
First Case ◽  
The Central Nervous System ◽  
The Right

Abstract OBJECTIVE AND IMPORTANCE Malignant fibrous histiocytoma in the central nervous system is uncommon. Fewer than 70 cases have been documented and, to the best of our knowledge, this is the first case arising from the cerebellum. CLINICAL PRESENTATION A 44-year-old woman presented with headaches, vomiting, and dizziness. A neurological examination revealed right cerebellar syndrome. Brain computed tomographic scans revealed an isodense tumor in the right cerebellar hemisphere. The breast ultrasonographic, bone scintigraphic, and thoracoabdominal computed tomographic findings were normal. INTERVENTION The patient was surgically treated. The tumor recurred 1.5 months later, demonstrating hemorrhagic characteristics on brain computed tomographic scans. The patient underwent a second operation, followed by radiotherapy. CONCLUSION Malignant fibrous histiocytoma is still a controversial entity, and the lack of specific criteria means that it must be diagnosed via the process of elimination. With currently available therapy, our review can provide only a very poor prognosis. The median survival time was 27 months. In attempts to develop better therapeutic strategies, total excision and radiotherapy seem to represent the best treatment approach.

Adult-to-Adult Living-Donor Liver Transplantation

Swiss Surgery ◽  
2003 ◽  
Vol 9 (5) ◽  
pp. 227-236 ◽  
Author(s):  
Majno ◽  
Mentha ◽  
Berney ◽  
Bühler ◽  
Giostra ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Living Donor ◽  
Living Donor Liver Transplantation ◽  
Therapeutic Option ◽  
Donor Liver ◽  
Donor Liver Transplantation ◽  
Multidisciplinary Program ◽  
First Case ◽  
Living Donor Transplantation ◽  
The Right

Living donor liver transplantation is a relatively new procedure in which the right side of the liver is harvested in a healthy donor and transplanted into a recipient. After the first case in 1994, over 3000 cases have been done worldwide. This review summarizes the reasons why the procedure is needed, describes its main technical aspects, highlights the boundaries in which it can be done safely, summarizes the current experience worldwide and describes the main points of the program in our unit. We argue that living-donor transplantation is a viable alternative to a long time on the waiting list for several patients, and it can be performed safely and successfully provided that all precautions are undertaken to minimize the risks in the donor and to increase the chances of a good outcome in the recipients. If these prerequisites are met, and within the framework of a structured multidisciplinary program, we believe that living-donor liver transplantation should be funded by health insurances as a recognized therapeutic option.

scholarly journals Primary Gastrointestinal Stromal Tumor of the Liver with Cystic Changes

2019 ◽  
Vol 13 (1) ◽  
pp. 58-65
Author(s):  
Takashi Tashiro ◽  
Fumihiro Uwamori ◽  
Yukiomi Nakade ◽  
Tadahisa Inoue ◽  
Yuji Kobayashi ◽  
...  
Keyword(s):  
Liver Tumors ◽  
Past History ◽  
Brain Infarction ◽  
Arterial Phase ◽  
First Case ◽  
Positron Emission ◽  
Cystic Changes ◽  
Multiple Bone Metastases ◽  
The Right ◽  
Cells Of Cajal

Gastrointestinal stromal tumors (GISTs) are known to originate specifically from the intestinal cells of Cajal located in the gastrointestinal mesenchyme. GISTs developing outside of the digestive tract have barely been reported. We encountered a first case of large primary GISTs in the liver with cystic changes. A 63-year-old man with a past history of brain infarction visited our hospital. The computed tomography (CT) revealed a 6-cm and a 10-cm mass in the right and the caudal lobe of the liver, respectively. These tumors have marginal enhancement in the arterial phase; however, they presented as hypodense in the internal tumor sites. Both liver tumors had cystic changes. Gastrointestinal examinations using endoscopy revealed no other gastrointestinal tumors, and [18F]-fluoro-2-deoxy-D-glucose positron emission tomography/CT revealed multiple bone metastases in addition to the liver tumors. The liver tumor specimens were composed of spindle cells, and the immunohistochemical staining for c-Kit and for DOG1, as discovered on GIST, was positive. The patient was diagnosed with primary hepatic GIST with cystic changes.

scholarly journals Emphysematous pyelonephritis with air bubble in the inferior vena cava

2020 ◽  
Vol 26 (1) ◽  
Author(s):  
Tiffany A. Perkins ◽  
Alberic Rogman ◽  
Murali K. Ankem
Keyword(s):  
Septic Shock ◽  
Inferior Vena Cava ◽  
Inferior Vena ◽  
Vena Cava ◽  
Rare Presentation ◽  
Emphysematous Pyelonephritis ◽  
Case Presentation ◽  
Air Bubble ◽  
First Case ◽  
The Right

Abstract Background Emphysematous pyelonephritis (EPN) with gas in the inferior vena cava (IVC) is a rare presentation and to our knowledge, this is the first case report in the urologic literature. Case presentation A 35-Year-old obese diabetic Hispanic female presented to the emergency room with a clinical picture of septic shock. Prompt computerized tomography scan revealed EPN with gas throughout the right renal parenchyma and extending to the right renal vein, IVC, and pulmonary artery. She died before surgical intervention Conclusion This case demonstrates that patients presenting with severe EPN have a high mortality risk and providers should acknowledge that septic shock, endogenous air emboli, or a combination of both could result in cardiovascular collapse and sudden death.

scholarly journals Stereotactic Body Radiotherapy of a Solitary Metachronous Sphenoid Metastasis from Renal Cell Cancer: A Case Report

2021 ◽  
pp. 269-273
Author(s):  
Charles Marchand Crety ◽  
Estelle Vigneau ◽  
Camille Invernizzi
Keyword(s):  
Renal Cell Cancer ◽  
Renal Cell ◽  
Stereotactic Body Radiation Therapy ◽  
Clinical Presentation ◽  
Histopathological Examination ◽  
Cell Cancer ◽  
Papillary Renal Cell Carcinoma ◽  
Complete Response ◽  
Resonance Imaging ◽  
The Right

Nasosinus metastases from kidney cancer are an unusual clinical presentation although some cases are reported in the literature. Among these cases, sphenoidal metastases are even rarer. Here we report a case of lone sphenoid metastasis in patients with papillary renal cell cancer. Eight months after radical nephrectomy, the patient presented with progressively worsening diplopia. Magnetic resonance imaging showed a mass in the right sphenoid sinus. Histopathological examination of the biopsy sample confirmed diagnosis of sinonasal metastasis from papillary renal cell carcinoma. The patient was declined for surgical management and received stereotactic body radiation therapy. Reassessment MRI at 4 months showed a complete response of the treated sphenoid lesion.

scholarly journals A First Case Report of Orbital Extra-Adrenal Paraganglioma in Cat

2021 ◽  
Vol 8 (5) ◽  
pp. 86
Author(s):  
Leonardo Leonardi ◽  
Raluca Ioana Rizac ◽  
Ilaria Pettinari ◽  
Luca Mechelli ◽  
Carlo De Feo
Keyword(s):  
Parasympathetic Nervous System ◽  
Neuron Specific Enolase ◽  
Final Diagnosis ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Neoplasm ◽  
Glial Fibrillary Acid Protein ◽  
Veterinary Clinic ◽  
First Case ◽  
The Right ◽  
Extra Adrenal Paraganglioma

Paraganglioma is a rare neuroendocrine neoplasm originating from paraganglia and consisting of neuroendocrine cells of the sympathetic and parasympathetic nervous system. Extra-adrenal paraganglioma occurs with a low incidence in both humans and animals. This report presents the first case of paraganglioma in a cat with orbital primary location. An 18-year-old spayed female European domestic shorthair cat of 3.60 kg body weight was evaluated in a private veterinary clinic in Perugia, Italy, for a pronounced exophthalmos of the right eye. The cat underwent surgery for the enucleation of the right eye and of the mass. The biopsy samples of the removed tissue were fixed in 10% buffered neutral formalin for histological and immunohistochemical evaluations. Therefore, specific markers were used for immunohistochemical investigations, such as anti-neuron specific enolase (NSE), anti-synaptophysin, anti-glial fibrillary acid protein, anti-cytokeratin and anti-chromogranin. The results of these investigations allowed establishing the final diagnosis of ocular extra-adrenal paraganglioma of the cat.

scholarly journals RARE-29. PRIMARY CENTRAL NERVOUS SYSTEM NON-HODGKIN LYMPHOMA IN AN 11-YEAR-OLD BOY: A CASE REPORT

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii448-iii448
Author(s):  
Jorge Luis Ramírez-Melo ◽  
Regina M Navarro-Martin del Campo ◽  
Manuel D Martinez-Albarran ◽  
Fernando Sánchez-Zubieta ◽  
Ana L Orozco-Alvarado ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hodgkin Lymphoma ◽  
Central Nervous System Lymphoma ◽  
Complete Response ◽  
High Dose ◽  
Illustrative Case ◽  
Tumor Biopsy ◽  
Non Hodgkin Lymphoma ◽  
The Right

Abstract BACKGROUND Primary central nervous system lymphoma (PCNSL) are very rare in children. CLINICAL CASE: An 11-year-old male presented with a 2 months history with myoclonic movements in the upper right limb, and a sudden frontal headache, gait disturbance due to right hemiparesis and an ipsilateral convulsive episode. Upon admission he had critical condition, with hypertensive skull syndrome, Glasgow of 12, Karnofsky 40%, right hemiparesis, swallowing disorder, facial paralysis, and loss of photo motor reflex and unilateral amaurosis. A CT and MRI showed a huge tumor mass in the left tempo-parietal region, infiltrating the white matter and shifting the midline. A Tumor biopsy was done, and reported diffuse small cell non-Hodgkin lymphoma of high-grade, Burkitt type. Systemic lymphoma workup was negative. He received six cycles of chemotherapy based on high dose methotrexate, rituximab and triple intrathecal.After the second cycle an ophthalmologic evaluation was done, and found infiltration to the right retina, for which 6 cycles of intra vitreous chemotherapy with methotrexate were applied, he showed an excellent response, and recovered all his neurological functions except that right hemianopia persist. Control MRI showed partial response at 2nd cycle and complete response after the 4th cycle. No Radiation was performed. CONCLUSION This report highlights the fact that pediatric PCNSL may be effectively treated by a combination of HDMTX and rituximab-based chemoimmunotherapy without irradiation. Lack of awareness of this rare entity may lead to extense resections of brain, and potential permanent secuelae that were avoided in this illustrative case.

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