bluuebunny/crossref_metadata_2025_split huggingface dataset:
Resistance to IL-10 inhibition of interferon gamma production and expression of suppressor of cytokine signaling 1 in CD4+T cells from patients with rheumatoid arthritis
Authors: Yamana Jiro , Yamamura Masahiro , Okamoto Akira , Aita Tetsushi , Iwahashi Mitsuhiro , Sunahori Katsue , Makino Hirofumi 
Published: October 2004

AbstractIL-10 has been shown to block the antigen-specific T-cell cytokine response by inhibiting the CD28 signaling pathway. We found that peripheral blood CD4+ T cells from patients with active rheumatoid arthritis (RA) were able to produce greater amounts of interferon gamma after CD3 and CD28 costimulation in the presence of 1 ng/ml IL-10 than were normal control CD4+ T cells, although their surface expression of the type 1 IL-10 receptor was increased. The phosphorylation of signal transducer and activator of transcription 3 was sustained in both blood and synovial tissue CD4+ T cells of RA, but it was not augmented by the presence of 1 ng/ml IL-10. Sera from RA patients induced signal transducer and activator of transcription 3 phosphorylation in normal CD4+ T cells, which was mostly abolished by neutralizing anti-IL-6 antibody. Preincubation of normal CD4+ T cells with IL-6 reduced IL-10-mediated inhibition of interferon gamma production. Blood CD4+ T cells from RA patients contained higher levels of suppressor of cytokine signaling 1 but lower levels of suppressor of cytokine signaling 3 mRNA compared with control CD4+ T cells, as determined by real-time PCR. These results indicate that RA CD4+ T cells become resistant to the immunosuppressive effect of IL-10 before migration into synovial tissue, and this impaired IL-10 signaling may be associated with sustained signal transducer and activator of transcription 3 activation and suppressor of cytokine signaling 1 induction.
Lower Incidence of Colorectal Cancer and Later Age of Disease Onset in 27 Families With Pathogenic MSH6 Germline Mutations Compared With Families With MLH1 or MSH2 Mutations: The German Hereditary Nonpolyposis Colorectal Cancer Consortium
Authors: Plaschke Jens , Engel Christoph , Krüger Stefan , Holinski-Feder Elke , Pagenstecher Constanze , Mangold Elisabeth , Moeslein Gabriela , Schulmann Karsten , Gebert Johannes , von Knebel Doeberitz Magnus , Rüschoff Josef , Loeffler Markus , Schackert Hans K. 
Published: October 2004

Purpose The aim of the study was the analysis of the involvement and phenotypic manifestations of MSH6 germline mutations in families suspected of hereditary nonpolyposis colorectal cancer (HNPCC). Patients and Methods Patients were preselected among 706 families by microsatellite instability, immunohistochemistry, and/or exclusion of MLH1 or MSH2 mutations and were subjected to MSH6 mutation analysis. Clinical and molecular data of MSH6 mutation families were compared with data from families with MLH1 and MSH2 mutations. Results We identified 27 families with 24 different pathogenic MSH6 germline mutations, representing 3.8% of the total of the families, and 14.7% of all families with DNA mismatch repair (MMR) gene mutations (n = 183). The median age of onset of colorectal cancer in putative mutation carriers was 10 years higher for MSH6 (54 years; 95% CI, 51 to 56) compared with MLH1 and MSH2 (44 years; 95% CI, 43 to 45; log-rank test, P = .0038). Relative to other malignant tumors, colorectal cancer was less frequent in MSH6 families compared with MLH1 and MSH2 families (Fisher's exact test, P < .001). In contrast, the frequency of non–HNPCC-associated tumors was increased (Fisher's exact test, P < .001). Conclusion Later age of disease onset and lower incidence of colorectal cancer may contribute to a lower proportion of identified MSH6 mutations in families suspected of HNPCC. However, in approximately half of these families, at least one patient developed colorectal or endometrial cancer in the fourth decade of life. Therefore, a surveillance program as stringent as that for families with MLH1 or MSH2 mutations is recommended.
Giant Infiltrative Cavernous Malformation: Clinical Presentation, Intervention, and Genetic Analysis: Case Report
Authors: Lawton Michael T. , Vates G Edward , Quiñones-Hinojosa Alfredo , McDonald William C. , Marchuk Douglas A. , Young William L. 
Published: October 2004

Abstract

OBJECTIVE AND IMPORTANCE:
Cavernous malformations can present in children with a sporadic course of repeated hemorrhage and enlargement, but they are rarely aggressive, infiltrative, or multilobar. We present the case of a young boy with a complex cavernous malformation that evolved during the course of a decade to encompass the majority of his right cerebral hemisphere.

CLINICAL PRESENTATION:
A 16-month-old boy presented with seizures, and radiographic studies demonstrated a large cavernous malformation in his right frontal pole. During the next 10 years, his seizures became intractable, and he developed progressive left hand weakness and atrophy. His malformation infiltrated his entire right frontal lobe as well as portions of his right parietal lobe, temporal lobe, and deep gray matter structures.

INTERVENTION:
The patient underwent right hemicraniotomy and near total resection of the lesion. Pathological analysis revealed dilated, thin-walled vessels separated by small amounts of intervening astrogliotic brain consistent with cavernous malformation. The patient recovered to his baseline neurological condition and has had no seizure or hemorrhage since his operation. Genetic testing did not reveal mutations in either the CCM1 (KRIT1) or CCM2 (malcavernin) genes.

CONCLUSION:
This case may represent an atypical variant of cavernous malformation best termed giant infiltrative cavernous malformation. Despite its unusual size, multilobar location, and aggressive infiltration, it can be managed effectively with standard surgical resection.
Intraparenchymal Myxopapillary Ependymoma: Case Report
Authors: Tzerakis Nikolaos , Georgakoulias Nikolaos , Kontogeorgos George , Mitsos Aristotelis , Jenkins Alistair , Orphanidis George 
Published: October 2004

Abstract

OBJECTIVE AND IMPORTANCE:
Myxopapillary ependymoma is a histological variant of ependymoma found in the cauda equina region. The most characteristic histological feature of myxopapillary tumors is the abundance of intercellular and perivascular mucin and the arborizing vasculature, which tends to form papillae. Primary intracerebral myxopapillary ependymomas are extremely rare; only three cases have been reported in the previous literature.

CLINICAL PRESENTATION:
A 68-year-old man presented with disorientation and dizziness caused by a cystic left frontal intraparenchymal lesion. This proved to be a myxopapillary ependymoma. Similarities to previously reported cases are discussed, as are the findings on magnetic resonance imaging. There is also a literature review of the histological findings, natural history, and outcome of surgically treated myxopapillary ependymoma.

INTERVENTION:
The lesion was totally removed. After surgery, the patient was neurologically intact and had an uneventful recovery.

CONCLUSION:
This is the fourth reported case of histologically proven primary myxopapillary intracranial ependymoma.
Aneurysmal Bone Cyst of the Atlas: Successful Treatment through Selective Arterial Embolization: Case Report
Authors: Mohit A Alex , Eskridge Joseph , Ellenbogen Richard , Shaffrey Christopher I. 
Published: October 2004

Abstract

OBJECTIVE AND IMPORTANCE:
Aneurysmal bone cysts (ABCs) are benign and expansile osteolytic lesions that can occur in any location in the spine, including the craniovertebral junction. Aggressive resection followed by bone grafting has been the mainstay of treatment, with selective arterial embolization as a presurgical adjunct. Complete excision of these lesions at the craniovertebral junction is associated with high surgical morbidity. We report a case of successful treatment of an ABC of the atlas in a child with selective arterial embolization alone.

CLINICAL PRESENTATION:
A 10-year-old girl presented with persistent neck pain after a snowboarding accident. Computed tomography and magnetic resonance imaging of the cervical spine revealed an expansile cystic mass involving the right lateral mass of C1. Digital subtraction angiography revealed a tumor blush, which, along with the cystic appearance of the lesion, was consistent with an ABC.

INTERVENTION:
The arterial feeders to the lesion were selectively embolized with polyvinyl alcohol particles. Three sessions of embolization were required to eradicate the blood supply to the lesion completely.

CONCLUSION:
Complete surgical resection of ABCs at the craniovertebral junction can be associated with high morbidity secondary to the highly vascular and destructive nature of these lesions. The case discussed here demonstrates the viability of selective arterial embolization as a primary and stand-alone modality of treatment.
Spinal Arachnoid Cyst Containing Nevus Cells in a Patient with a Large Congenital Melanocytic Nevus: Case Report
Authors: Morioka Motohiro , Hamada Jun-ichiro , Ohmori Yuki , Kageshita Toshiro , Ushio Yukitaka 
Published: October 2004

Abstract

OBJECTIVE AND IMPORTANCE:
Spinal arachnoid cysts are rare, and their cause and pathogenesis remain controversial. We experienced a rare case with a large congenital melanocytic nevus in which a spinal arachnoid cyst contained nevus cells, suggesting the congenital nature of a spinal arachnoid cyst.

CLINICAL PRESENTATION:
A 37-year-old Japanese man had been born with a large melanocytic nevus on his back. He experienced intermittent pain radiating to both thighs and to the lower back and waist. A magnetic resonance imaging study disclosed the presence of a posterior intradural extramedullary arachnoid cyst extending from T10 through T12. His spinal cord was displaced anteriorly and flattened.

INTERVENTION:
An osteoplastic laminoplasty was performed, and the arachnoid cyst was totally removed. The cyst membrane exhibited many foci of brown deposits, and histological examination disclosed the presence of melanin-containing cells in the cyst membrane. Morphologically and immunohistochemically, the melanin-containing cells in the cyst membrane were similar to nevus cells in the dermis.

CONCLUSION:
The histological findings of our case suggest that the patient's spinal arachnoid cyst was formed at the same stage of development as the melanocytic nevus.
Multiple Successful Revisions of a Ventriculoureteral Shunt without Nephrectomy for the Treatment of Hydrocephalus: Case Report
Authors: Ohaegbulam Chima , Peters Craig , Goumnerova Liliana 
Published: October 2004

Abstract

OBJECTIVE:
Ventriculoureteral shunts have been described occasionally for patients with complicated hydrocephalus. Repeated successful revision of the distal end of such a shunt has not been reported previously. We report a case in which this was achieved.

CLINICAL PRESENTATION:
A 21-year-old woman with long-standing hydrocephalus, numerous prior shunt revisions and failures, and several distal shunt sites was shunted successfully into her right ureter.

INTERVENTION:
On two occasions, proximal shunt infections required shunt externalization, and the distal end was reinserted successfully into the same distal ureter both times. The patient recovered well from surgery with no clinical evidence of shunt malfunction.

CONCLUSION:
Multiple distal target failures are an unusual but difficult problem in patients with hydrocephalus. Ventriculoureteral shunts without nephrectomy should be considered, and they can be reused successfully if necessary.
Transient Magnetic Resonance Imaging Signal Alterations in the Brainstem after Microvascular Decompression for Trigeminal Neuralgia: Case Report
Authors: Ali M Jafer , Gebarski Stephen , Thompson B Gregory 
Published: October 2004

Abstract

OBJECTIVE AND IMPORTANCE:
We present a case of transient signal alterations along the brainstem path of the trigeminal nerve after microvascular decompression in a patient with trigeminal neuralgia. Such signal changes have been previously reported to represent infarction, but in our patient, they seemed to represent reactive neural and nuclear changes attributable to operative manipulation.

CLINICAL PRESENTATION:
A 43-year-old woman presented with gradually increasing, medically refractory recurrent trigeminal neuralgia 16 months after initial successful microvascular decompression for trigeminal neuralgia. Repeat microvascular decompression resulted initially in complete pain relief; however, her pain suddenly returned on the 3rd postoperative day. Magnetic resonance imaging (MRI) of this recurrent pain revealed new signal alterations along the brainstem path of the trigeminal nerve. Other than marked reduction in her trigeminal neuralgia, the patient had no postoperative alterations of trigeminal sensory or motor function.

INTERVENTION:
The patient's residual postoperative trigeminal neuralgia was successfully treated with carbamazepine. Because of the absence of worrisome signs and symptoms corresponding to the MRI signal alterations, no additional therapy was elected. Follow-up MRI in 1 month revealed near-complete resolution of the postoperative signal alterations along the brainstem path of the trigeminal nerve.

CONCLUSION:
MRI signal alterations along the brainstem path of the trigeminal nerve after microvascular decompression may be only reactive and do not always represent infarction. Depending on the clinical picture, observation may be all that is necessary in such cases.
Resolution of Intracranial Hypertension after Elevation of Depressed Cranial Fracture over the Superior Sagittal Sinus:Case Report
Authors: Binder Devin K. , Sarkissian Vahé , Schmidt Meic H. , Pitts Lawrence H. 
Published: October 2004

Abstract

OBJECTIVE AND IMPORTANCE:
It is common neurosurgical wisdom that depressed cranial fractures (DCFs) over the superior sagittal sinus (SSS) should not be elevated because of the risk of fatal venous hemorrhage.

CLINICAL PRESENTATION:
A 34-year-old man presented with severe headache and diplopia after a motor vehicle accident. Clinical examination demonstrated severe papilledema and bilateral abducens palsy. Imaging findings demonstrated a DCF over the posterior third of the SSS and absent flow distal to the fracture with dilated cortical venous drainage.

INTERVENTION:
Conservative treatment with acetazolamide only partially alleviated the patient's headache and diplopia. Definitive surgical treatment via elevation of the DCF was discussed and decided upon. Twelve days after injury, the patient underwent midline parieto-occipital craniotomy with successful elevation of the DCF off the posterior third of the SSS. Postoperative magnetic resonance venograms revealed restoration of patency in the SSS with reduced tortuosity of cortical veins. The patient's headache resolved, and his papilledema and diplopia resolved gradually.

CONCLUSION:
Elevation of DCF over the SSS can be attempted in cases in which favorable bone anatomy and the patient's clinical condition warrant. This may result in rapid and dramatic resolution of signs and symptoms of secondary intracranial hypertension.
Endoscopic Endonasal Transsphenoidal Surgery
Authors: Cappabianca Paolo , Cavallo Luigi Maria , de Divitiis Enrico 
Published: October 2004

Abstract
THE ENDOSCOPIC ENDONASAL TRANSSPHENOIDAL approach is a minimally invasive surgical technique for the removal of sellar and parasellar lesions. The procedure is performed via an anterior sphenoidotomy. The two main characteristics of the endoscopic approach, when compared with the standard microsurgical operation, arise from the use of the endoscope as a unique optical device and from the absence of a transsphenoidal retractor. More convenient straight surgical instruments are employed, whereas bayonet-shaped tools are used in the microsurgical procedure, to avoid any interference with the light beam generated by the microscope. The standard surgical technique is composed of three main time phases: the nasal, sphenoid, and sellar phase. During the nasal phase, the scope is introduced through the chosen nostril and advanced up to the sphenoethmoid recess, where the sphenoidotomy is performed. The sphenoid phase consists of the detachment of the nasal septum from the sphenoid rostrum, the anterior sphenoidotomy, removal of the sphenoid septum or septa, and identification of the landmarks inside the sphenoid sinus. In the sellar phase, an opening of the sellar floor is performed for removal of the lesion. A wide view of the sellar environment is obtained through angled scopes to detect eventual tumor remnants. The procedure ends with the reconstruction of the sella and removal of the endoscope from the nostril, without any postoperative nasal packing.
Functional Magnetic Resonance Imaging-integrated Neuronavigation: Correlation between Lesion-to-Motor Cortex Distance and Outcome
Authors: Krishnan René , Raabe Andreas , Hattingen Elke , Szelényi Andrea , Yahya Hilal , Hermann Elvis , Zimmermann Michael , Seifert Volker 
Published: October 2004

Abstract

OBJECTIVE:
The integration of functional magnetic resonance imaging (fMRI) data into neuronavigation is a new concept for surgery adjacent to the motor cortex. However, the clinical value remains to be defined. In this study, we investigated the correlation between the lesion-to-fMRI activation distance and the occurrence of a new postoperative deficit.

METHODS:
fMRI-integrated “functional” neuronavigation was used for surgery around the motor strip in 54 patients. During standardized paradigms for hand, foot, and tongue movements, echo-planar imaging T2* blood oxygen level-dependent sequences were acquired and processed with BrainVoyager 2000 software (Brain Innovation, Maastricht, The Netherlands). Neuronavigation was performed with the VectorVision2 system (BrainLAB, Heimstetten, Germany). For outcome analysis, patient age, histological findings, size of lesion, distance to the fMRI areas, preoperative and postoperative Karnofsky index, postoperative motor deficit, and type of resection were analyzed.

RESULTS:
In 45 patients, a gross total resection (&gt;95%) was performed, and for 9 lesions (low-grade glioma, 4; glioblastoma, 5), a subtotal resection (80–95%) was achieved. The neurological outcome improved in 16 patients (29.6%), was unchanged in 29 patients (53.7%), and deteriorated in 9 patients (16.7%). Significant predictors of a new neurological deficit were a lesion-to-activation distance of less than 5 mm (P &lt; 0.01) and incomplete resection (P &lt; 0.05).

CONCLUSION:
fMRI-integrated neuronavigation is a useful concept to assess the risk of a new motor deficit after surgery. Our data suggest that a lesion-to-activation distance of less than 5 mm is associated with a higher risk of neurological deterioration. Within a 10-mm range, cortical stimulation should be performed. For a lesion-to-activation distance of more than 10 mm, a complete resection can be achieved safely. The visualization of fiber tracks is desirable to complete the representation of the motor system.
Feasibility of Radiosurgery for Malignant Brain Tumors in Infants by Use of Image-guided Robotic Radiosurgery: Preliminary Report
Authors: Giller Cole A. , Berger Brian D. , Gilio Joseph P. , Delp Janice L. , Gall Kenneth P. , Weprin Bradley , Bowers Daniel 
Published: October 2004

AbstractOBJECTIVE:The benefits of radiation therapy are generally denied to infants with malignant brain tumors because of the risk of devastating cognitive decline. Efforts to limit this morbidity with radiosurgical techniques have not been feasible for infants because of the dual requirements of rigid head fixation and high precision. We report the radiosurgical treatment of five infants by use of a robotically controlled system without rigid head fixation.METHODS:Five infants with malignant brain tumors received radiosurgical treatment with a robotically driven linear accelerator. Immobilization was aided by general anesthesia, form-fitting head supports, face masks, and body molds. The average marginal dose was 17 ± 2 Gy, and the average treatment volume was 18 ± 22 ml.RESULTS:X-rays obtained during treatment revealed acceptable agreement with preoperative computed tomographic scans in all patients. In one patient, the lesion did not progress, but a distant recurrence occurred 15 months after radiosurgery and also was treated with radiosurgery. In another patient, tumor in the treated region did not progress, but recurrence elsewhere led to death 7 months after treatment. Tumor enlargement occurred in Patient 3 at 3 months posttreatment, leading to death 2 months later. Tumor size was smaller in the remaining two patients at 9 and 11 months after treatment. There has been no toxicity attributed to treatment.CONCLUSION:Radiosurgery with minimal toxicity can be delivered to infants by use of a robotically controlled system that does not require rigid fixation. A formal dose-escalation trial is under way to address dose and toxicity for infants more thoroughly.
Thoracic Outlet Syndrome
Authors: Huang Jason H. , Zager Eric L. 
Published: October 2004

Abstract

OBJECTIVE:
Thoracic outlet syndrome (TOS) is one of the most controversial clinical entities in medicine. We provide a review of this difficult-to-treat disorder, including a brief overview, clinical presentations, surgical anatomy, treatment options, and outcomes.

METHODS:
TOS represents a spectrum of disorders encompassing three related syndromes: compression of the brachial plexus (neurogenic TOS), compression of the subclavian artery or vein (vascular TOS), and the nonspecific or disputed type of TOS. Neurovascular compression may be observed most commonly in the interscalene triangle, but it also has been described in the costoclavicular space and in the subcoracoid space. Patients present with symptoms and signs of arterial insufficiency, venous obstruction, painless wasting of intrinsic hand muscles, paresthesia, and pain. A careful and detailed medical history and physical examination are the most important diagnostic tools for proper identification of TOS. Electromyography, nerve conduction studies, and imaging of the cervical spine and the chest also can provide helpful information regarding diagnosis. Clinical management usually starts with conservative treatment including exercise programs and physical therapy; when these therapies fail, patients are considered for surgery. Two of the most commonly used surgical approaches are the supraclavicular exposure and the transaxillary approach with first rib resection. On occasion, these approaches may be combined or, alternatively, posterior subscapular exposure may be used in selected patients.

CONCLUSION:
TOS is perhaps the most difficult entrapment neuropathy encountered by neurosurgeons. Surgical intervention is indicated for vascular and true neurogenic TOS and for some patients with the common or nonspecific type of TOS in whom nonoperative therapies fail. With careful patient selection, operative intervention usually yields satisfactory results.