Organization of area hV5/MT+ in subjects with homonymous visual field defects.

Damage to the primary visual cortex (V1) leads to a visual field loss (scotoma) in the retinotopically corresponding part of the visual field. Nonetheless, a small amount of residual visual sensitivity persists within the blind field. This residual capacity has been linked to activity observed in the middle temporal area complex (V5/MT+). However, it remains unknown whether the organization of hV5/MT+ changes following early visual cortical lesions. We studied the organization of area hV5/MT+ of five patients with dense homonymous defects in a quadrant of the visual field as a result of partial V1+ or optic radiation lesions. To do so, we developed a new method, which models the boundaries of population receptive fields directly from the BOLD signal of each voxel in the visual cortex. We found responses in hV5/MT+ arising inside the scotoma for all patients and identified two possible sources of activation: 1) responses might originate from partially lesioned parts of area V1 corresponding to the scotoma, and 2) responses can also originate independent of area V1 input suggesting the existence of functional V1-bypassing pathways. Apparently, visually driven activity observed in hV5/MT+ is not sufficient to mediate conscious vision. More surprisingly, visually driven activity in corresponding regions of V1 and early extrastriate areas including hV5/MT+ did not guarantee visual perception in the group of patients with post-geniculate lesions that we examined. This suggests that the fine coordination of visual activity patterns across visual areas may be an important determinant of whether visual perception persists following visual cortical lesions.

Diffusion Tensor Imaging in brain tumors: A study on gliomas and metastases.

PURPOSE: To explore the role of Diffusion Tensor Imaging in preoperative glioma grading, as well as in differentiation between gliomas and metastatic brain tumors. We measured diffusion tensor variables in enhancement and edema regions, which were compared between the different subject groups. MATERIALS AND METHODS: We performed DTI in 48 patients (11 Low Grade Gliomas, 27 High Grade Gliomas, 10 Single Metastatic brain tumors). We measured FA, λ1, λ2, λ3, ADC, Cl, Cp, Cs, RA, and VR in enhancing portions of tumors and edema regions. Additionally, ratios of enhancement to edema values were created for each variable. RESULTS: In peritumoral edema, Cl and RA were proven to be significantly different in pair-wise comparisons, in addition to ADC, Cp, Cs and VR in enhancement regions. Enhancement to edema values were significantly different as well. CONCLUSION: Diffusion tensor indices could be used for the differentiation between low and high grade gliomas, as well as for distinction between gliomas and metastases.

Population receptive field analysis of the primary visual cortex complements perimetry in patients with homonymous visual field defects.

Injury to the primary visual cortex (V1) typically leads to loss of conscious vision in the corresponding, homonymous region of the contralateral visual hemifield (scotoma). Several studies suggest that V1 is highly plastic after injury to the visual pathways, whereas others have called this conclusion into question. We used functional magnetic resonance imaging (fMRI) to measure area V1 population receptive field (pRF) properties in five patients with partial or complete quadrantic visual field loss as a result of partial V1+ or optic radiation lesions. Comparisons were made with healthy controls deprived of visual stimulation in one quadrant ["artificial scotoma" (AS)]. We observed no large-scale changes in spared-V1 topography as the V1/V2 border remained stable, and pRF eccentricity versus cortical-distance plots were similar to those of controls. Interestingly, three observations suggest limited reorganization: (i) the distribution of pRF centers in spared-V1 was shifted slightly toward the scotoma border in 2 of 5 patients compared with AS controls; (ii) pRF size in spared-V1 was slightly increased in patients near the scotoma border; and (iii) pRF size in the contralesional hemisphere was slightly increased compared with AS controls. Importantly, pRF measurements yield information about the functional properties of spared-V1 cortex not provided by standard perimetry mapping. In three patients, spared-V1 pRF maps overlapped significantly with dense regions of the perimetric scotoma, suggesting that pRF analysis may help identify visual field locations amenable to rehabilitation. Conversely, in the remaining two patients, spared-V1 pRF maps failed to cover sighted locations in the perimetric map, indicating the existence of V1-bypassing pathways able to mediate useful vision.

Brain-computer interfaces increase whole-brain signal to noise.

Brain-computer interfaces (BCIs) can convert mental states into signals to drive real-world devices, but it is not known if a given covert task is the same when performed with and without BCI-based control. Using a BCI likely involves additional cognitive processes, such as multitasking, attention, and conflict monitoring. In addition, it is challenging to measure the quality of covert task performance. We used whole-brain classifier-based real-time functional MRI to address these issues, because the method provides both classifier-based maps to examine the neural requirements of BCI and classification accuracy to quantify the quality of task performance. Subjects performed a covert counting task at fast and slow rates to control a visual interface. Compared with the same task when viewing but not controlling the interface, we observed that being in control of a BCI improved task classification of fast and slow counting states. Additional BCI control increased subjects' whole-brain signal-to-noise ratio compared with the absence of control. The neural pattern for control consisted of a positive network comprised of dorsal parietal and frontal regions and the anterior insula of the right hemisphere as well as an expansive negative network of regions. These findings suggest that real-time functional MRI can serve as a platform for exploring information processing and frontoparietal and insula network-based regulation of whole-brain task signal-to-noise ratio.

Brain natriuretic peptide as a cardiotoxicity biomarker in children with hematological malignancies.

AIM: Aim of our study was to evaluate BNP as early cardiotoxicity biomarker after completion of chemotherapy in twenty children with hematological malignancies at diagnosis (t=0) and after completion of intensive chemotherapy (t=1). METHODS: Demographic data, underlying disease, cumulative anthracyclines dose, measurement of serum BNP and evaluation of systolic function of left ventricle with ejection fraction (EF) and shortening fraction (FS) in both times . Pathological values for EF and FS were found in 4 (20%) and 1 (5%) patient at t=1, while respective values were normal at diagnosis. RESULTS: Mean BNP values at t=0 were 59.09±19.95 pg/mL and differ significantly from values at t=1 (153.22±29.14 pg/mL) (P=0.04). Mean value of EF also differed significantly (75.42±4.11% vs. 69.87±10.51%, P=0.04). No statistic difference was found regarding FS values at both (P=0.102). CONCLUSION: Present data indicate that anthracyclines related cardiotoxicity is registered in children with hematological malignancies and BNP represents a useful biomarker of myocardial dysfunction.

Epileptic seizures after octreotide administration in a 6.5-year-old female with ALL and L-asparaginase associated pancreatitis: a possible drug interaction.

INTRODUCTION: Octreotide is a synthetic somatostatin analogue which has been suggested for use in the management of acute pancreatitis, though its safety and effectiveness in the pediatric setting has not been extensively studied. CASE REPORT: we present a rare case of a 6.5-year-old female with acute lymphoblastic leukemia (ALL) and L-asparaginase (L-asp) induced pancreatitis, who developed epileptic seizures, possibly associated with octreotide administration. Her imaging and laboratory findings ruled out a leukemic involvement or infection of CNS. The EEG revealed repetitive sharp waves maximal on the frontal and temporal areas of the right hemisphere. The child was treated with diazepam and she continued with systemic anticonvulsant treatment with levetiracetam. After 2 weeks of conservative treatment, pancreatitis resolved and she continued her chemotherapy protocol. Levetiracetam treatment lasted 8 months. 7 months after the first episode, EEG was reported as normal, and the child completed the chemotherapy protocol without any further severe complications. CONCLUSIONS: Larger and well designed studies are needed to warrant the safety of octreotide in pediatric population.

Weaver syndrome associated with bilateral congenital hip and unilateral subtalar dislocation.

BACKGROUND: Weaver syndrome is a congenital paediatric syndrome characterized by mental, respiratory and musculoskeletal manifestations. The coexisting deformities of the skull, the face, fingers and toes are typical. We report a case of a girl with Weaver syndrome associated with rare bilateral congenital dislocation of the hips associated with congenital hypoplastic talus and subtalar dislocation of her ankle joint. CASE REPORT: A 3-year old girl was admitted in our department with typical manifestations of Weaver syndrome, associated with congenital dislocation of bilateral hips, hypoplastic talus and subtalar dislocation of her right ankle. She was in pain while standing upright and incapable of independent walking. Both hips were treated operatively with open reduction and bilateral iliac osteotomy. Two years afterwards she had an open reduction of her talus and extraarticular arthrodesis of her subtalar joint in her right ankle. Six years postoperatively after the hip operations and four years after the ankle operation the girl is ambulant with a painless independent and unaided walking with a mild limp and full range of movements in all the operated joints. CONCLUSIONS: We suggest that children with Weaver syndrome and disabling musculosceletal deformities, particularly affecting their ability to stand up and walk should be treated early, before bone maturity, in order to achieve the best potential musculoskeletal as well as developmental outcome.

Neurofeedback of two motor functions using supervised learning-based real-time functional magnetic resonance imaging.

This study examines the effects of neurofeedback provided by support vector machine (SVM) classification-based real-time functional magnetic resonance imaging (rt-fMRI) during two types of motor tasks. This approach also enables the examination of the neural regions associated with predicting mental states in different domains of motor control, which is critical to further our understanding of normal and impaired function. Healthy volunteers (n = 13) performed both a simple button tapping task, and a covert rate-of-speech counting task. The average prediction accuracy was approximately 95% for the button tapping task and 86% for the speech task. However, subsequent offline analysis revealed that classification of the initial runs was significantly lower - 75% (p<0.001) for button and 72% (p<0.005) for speech. To explore this effect, a group analysis was performed using the spatial maps derived from the SVM models, which showed significant differences between the two fMRI runs. One possible explanation for the difference in spatial patterns and the asymmetry in the prediction accuracies is that when subjects are actively engaged in the task (i.e. when they are trying to control a computer interface), they are generating stronger BOLD responses in terms of both intensity and spatial extent.

Management of paediatric sinonasal rhabdomyosarcoma.

BACKGROUND AND AIM: Rhabdomyosarcoma is the commonest malignant tumour of the nose and paranasal sinuses in the paediatric population. Due to its rarity and largely unknown biological behaviour, the treatment of this tumour is complex and controversial. We present the results of multimodality treatment of paediatric sinonasal rhabdomyosarcoma, and we explore the role of surgery in the management of this malignancy. METHODS: We retrospectively reviewed the records of 14 patients (median age 7.5 years) with sinonasal rhabdomyosarcoma. Six patients underwent major surgery with post-operative chemoradiation. Eight patients received multi-agent chemotherapy and radiotherapy. The mean follow-up time was 58 months (range seven to 276 months). RESULTS: The five-year overall survival rates for all patients and for the surgery group were 53.9 and 83.3 per cent, respectively. All patients with alveolar rhabdomyosarcoma had a poor prognosis, with a median survival time of 17 months. Intracranial extension and an age greater than 10 years were also associated with an unfavourable outcome. Non- or partial responders to initial chemoradiation died within a year of diagnosis. CONCLUSIONS: Management of paediatric rhabdomyosarcoma requires a combination of chemotherapy, radiotherapy and surgery. Primary chemoradiotherapy is the established treatment approach for advanced tumours. Early stage tumours with favourable histology can be treated successfully with radical surgery, provided that function and cosmetic appearance are preserved.

An insight into what superconducts in polycrystalline boron-doped diamonds based on investigations of microstructure.

The discovery of superconductivity in polycrystalline boron-doped diamond (BDD) synthesized under high pressure and high temperatures [Ekimov, et al. (2004) Nature 428:542-545] has raised a number of questions on the origin of the superconducting state. It was suggested that the heavy boron doping of diamond eventually leads to superconductivity. To justify such statements more detailed information on the microstructure of the composite materials and on the exact boron content in the diamond grains is needed. For that we used high-resolution transmission electron microscopy and electron energy loss spectroscopy. For the studied superconducting BDD samples synthesized at high pressures and high temperatures the diamond grain sizes are approximately 1-2 mum with a boron content between 0.2 (2) and 0.5 (1) at %. The grains are separated by 10- to 20-nm-thick layers and triangular-shaped pockets of predominantly (at least 95 at %) amorphous boron. These results render superconductivity caused by the heavy boron doping in diamond highly unlikely.

Expression of multidrug resistance 1 (MDR1), multidrug resistance-related protein 1 (MRP1), lung resistance protein (LRP), and breast cancer resistance protein (BCRP) genes and clinical outcome in childhood acute lymphoblastic leukemia.

The aim of this prospective study was to analyze the expression of messenger RNA of genes, such as MDR1, MRP1, BCRP, and LRP, implicated in the mechanism of multidrug resistance (MDR) in relation to the response to induction chemotherapy and relapse and these genes' correlation with each other and with pretreatment laboratory and clinical characteristics. We prospectively studied 49 children (26 boys and 23 girls) with acute lymphoblastic leukemia (ALL) (median age, 5.5 years; range, 15 months to 12.5 years) who were treated with the BFM95 chemotherapy protocol. We used bone marrow mononuclear cells from 7 healthy children as controls. The expression of MDR genes and the beta-actin housekeeping gene was detected by the reverse transcription-polymerase chain reaction with the appropriate primers. The mean expression of each MDR gene was significantly higher in the patients than in the control group (P < .01). We found statistically significant correlations between MRP1 and LRP expression and between MRP1 or LRP expression and MDR1 expression (P < .05). High expression for the MDR1 gene was found in 18 patients (36.7%), and their prognoses were significantly worse than those with low expression (event-free survival, 55.56% versus 86.67%; P = .03, log-rank test). Expression of each of the MDR genes was independent of the initial white blood cell count, immunophenotype, National Cancer Institute risk classification, and prednisone response. Interestingly, MDR1 expression was significantly higher at relapse than at diagnosis for 4 sample pairs. Evaluation of MDR1 expression at diagnosis of childhood ALL may contribute to the early identification of patients at risk of treatment failure.

Significant differences of lymphocytes isolated from ascites of patients with ovarian cancer compared to blood and tumor lymphocytes. Association of CD3+CD56+ cells with platinum resistance.

OBJECTIVES: Tumor infiltrating lymphocytes (TILs) and T regulatory cells (Tregs) have been associated with prognosis in ovarian cancer, but their prognostic significance in ascites has not been studied. We performed a prospective study of T lymphocytes isolated from ascites from patients with ovarian carcinoma and we compared them with the respective populations in blood and tumors. METHODS: Mononuclear cells from ascites (n=71) and blood were isolated by Ficoll, while tumor lymphocytes (n=20) were obtained upon mechanical dissociation. Phenotypic analysis was performed with flow cytometry. Ascites from 10 patients with cirrhosis was used as control. RESULTS: Tregs containing CD4(+)CD25(+) cells, NK-T containing CD3(+)CD56(+) cells and CD69 and HLADR expression of CD4 and CD8 lymphocytes were significantly increased in tumor ascites compared to blood and control ascites. A selective accumulation of these populations in the ascites of cancer patients, was suggested by the significantly higher ascites/blood (A/B) ratios in cancer patients but not controls. Cancer cell content in ascites was correlated with CD4(+)CD25(+), CD4(+)CD69(+), CD4(+)HLADR(+) and CD8(+)CD69(+) cells. There was no correlation of lymphocyte populations between ascites and samples from peritoneal metastases. Higher tumor grade was associated with increased A/B CD4(+)CD25(+) ratio and reduced CD3(+)CD56(+) cells, while platinum resistance was associated with reduced A/B CD3(+)CD56(+) ratio. CONCLUSIONS: There are significant differences of CD3(+)CD56(+) and CD25(+)CD4(+) lymphocytes and increase in lymphocyte activation between blood, ascites and peritoneal metastases from patients with ovarian cancer. The selective accumulation of CD3(+)CD56(+) population in ascites may be a predictive factor for platinum resistance.

Laboratory measured behavioral impulsivity relates to suicide attempt history.

The purpose of this study was to examine the relationship between laboratory behavioral measured impulsivity (using the Immediate and Delayed Memory Tasks) and suicidal attempt histories. Three groups of adults were recruited, those with either: no previous suicide attempts (Control, n = 20), only a single suicide attempt (Single, n = 20), or multiple suicidal attempts (Multiple, n = 10). As hypothesized, impulsive responses increased with the number of suicide attempts (Control < Single < Multiple). This study helps to demonstrate how laboratory behavioral measures of impulsivity can be used to discriminate groups based on suicidal histories among samples not currently exhibiting significant suicidal behaviors.

Hemangiopericytoma in an adolescent girl: a case report.

Hemangiopericytoma (HPC) is a rare soft tissue tumor The few published reports account for the little information available on its clinical management. Here the authors report the successful treatment of an adolescent girl with rare HPC of the tongue. After incomplete surgical excision of the tumor she was admitted to the Hematology-Oncology Department and was treated with a 3-drug combination regimen (ifosfamide, actinomycin D, vincristine) for 8 weeks. She achieved partial remission in week 9 based on the magnetic resonance imaging (MRI)findings. Conventional radiation therapy was initiated at week 9 and continued until week 16. At week 20, according to the MRI findings, she achieved complete remission and continuation therapy was initiated. The young girl has been alive without evidence of the disease for the last 3 years of follow-up. In conclusion, the current report indicates that in cases of incomplete surgical excision of the tumor, chemotherapy and radiotherapy seem to be effective.

Laparoscopic restaging of early stage invasive adnexal tumors: a 10-year experience.

UNLABELLED: Surgical staging of apparent early stage adnexal carcinoma provides indispensable information. A significant number of patients are referred to tertiary centers with inadequate staging information. We report on our experience with late results of laparoscopic restaging procedure in uncompletely managed early adnexal carcinomas. MATERIALS AND METHODS: From 1991 to 2001, 53 laparoscopic restaging operations were performed: 42 patients were restaged early after initial surgery for an ovarian carcinoma (OC) in 35 of them, and for fallopian tube carcinomas (FTCs) in 7 others. Eleven patients were assessed as a second-look procedure, after six courses of platinum-based chemotherapy indicated for a high-risk tumor. The procedure systematically followed the guidelines of laparotomy. RESULTS: All except one (adhesions) procedures were successfully completed. Operative room time averaged 238 min and hospital stay 3.1 days. Only one major complication required laparotomy (1.8%). In the primary restaging group, eight patients were upstaged (19%) and were given chemotherapy. After a 54-month median follow-up, 3 out of the 34 remaining patients diagnosed as stage IA grades 1-2 (6.4%) recurred and died. In the group of 11 second-look operations, 4 were found positive after chemotherapy. One of the positive patient recurred and died. CONCLUSIONS: Laparoscopy seems to be an acceptable technical option to perform restaging of apparently early adnexal carcinomas. It spares the patients the discomfort of repeat laparotomy. Long-term outcome results suggest that laparoscopic staging, provided it meets the standards, accurately detects the patients who need chemotherapy and safely select the patients who can be proposed surgery only.

Prospective evaluation of surgical staging of advanced cervical cancer via a laparoscopic extraperitoneal approach.

OBJECTIVES: To report on a large series of cervical cancer patients at risk for lymph node metastasis who underwent surgical staging by a novel technique. METHODS: Between 1/97 and 3/02, we identified 111 patients who underwent an infrarenal aortic and common iliac lymph node dissection via a laparoscopic extraperitoneal approach for either bulky or locally advanced cervical cancer. We reviewed the medical records and extracted pertinent information. RESULTS: There were no intraoperative complications. Mean patient age was 46 (+/- 9) years. Mean node count was 19 (+/- 12). Thirty (27%) patients had lymph node metastasis. The mean operative time was 157 (+/- 46) min, and mean postoperative stay was 2 days. The majority of complications in the early part of the series were symptomatic lymphoceles. Since 4/01, preventive peritoneal marsupialization has been performed without lymphocele occurrence (37 patients). Two patients (2%) required reoperation. In the node-positive group, extended-field radiation and chemotherapy were well tolerated, but prognosis was dismal (median survival, 27 months). In the node-negative group, the median survival after pelvic radiation limited to the lower level of the surgical dissection was not reached after an average follow-up of 16.6 months. CONCLUSIONS: This novel technique is feasible and combines the benefits of laparoscopy with those of a retroperitoneal approach. It can be used to tailor external radiation therapy. The benefits of extended-field radiation therapy remain unclear; however, this approach does not preclude later use of radiation therapy, whereas possibly minimizing associated toxicities secondary to adhesions.

[Endometrial cancer: what's new?]. / Controverses et nouveautés dans l'adénocarcinome de l'endomètre.

OBJECTIVES: New and much debated data of the endometrial cancer concerning the preoperative assessment of myometrial invasion, the surgical staging, and the adjuvant treatment. PATIENTS AND METHODS: Medline (1998-2002): searching for "endometrial carcinoma". RESULTS: The pap smears are useful when it is difficult to have a transvaginal ultrasonography or an MRI. We can perform the pap smears and the endometrial biopsy in the clinic. If a patient has pap smears with malignant cells or elevated preoperative CA 125, it probably is a cancer with poor prognostic factors. Surgical staging with abdominal and node evaluation is necessary. The MRI seems to be the best preoperative imaging because we have information about adnexal and abdominal metastases, pelvic or aortic nodes and the invasion of the myometrium. So it gives us information on the surgical route, and provides indication for a lymphadenectomy. The surgical staging is a part of the treatment of the endometrial cancer: an exploration of the peritoneal cavity, a pelvic lymphadenectomy, a para-aortic lymphadenectomy if the pelvic nodes are positive or if there are factors of bad prognosis (deep stage IC, grade 3, adnexal or abdominal involvement, serous carcinoma of the endometrium). It can be performed if technical conditions are correct. The adjuvant teletherapy in the documented stage IpN0 (surgical staging with pelvic lymphadenectomy) does not seem to be necessary. But we can perform an adjuvant brachytherapy (high-dose rate if it is possible) in patients with a high local recurrence (stage IC, stage I with grade 3, stage IB grade 2). CONCLUSION: The preoperative MRI is useful choosing the surgical approach, and the depth of the myometrial invasion, which can be an indication for a pelvic lymphadenectomy. The surgical staging must be a part of the treatment of the endometrial cancer. So the adjuvant teletherapy in patients with stage IpN0 documented should not be used.

[Marfan syndrome and pregnancy. Apropos of 4 cases]. / Syndrome de Marfan et grossesse. A propos de quatre cas.

OBJECTIVES: To create a follow-up protocol for pregnant patients with Marfan syndrome. PATIENTS AND METHODS: We retrospectively reviewed the charts of patients who delivered in the Jeanne de Flandre University Hospital between June 1996 and June 1999. Four pregnant patients with Marfan syndrome were identified. RESULTS: Three of these patients had Bentall procedure. One of them had vaginal delivery and the two others underwent cesarean section. One of these two patients developed aortic valve thrombus at 14 weeks of amenorrhea. The fourth patient did not have surgery and had two vaginal deliveries. DISCUSSION: According to our results and after reviewing literature pregnant patients with Marfan syndrome were divided into two groups. The 1st group was comprised of patients who underwent Bentall procedure. The 2nd one was comprised of patients who did not undergo any surgical procedure. The possibility of vaginal delivery for patients who underwent Bentall procedure (one case) and the interest of Propanolol and anticoagulant treatment are emphasized. CONCLUSION: The multivariant approach of pregnant patients with Marfan syndrome is stressed out with special reference to the potential complications of this syndrome such as aortic dissection and to the problems related to the anticoagulant treatment.