bluuebunny/crossref_metadata_2025_split huggingface dataset:
Surgical management of cerebellar hemangioblastomas in patients with von Hippel–Lindau disease
Authors: Jagannathan Jay , Lonser Russell R. , Smith Rene , DeVroom Hetty L. , Oldfield Edward H. 
Published: February 2008

Object
Despite the frequency of cerebellar hemangioblastomas in von Hippel–Lindau (VHL) disease, their optimum contemporary management has not been defined, and is made complex because of the multiple, progressive, and protean nature of the tumors found in patients with this disorder. To examine modern management and outcomes of cerebellar hemangioblastomas in VHL disease, the authors reviewed findings in patients with this disease who underwent resection of cerebellar hemangioblastomas.

Methods
Consecutive patients with VHL disease who underwent surgery for cerebellar hemangioblastoma(s) at the National Institutes of Health were included. Eighty consecutive patients (44 female and 36 male patients) underwent 126 operations for removal of 164 cerebellar hemangioblastomas (age at surgery 37.8 ± 10.3 years, follow-up duration 96.0 ± 60.3 months). Serial clinical examinations, imaging studies, and operative records were analyzed.

Results
Symptoms and signs included headache (94 operations; 75%), ataxia (55%), dysmetria (29%), and hydrocephalus (28%). Although the primary objective of surgery was resection of the hemangioblastoma considered responsible for symptoms (136 of the hemangioblastomas [83%]), 28 additional hemangioblastomas (17%) were removed during the same surgeries. Tumors associated with symptoms were larger (diameter 1.8 ± 1.9 cm; volume 2.8 ± 3.4 cm3; p < 0.05) and more likely to be associated with peritumoral edema or peritumoral cysts (100% associated with edema and/or cyst; p < 0.05) than asymptomatic tumors (diameter 1.1 ± 0.9 cm; volume 0.7 ± 0.4 cm3; 18%). More tumors were located in the posterior (74%) compared with the anterior (26%) half of the cerebellum (p < 0.05). Three months after resection, symptom improvement/stabilization had occurred following 124 of the operations (98%). Preoperative hydrocephalus resolved after tumor removal in 33 cases (94%) and did not require cerebrospinal fluid diversion. Long-term imaging follow-up (61.5 ± 15.0 months) revealed no recurrences.

Conclusions
Symptoms and signs caused by cerebellar hemangioblastomas in VHL disease are associated with edema and peritumoral cyst formation/propagation and are treated safely and effectively with resection. Cerebrospinal fluid diversion is rarely necessary after complete tumor removal in patients with preoperative hydrocephalus. Cerebellar hemangioblastomas are preferentially distributed in the posterior half of the cerebellum, as they are in the brainstem and spinal cord. Tumor recurrence is avoided by meticulous extracapsular resection.
Comparative risk of leptomeningeal disease after resection or stereotactic radiosurgery for solid tumor metastasis to the posterior fossa
Authors: Suki Dima , Abouassi Hiba , Patel Akash J. , Sawaya Raymond , Weinberg Jeffrey S. , Groves Morris D. 
Published: February 2008

Object
The authors tested the hypothesis that patients with metastatic posterior fossa lesions (MPFLs) treated with resection have a higher risk of leptomeningeal disease (LMD) than those with MPFLs treated with stereotactic radiosurgery (SRS).

Methods
Between 1993 and 2004, 379 patients with MPFLs were treated with resection or SRS at The University of Texas M. D. Anderson Cancer Center. The authors' primary study outcome was the incidence of LMD, as diagnosed with cerebrospinal fluid cytological analysis and/or neuroimaging.

Results
Resection was performed in 260 patients, whereas 119 patients underwent SRS. The median patient age was 56 years, 51% of patients were male, and 93% had a Karnofsky Performance Scale score $ 70. The most common primary cancers were those of the lung, breast, and kidney, as well as melanoma. Leptomeningeal dissemination of cancer occurred in 33 patients: 26 in the resection group and 7 in the SRS group (resection group: rate ratio [RR] 2.06, 95% confidence interval [CI] 0.89–4.75, p = 0.09). Piecemeal tumor resection (137 cases) was associated with a significantly higher risk of LMD than en bloc resection (123 cases; RR 3.4, 95% CI 1.43–8.12, p = 0.006) or SRS (RR 3.37, 95% CI 1.41–8.04, p = 0.006), and there was no significant difference in the risk for LMD between en bloc resection and SRS (en bloc resection: RR 0.98, 95% CI 0.34–2.81, p = 0.98). The multivariate RR and significance associated with piecemeal resection, however, were consistent, with a strong effect (RR 2.45, 95% CI 1.19–5.02, p = 0.02) and no indication of biases associated with tumor size, location, or cystic/necrotic appearance.

Conclusions
There is an increased risk of LMD after piecemeal resection of an MPFL. This increase, although clinically and statistically significant, is not as alarming as previously reported and is absent when en bloc removal is achieved. Further assessment of the role of resection in a controlled prospective setting is warranted.
Preoperative functional magnetic resonance imaging assessment of higher-order cognitive function in patients undergoing surgery for brain tumors
Authors: Amiez Céline , Kostopoulos Penelope , Champod Anne-Sophie , Collins D. Louis , Doyon Julien , Maestro Rolando Del , Petrides Michael 
Published: February 2008

Object
Resection of brain tumors has been shown to increase patient survival. The extent of the possible resection, however, depends on whether the tumor has invaded brain regions important for motor, sensory, or cognitive processes and whether the brain tissue surrounding the tumor maintains its functional role. The goal of the present study was to develop new pre- and intraoperative tools to specifically assess the function of the rostral part of the dorsal premotor cortex (PMdr) in 4 patients with brain tumors close to this region.

Methods
Using functional magnetic resonance (fMR) imaging and a task developed to assess accurate selection between competing responses based on conditional rules, the authors preoperatively assessed the function of the PMdr in 4 patients with brain tumors close to this region. In 1 patient, the authors developed an intraoperative procedure to assess performance on the task during the tumor resection.

Results
Preoperative fMR imaging data showed specific activity increases in the vicinity of the tumors, that is, in the PMdr. As confirmed by postoperative structural MR imaging, the extent of the tumor resection was optimal and the functional region within the PMdr was preserved. Furthermore, patients exhibited no postoperative deficits during task performance, demonstrating that the function was preserved. Intraoperative behavioral results demonstrated that the cognitive processes underlying performance on the task remained intact throughout the tumor resection.

Conclusions
These findings suggest that preoperative fMR imaging, together with intraoperative behavioral evaluation, may be a useful paradigm to assist neurosurgeons in preserving cognitive function in patients with brain tumors.
Cerebrospinal fluid leakage into the subdural space: possible influence on the pathogenesis and recurrence frequency of chronic subdural hematoma and subdural hygroma
Authors: Kristof Rudolf A. , Grimm Jochen M. , Stoffel-Wagner Birgit 
Published: February 2008

Object
The purpose of this study was to clarify whether cerebrospinal fluid (CSF) leakage into the subdural space is involved in the genesis of chronic subdural hematoma (CSDH) and subdural hygroma (SH) and to clarify whether this leakage of CSF into the subdural space influences the postoperative recurrence rate of CSDH and SH.

Methods
In this prospective observational study, 75 cases involving patients treated surgically for CSDH (67 patients) or SH (8 patients) were evaluated with respect to clinical and radiological findings at presentation, the content of β -trace protein (β TP) in the subdural fluid (βTPSF) and serum (βTPSER), and the CSDH/SH recurrence rate. The βTPSF was considered to indicate an admixture of CSF to the subdural fluid if βTPSF/βTPSER > 2.

Results
The median β TPSF level for the whole patient group was 4.29 mg/L (range 0.33–51 mg/L). Cerebrospinal fluid leakage, as indicated by βTPSF/βTPSER > 2, was found to be present in 93% of the patients with CSDH and in 100% of the patients with SH (p = 0.724). In patients who later had to undergo repeated surgery for recurrence of CSDH/SH, the βTPSF concentrations (median 6.69 mg/L, range 0.59–51 mg/L) were significantly higher (p = 0.04) than in patients not requiring reoperation (median 4.12 mg/L, range 0.33–26.8 mg/L).

Conclusions
As indicated by the presence of βTP in the subdural fluid, CSF leakage into the subdural space is present in the vast majority of patients with CSDH and SH. This leakage could be involved in the pathogenesis of CSDH and SH. Patients who experience recurrences of CSDH and SH have significantly higher concentrations of βTPSF at initial presentation than patients not requiring reoperation for recurrence. These findings are presented in the literature for the first time and have to be confirmed and expanded upon by further studies.
Neurosurgery patients' feelings about the role of residents in their care: a qualitative case study
Authors: Knifed Eva , July Julius , Bernstein Mark 
Published: February 2008

Object
The role of residents in surgery is not clearly explained to patients. The authors undertook a study to explore the level of knowledge and anxiety in patients regarding residents' involvement in their surgery.

Methods
Qualitative case study methodology was used. Thirty face-to-face interviews of patients were conducted prior to elective neurosurgery. Interviews were transcribed and subjected to modified thematic analysis by 4 reviewers. The majority of patients had a postsecondary education, and there was substantial religious and ethnic diversity among them. Most underwent craniotomy for brain tumor.

Results
Six prominent themes arose from the analysis: 1) the level of knowledge about residents is low; 2) the level of anxiety about residents is low; 3) it is desirable for patients to meet the residents before surgery; 4) residents' educational needs are understood and supported; 5) anxiety was not increased by the interview; and 6) patients trust in the medical system.

Conclusions
Patients appear to be unaware of the role of residents in their surgical care but do not seem anxious about it. Trust in the medical system helps patients proceed with risky operations. Surgeons could be more forthcoming with patients about the role of residents.
Harvested human neurons engineered as live nervous tissue constructs: implications for transplantation
Authors: Huang Jason H. , Zager Eric L. , Zhang Jun , Groff Robert F. , Pfister Bryan J. , Cohen Akiva S. , Grady M. Sean , Maloney-Wilensky Eileen , Smith Douglas H. 
Published: February 2008

Object
Although neuron transplantation to repair the nervous system has shown promise in animal models, there are few practical sources of viable neurons for clinical application and insufficient approaches to bridge extensive nerve damage in patients. Therefore, the authors sought a clinically relevant source of neurons that could be engineered into transplantable nervous tissue constructs. The authors chose to evaluate human dorsal root ganglion (DRG) neurons due to their robustness in culture.

Methods
Cervical DRGs were harvested from 16 live patients following elective ganglionectomies, and thoracic DRGs were harvested from 4 organ donor patients. Following harvest, the DRGs were digested in a dispase–collagenase treatment to dissociate neurons for culture. In addition, dissociated human DRG neurons were placed in a specially designed axon expansion chamber that induces continuous mechanical tension on axon fascicles spanning 2 populations of neurons originally plated ∼ 100 μm apart.

Results
The adult human DRG neurons, positively identified by neuronal markers, survived at least 3 months in culture while maintaining the ability to generate action potentials. Stretch-growth of axon fascicles in the expansion chamber occurred at the rate of 1 mm/day to a length of 1 cm, creating the first engineered living human nervous tissue constructs.

Conclusions
These data demonstrate the promise of adult human DRG neurons as an alternative transplant material due to their availability, viability, and capacity to be engineered. Also, these data show the feasibility of harvesting DRGs from living patients as a source of neurons for autologous transplant as well as from organ donors to serve as an allograft source of neurons.
Virtual reality system for planning minimally invasive neurosurgery
Authors: Stadie Axel Thomas , Kockro Ralf Alfons , Reisch Robert , Tropine Andrei , Boor Stephan , Stoeter Peter , Perneczky Axel 
Published: February 2008

Object
The authors report on their experience with a 3D virtual reality system for planning minimally invasive neurosurgical procedures.

Methods
Between October 2002 and April 2006, the authors used the Dextroscope (Volume Interactions, Ltd.) to plan neurosurgical procedures in 106 patients, including 100 with intracranial and 6 with spinal lesions. The planning was performed 1 to 3 days preoperatively, and in 12 cases, 3D prints of the planning procedure were taken into the operating room. A questionnaire was completed by the neurosurgeon after the planning procedure.

Results
After a short period of acclimatization, the system proved easy to operate and is currently used routinely for preoperative planning of difficult cases at the authors' institution. It was felt that working with a virtual reality multimodal model of the patient significantly improved surgical planning. The pathoanatomy in individual patients could easily be understood in great detail, enabling the authors to determine the surgical trajectory precisely and in the most minimally invasive way.

Conclusions
The authors found the preoperative 3D model to be in high concordance with intraoperative conditions; the resulting intraoperative “déjà-vu” feeling enhanced surgical confidence. In all procedures planned with the Dextroscope, the chosen surgical strategy proved to be the correct choice.
Three-dimensional virtual reality models of a patient allow quick and easy understanding of complex intracranial lesions.
Association of the HLA–DRB1 gene with premature death, particularly from cardiovascular disease, in patients with rheumatoid arthritis and inflammatory polyarthritis
Authors: Farragher Tracey M. , Goodson Nicola J. , Naseem Haris , Silman Alan J. , Thomson Wendy , Symmons Deborah , Barton Anne 
Published: January 2008

AbstractObjectiveTo examine the role of the variants of the PTPN22 and HLA–DRB1 genes as predictors of mortality in inflammatory polyarthritis (IP) and rheumatoid arthritis (RA).MethodsPatients were recruited from a primary care–based inception cohort of patients with IP and were followed up prospectively. For patients who died, the cause and date of death was obtained. Cox proportional hazards regression models were used to assess the association of the HLA–DRB1 (including the shared epitope [SE]) and PTPN22 genes with the risk of death from all causes and from cardiovascular disease (CVD) and to assess the interactions between SE, smoking, and anti–cyclic citrullinated peptide (anti‐CCP) status, adjusted by age at symptom onset and sex.ResultsDNA samples were available from 1,022 IP patients. During followup, 751 of them (74%) satisfied the American College of Rheumatology 1987 criteria for RA, and 242 of them (24%) died. Carriage of 2 copies of SE alleles predicted death from all causes (hazard ratio [HR] 1.57 [95% confidence interval (95% CI) 1.1–2.2]) and from CVD (HR 1.68 [95% CI 1.1–2.7]). This effect was most marked for individuals with the HLA–DRB1*01/*04 combination. An interaction of smoking, SE alleles, and anti‐CCP antibodies was observed and was associated with the greatest risk of death from CVD (HR 7.81 [95% CI 2.6–23.2]). No association of the PTPN22 gene with mortality was detected.ConclusionSE alleles, particularly compound heterozygotes, are associated with death from all causes and from CVD, independently of autoantibody status. However, the combination of SE, smoking, and anti‐CCP antibodies is associated with a high risk of premature death in patients with IP and RA, which raises the possibility of a targeted strategy to prevent CVD in these patients.
Haplotypes of the HRES‐1 endogenous retrovirus are associated with development and disease manifestations of systemic lupus erythematosus
Authors: Pullmann Rudolf , Bonilla Eduardo , Phillips Paul E. , Middleton Frank A. , Perl Andras 
Published: January 2008

AbstractObjectiveEndogenous retroviral sequences represent a link between viral and genetic factors that may influence the development of systemic lupus erythematosus (SLE). The HRES‐1 human endogenous retroviral sequence is centrally located at the 1q42 chromosomal region relative to microsatellites previously associated with SLE. We therefore undertook the present study to determine the haplotypes of the HRES‐1 locus and their linkage to SLE.MethodsOne hundred six patients with SLE, 82 unrelated healthy Caucasian individuals, and 70 healthy members of 34 lupus families were examined. HRES‐1 was amplified by polymerase chain reaction (PCR) and analyzed by sequencing and restriction enzyme mapping. Microsatellites were analyzed by PCR. Haplotype construction and transmission disequilibrium testing (TDT) were performed in lupus families.ResultsBased on 4 single‐nucleotide polymorphisms (SNPs) within a 935‐base interval, we detected 6 HRES‐1 haplotypes that were differentially segregated in unrelated Caucasian patients and control subjects (χ2 = 16.86, P = 0.0048) and were in linkage disequilibrium (LD) with the D1S225 microsatellite (P = 0.0002). The microsatellites D1S225, D1S235, and D1S2785 (but not D1S229) were linked to SLE by TDT. Interestingly, LD between HRES‐1 SNPs at bases 653 and 1259 was reduced in patients with SLE (P = 0.048). The HRES‐1 653C/1259C–harboring alleles were associated with the presence of renal disease (P = 0.0021) and with the absence of lung disease (P = 0.0323), while the 956A allele was associated with the antiphospholipid syndrome in patients with SLE (P = 0.0036).ConclusionThe HRES‐1 locus represents a recombination hot spot at the 1q42 chromosomal region that influences the development and disease manifestations of SLE.
Hsp60 in inflamed muscle tissue is the target of regulatory autoreactive T cells in patients with juvenile dermatomyositis
Authors: Elst Elisabeth F. , Klein Mark , de Jager Wilco , Kamphuis Sylvia , Wedderburn Lucy R. , van der Zee Ruurd , Albani Salvatore , Kuis Wietse , Prakken Berent J. 
Published: January 2008

AbstractObjectiveJuvenile dermatomyositis (DM) is an autoimmune disease of unknown origin characterized by muscle weakness and skin manifestations. No definite autoantigen has yet been identified. Heat‐shock proteins (HSPs) can be up‐regulated at sites of inflammation, and immune reactivity to Hsp60 is suggested to play a regulatory role in various chronic inflammatory diseases. The purpose of this study was to determine whether Hsp60 could serve as an autoantigen in juvenile DM.MethodsMuscle tissue from 4 patients with juvenile DM and 1 healthy control subject without evidence of muscle disease was stained for Hsp60. Peripheral blood mononuclear cells (PBMCs) from 22 patients and 10 healthy control subjects were tested for T cell proliferation induced by human and microbial Hsp60. Cytokine production in response to Hsp60 was examined in 15 patients and 6 healthy controls. T cell reactivity to Hsp60 was determined in muscle biopsy samples from 2 patients.ResultsWe found significantly increased T cell proliferation to human Hsp60 in PBMCs from juvenile DM patients, which was higher during disease remission. Following in vitro activation with Hsp60, significant amounts of tumor necrosis factor α, interleukin‐1β (IL‐1β), and IL‐10 were produced. In contrast to muscle biopsy samples from healthy controls, samples from juvenile DM patients showed up‐regulation of Hsp60, induction of T cell proliferation, and production of cytokines. Production of proinflammatory cytokines by muscle‐derived cells in response to Hsp60 was associated with a poor clinical prognosis, whereas human Hsp60–specific induction of IL‐10 was followed by clinical remission.ConclusionThese findings suggest that human (self) Hsp60 is a disease‐relevant autoantigen in juvenile DM. The difference in T cell response with regard to disease activity indicates an immune regulatory effect of Hsp60‐specific T cells, opening up perspectives for antigen‐specific immunotherapy.
A six‐month randomized controlled trial of exercise and pyridostigmine in the treatment of fibromyalgia
Authors: Jones K. D. , Burckhardt C. S. , Deodhar A. A. , Perrin N. A. , Hanson G. C. , Bennett R. M. 
Published: January 2008

AbstractObjectiveA subset of fibromyalgia (FM) patients have a dysfunctional hypothalamic–pituitary–insulin‐like growth factor 1 (IGF‐1) axis, as evidenced by low serum levels of IGF‐1 and a reduced growth hormone (GH) response to physiologic stimuli. There is evidence that pyridostigmine (PYD) improves the acute response of GH to exercise in FM patients. The purpose of this study was to evaluate the clinical effectiveness of 6 months of PYD and group exercise on FM symptoms.MethodsFM patients were randomized to 1 of the following 4 groups: PYD plus exercise, PYD plus diet recall but no exercise, placebo plus exercise, and placebo plus diet recall but no exercise. The primary outcome measures were the visual analog scale (VAS) score for pain, tender point count, and total myalgic score. Secondary outcome measures were the total score on the Fibromyalgia Impact Questionnaire (FIQ) and FIQ VAS scores for individual symptoms (fatigue, poor sleep, stiffness, and anxiety), as well as quality of life (QOL) and physical fitness (lower body strength/endurance, upper and lower body flexibility, balance, and time on the treadmill).ResultsA total of 165 FM patients completed baseline measurements; 154 (93.3%) completed the study. The combination of PYD and exercise did not improve pain scores. PYD groups showed a significant improvement in sleep and anxiety in those who completed the study and in QOL in those who complied with the therapeutic regimen as compared with the placebo groups. Compared with the nonexercise groups, the 2 exercise groups demonstrated improvement in fatigue and fitness. PYD was generally well tolerated.ConclusionNeither the combination of PYD plus supervised exercise nor either treatment alone yielded improvement in most FM symptoms. However, PYD did improve anxiety and sleep, and exercise improved fatigue and fitness. We speculate that PYD may have improved vagal tone, thus benefiting sleep and anxiety; this notion warrants further study.