NF2 and Spinal Tumors

2006 ◽  
Vol 5 (2) ◽  
pp. 179 ◽  
Author(s):  
Dilys M. Parry

Object The authors conducted a study to examine the incidence, classification, and progression of spinal tumors in patients with neu-rofibromatosis Type 2 (NF2) treated at a single center, and to examine relationships with the known mutational subtypes of NF2. Methods They performed a retrospective review of clinical records, neuroimaging studies, and genetic data obtained in 61 patients with NF2. Forty-one (67%) of 61 patients harbored one or more spinal tumors. Thirty-four patients had undergone serial spinal magnetic resonance imaging during a mean follow-up period of 52 months (range 10–103 months; median 53 months). In 16 patients there were multiple extramedullary tumors smaller than 5 mm, which did not progress. Fourteen patients harbored at least one extramedullary tumor that was greater than 5 mm; of these, radiological progression was demonstrated or spinal tumor excision was performed during the follow-up period in eight cases (57%). Eleven patients harbored intramedullary cord tumors in addition to small and large extramedullary tumors, three (27%) of which exhibited radiological progression. In cases in which genotypes were known, protein-truncating mutations were significantly more likely to be associated with the presence of spinal tumors than in other types (p = 0.03, Fisher exact test). No associations between clinical behavior of spinal tumors and genotype, however, could be demonstrated. Conclusions Spinal tumors in cases involving NF2 are heterogeneous in type, distribution, and behavior but larger-size tumors are more likely to progress significantly. Intramedullary tumors usually accompany multiple extramedullary tumors. In the authors' experience subtyping of the NF2 mutation has not yet influenced management. Protein-truncating mutations are associated with an increased prevalence of spinal tumors.

2005 ◽  
Vol 2 (5) ◽  
pp. 574-579 ◽  
Author(s):  
Graham Dow ◽  
Nigel Biggs ◽  
Gareth Evans ◽  
Jimmie Gillespie ◽  
Richard Ramsden ◽  
...  

Object. The authors conducted a study to examine the incidence, classification, and progression of spinal tumors in patients with neurofibromatosis Type 2 (NF2) treated at a single center, and to examine relationships with the known mutational subtypes of NF2. Methods. They performed a retrospective review of clinical records, neuroimaging studies, and genetic data obtained in 61 patients with NF2. Forty-one (67%) of 61 patients harbored one or more spinal tumors. Thirty-four patients had undergone serial spinal magnetic resonance imaging during a mean follow-up period of 52 months (range 10–103 months; median 53 months). In 16 patients there were multiple extramedullary tumors smaller than 5 mm, which did not progress. Fourteen patients harbored at least one extramedullary tumor that was greater than 5 mm; of these, radiological progression was demonstrated or spinal tumor excision was performed during the follow-up period in eight cases (57%). Eleven patients harbored intramedullary cord tumors in addition to small and large extramedullary tumors, three (27%) of which exhibited radiological progression. In cases in which genotypes were known, protein-truncating mutations were significantly more likely to be associated with the presence of spinal tumors than in other types (p = 0.03, Fisher exact test). No associations between clinical behavior of spinal tumors and genotype, however, could be demonstrated. Conclusions. Spinal tumors in cases involving NF2 are heterogeneous in type, distribution, and behavior but larger-size tumors are more likely to progress significantly. Intramedullary tumors usually accompany multiple extramedullary tumors. In the authors' experience subtyping of the NF2 mutation has not yet influenced management. Protein-truncating mutations are associated with an increased prevalence of spinal tumors.


2021 ◽  
Vol 9 ◽  
Author(s):  
Antonella Savio ◽  
Stefano Calza ◽  
Gianbattista Guerrini ◽  
Valentina Romano ◽  
Eleonora Marchina

Background: COVID-19 outbursts have been registered worldwide within care homes with asymptomatic transmission combined with shortage/inaccuracy of diagnostic tests undermining the efforts at containment of the disease. Nursing facilities in Lombardy (Italy) were left with no, or limited, access to testing for 8 weeks after the outbreak of COVID-19.Methods: This study includes 246 residents and 286 workers of three different nursing homes in Brescia-Lombardy. Clinical questionnaires and rapid serology tests were devised to integrate the data of the first available RT-PCR screening. Follow-up serology after 60-days was performed on 67 of 86 workers with positive serology or clinically suspicious.Findings: Thirty-seven residents and 18 workers had previous positive RT-PCR. Thorough screening disclosed two additional RT-PCR-positive workers. Serology screening revealed antibodies in 59 residents and 48 workers, including 32/37 residents and all workers previously positive at RT-PCR. Follow up serology disclosed antibodies in two additional workers with recent symptoms at the time of screening. The professionals in close contact with residents had more infections (47/226–20.79% vs. 1/60–1.66%; p = 0.00013 Fisher exact-test). A suspicious clinical score was present in 44/64 residents and in 41/50 workers who tested positive with either method with totally asymptomatic disease more frequent among residents 28.1 vs. 10.0% (p = 0.019 Fisher exact-test).Interpretation: Based on the available RT-PCR ± results at the time of symptoms/contacts, our integrated clinical and serological screening demonstrated sensitivity 89% and specificity 87%. This multimodal assessment proved extremely useful in understanding the viral spread in nursing homes, in defining its stage and in implementing protective measures. Rapid serology tests demonstrated efficient and particularly suited for older people less able to move/cooperate.


Neurosurgery ◽  
2006 ◽  
Vol 58 (5) ◽  
pp. 891-898 ◽  
Author(s):  
Jack P. Rock ◽  
Samuel Ryu ◽  
Mohammad S. Shukairy ◽  
Fang-Fang Yin ◽  
Aktham Sharif ◽  
...  

Abstract OBJECTIVE: Although, as a primary therapy, radiosurgery for spinal tumors is becoming more common in clinical practice and is associated with encouraging clinical results, we wanted to evaluate outcomes after radiosurgery in a series of postoperative patients. METHODS: We examined the medical records of 18 postoperative patients who received radiosurgical treatment to their residual spinal tumors: metastatic carcinoma (10), sarcoma (3), multiple myeloma/plasmacytoma (4), and giant cell tumor (1). Marginal radiosurgical doses ranged from 6 to 16 Gy (mean, 11.4 Gy) prescribed to the 90% isodose line. All regions of the spine received treatment: 2 cervical, 15 thoracic, and 1 lumbosacral. The volume of irradiated spinal elements receiving 30, 50, and 80% of the total dose ranged from 0.51 to 11.05, 0.19 to 6.34, and 0.06 to 1.73 cm3, respectively. Treatment sessions (i.e., patient in to patient out of the room) varied between 20 and 40 minutes. Follow-up ranged from 4 to 36 months (median, 7 mo). RESULTS: Even though significant doses of radiation were delivered to all regions of the spinal cord and nerve roots coincidentally involved in the treatments, only one patient in this series developed progressive symptoms possibly attributable to a toxic effect of the radiosurgery. Of those patients initially presenting with neurological deficits, 92% either remained neurologically stable or improved. CONCLUSION: Our observations suggest that radiosurgery as prescribed in this series of postoperative patients with residual spinal tumor is well-tolerated and associated with little to no significant morbidity.


2016 ◽  
Vol 76 (4) ◽  
pp. 666-672 ◽  
Author(s):  
Mads Ammitzbøll-Danielsen ◽  
Mikkel Østergaard ◽  
Viktoria Fana ◽  
Daniel Glinatsi ◽  
Uffe Møller Døhn ◽  
...  

ObjectiveThe aim of this study was to compare the efficacy of intramuscular versus ultrasound (US)-guided intratenosynovial glucocorticoid injection in providing disease control after 2, 4 and 12 weeks in patients with rheumatoid arthritis(RA) with tenosynovitis.MethodsFifty patients with RA and tenosynovitis were randomised into two double-blind groups: (A) ‘intramuscular group’, receiving intramuscular injection of betamethasone and US-guided intratenosynovial isotonic saline injection and (B) ‘intratenosynovial group’ receiving saline intramuscularly and US-guided intratenosynovial betamethasone injection. All patients were in stable disease-modifying anti-rheumatic drug treatment prior to and during the study. Patients were excluded, and considered non-responders, if any treatments were altered during the follow-up period. ‘US tenosynovitis remission’, defined as US tenosynovitis grey-scale score ≤1 and colour Doppler score=0, was assessed at week 4 (primary outcome), and weeks 2 and 12, using non-responder imputation for missing data.ResultsUS tenosynovitis remission at week 4 was achieved in 25% (6/24) in the ‘intramuscular group’ versus 64% (16/25) in the ‘intratenosynovial group’, that is, a difference of −39 percentage point (pp) (CI −65pp to −13pp), Fisher exact test p=0.001. Corresponding values for the ‘intramuscular group’ versus the ‘intratenosynovial group’ at 2 and 12 weeks were 21% (5/24) versus 48% (13/25), that is, a difference of −27pp (CI −53pp to −2pp), p=0.072 and 8% (2/24) versus 44% (11/25), that is, difference of −36pp (−58pp to −13pp), p=0.003. Most US, clinical and patient-reported scores improved more in the ‘intratenosynovial group’ at all follow-up visits.ConclusionsIn this randomised double-blind clinical trial, patients with RA and tenosynovitis responded significantly better to US-guided intratenosynovial glucocorticoid injection than to intramuscular glucocorticoid injection, both at 4 and 12 weeks follow-up.Trial registration numberEudraCT nr: 2013-003486-34.


2014 ◽  
pp. 33-38
Author(s):  
Gabriel Braga Diégues Serva ◽  
Leonardo Santos Calvacanti Guerra ◽  
Vilneide Maria Santos Braga Diégues Serva ◽  
Waldmiro Antônio Diégues Serva ◽  
Marcela Patrícia Macêdo Belo ◽  
...  

Objectives: To identify if the presence of migraine before pregnancy predisposes to hypertensive disorders of pregnancy. Methods: Observational study undertaken from a database of a follow-up study, composed of women consecutively assisted, at the first postnatal week, at IMIP. Its objective was to evaluate the course of migraine during pregnancy and postpartum in women with migraine before pregnancy. The Fisher exact test was used considering the significance level of less than 5%. Results: Of the 686 women, 38.8% were migraine sufferers before pregnancy. 14.3% referred hypertensive disorders of pregnancy. The presence of migraine before pregnancy and to have been submitted to a cesarean section (p<0.001) were factors associated with the presence of hypertensive disorders in pregnancy. Conclusion: Migraine before pregnancy is an associated factor to hypertensive disorders of pregnancy. The diagnosis of migraine should always be taken into consideration during antenatal care, for the prevention of complications.


2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 10620-10620
Author(s):  
M. Castiel ◽  
S. Masakhalia ◽  
M. Krychman ◽  
B. Stier ◽  
A. Amsterdam ◽  
...  

10620 Background: To determine if differences exist in the use of minimally absorbed vaginal estrogens in a breast cancer population versus one with non-breast malignancies. Methods: A retrospective review of all patients with documented malignancy who were prescribed vaginal 17-β-estradiol tablets (Vagifem, Novo-Disc, Princeton, New Jersey) through the General Gynecology Service from July 1, 2003 to June 30, 2004. Data were accessed from the pharmacy health information computerized system. All patients complained of dyspareunia, and examination was consistent with vaginal atrophy. Comparison between groups was performed using the Fisher Exact Test and t-test. Results: 152 patients were identified_81 patients with breast cancer diagnoses and 71 with non-breast malignancies. There was no difference in the mean age of patients in either group, 56.9 vs. 55.1 years. (p = 0.30). Overall, 60 of the 152 patients refilled their prescriptions through the time of last follow-up. There was no difference in the proportion of breast versus non-breast cancer patients who continued vaginal estradiol, 39.5% (32/81) versus 39.4% (28/71) respectively. When the cohorts were stratified by age ≤ 50 or >50 at the time of initial consultation, 79% (11/14) of breast cancer patients age ≤ 50 years refilled their prescriptions at follow-up compared to 31.3% (21/67) of patients >50 (p = 0.002). In women with non-breast malignancies there was no age-related difference in the proportion who obtained refills: 35% (7/20) ≤ 50 years versus 41.2 % (21/51) >50 years (p = 0.79). Conclusions: Minimally absorbed vaginal estrogens appear to be viewed favorably amongst the younger breast cancer patient population. The older breast cancer population does not appear to continue vaginal estradiol use long-term. This may be due to differences in relationship status, interval from menopause to diagnosis, prior exposure to estrogen, or relief of symptoms with alternative therapies. These findings are different than those observed for similar patients with non-breast malignancies. Further studies looking at the use of minimally absorbed vaginal estrogens are needed to further evaluate the usage trends in differing patient populations. No significant financial relationships to disclose.


Neurosurgery ◽  
2012 ◽  
Vol 71 (5) ◽  
pp. 1003-1011 ◽  
Author(s):  
Michael R. Folkert ◽  
Mark H. Bilsky ◽  
Gil'ad N. Cohen ◽  
Marco Zaider ◽  
Lawrence T. Dauer ◽  
...  

Abstract BACKGROUND: Treatment of spinal and intracranial tumors with dural involvement is complicated by radiation tolerance of sensitive structures, especially in the setting of previous treatment. OBJECTIVE: To evaluate whether intraoperative brachytherapy with short-range sources allows therapeutic dose delivery without damaging sensitive structures. METHODS: The median doses of previous treatment were 3000 cGy (range, 1800-7200 cGy) for 8 patients with primary/recurrent and 17 patients with metastatic spinal tumors and 5040 cGy (range, 1300-6040 cGy) for 5 patients with locally recurrent and 2 patients with metastatic intracranial tumors. Patients underwent gross total or maximal resection of the tumor and were then treated with an intraoperative brachytherapy plaque consisting of a flexible silicone film incorporating 32P. A dose of 1000 cGy was delivered to a depth of 1 mm; the percent depth dose was less than 1% at 4 mm from the prescription depth. Median postoperative radiation doses of 2700 cGy (range, 1800-3000 cGy) were delivered to 15 spinal tumor patients and 3000 cGy (range, 1800-3000 cGy) to 3 intracranial tumor patients. The median follow-up was 4.4 months (range, 2.6-23.3 months) for spinal tumor patients and 5.3 months (range, 0.7-16.2) for intracranial tumor patients. RESULTS: At 6-month follow-up, for all spinal tumor patients, local progression-free survival and overall survival rates were both 83.3% (95% confidence interval [CI]: 62.3%-94.3%); for all intracranial tumor patients, the local progression-free survival rate was 62.5% (95% CI: 23.8%-90.9%) and the overall survival rate was 66.7% (95% CI: 26.7%-92.9%). There were no intraoperative or postoperative complications secondary to radiotherapy. CONCLUSION: Use of the 32P brachytherapy plaque is technically simple and not associated with increased risk of complications, even after multiple radiation courses. Local control rates were more than 80% in patients with proven radiation-resistant spinal disease.


2014 ◽  
Vol 120 (6) ◽  
pp. 1437-1445 ◽  
Author(s):  
Paul M. Foreman ◽  
Christoph J. Griessenauer ◽  
Michael Falola ◽  
Mark R. Harrigan

Object Traumatic aneurysms occur in 10% of extracranial blunt traumatic cerebrovascular injuries (TCVI). The clinical consequences and optimal management of traumatic aneurysms are poorly understood. Methods A prospective study of TCVI at a Level I trauma center identified 7 patients with 19 extracranial traumatic carotid artery or vertebral artery aneurysms. An additional 6 patients with 7 traumatic aneurysms were followed outside of the prospective study, giving a total of 13 patients with 26 traumatic aneurysms. All patients were treated with 325 mg aspirin daily and underwent clinical and imaging follow-up beyond the initial hospitalization. Endovascular treatment was reserved for aneurysms demonstrating significant enlargement on follow-up imaging. Clinical and radiographic features were assessed. Results The 7 patients with traumatic aneurysms identified in the prospective cohort comprised 10.3% of all patients with TCVI. Two (15.4%) of the 13 total patients suffered an ischemic stroke in the setting of TCVI with traumatic aneurysm formation. No patient experienced an ischemic stroke or new symptoms after the initiation of antiplatelet therapy. Clinical and radiographic follow-up averaged 15.8 months (range 0.4–41.7 months) and 22.0 months (range 6.6–55.7 months), respectively. Ten (38.5%) of 26 aneurysms were not visualized on last follow-up, 10 (38.5%) were smaller, 1 (3.8%) was unchanged, and 5 (19.2%) were larger. Saccular aneurysms were more likely to enlarge than fusiform aneurysms (33.3% vs 11.8%). Results of a Fisher exact test tend to support the assertion that the 2 different aneurysm morphologies behave differently (p = 0.07). Two saccular aneurysms were treated with stenting. Conclusions The majority of traumatic aneurysms can be managed with an antiplatelet regimen of 325 mg aspirin daily and serial imaging. Saccular aneurysms have a greater tendency to enlarge when compared with fusiform aneurysms.


2016 ◽  
Vol 25 (5) ◽  
pp. 640-645 ◽  
Author(s):  
Matthew J. Viereck ◽  
George M. Ghobrial ◽  
Sara Beygi ◽  
James S. Harrop

OBJECTIVE Resection significantly improves the clinical symptoms and functional outcomes of patients with intradural extramedullary tumors. However, patient quality of life following resection has not been adequately investigated. The aim in this retrospective analysis of prospectively collected quality of life outcomes is to analyze the efficacy of resection of intradural extramedullary spinal tumors in terms of quality of life markers. METHODS A retrospective review of a single institutional neurosurgical administrative database was conducted to analyze clinical data. The Oswestry Disability Index (ODI), visual analog scale (VAS) for pain, and the EQ-5D-3 L descriptive system were used to analyze quality of life preoperatively, less than 1 month postoperatively, 1–3 months postoperatively, 3–12 months postoperatively, and more than 12 months postoperatively. RESULTS The ODI scores increased perioperatively at the < 1-month follow-up from 36 preoperatively to 47. Relative to preoperative values, the ODI score decreased significantly at 1–3, 3–12, and > 12 months to 23, 17, and 20, respectively. VAS scores significantly decreased from 6.1 to 3.5, 2.4, 2.0, and 2.9 at the < 1-month, 1- to 3-, 3- to 12-, and > 12-month follow-ups, respectively. EQ-5D mobility significantly worsened at the < 1-month follow-up but improved at the 3- to 12-and > 12-month follow-ups. EQ-5D self-care significantly worsened at the < 1-month follow-up but significantly improved by the 3- to 12-month follow-up. EQ-5D usual activities improved at the 1- to 3-, 3- to 12-, and > 12-month follow-ups. EQ-5D pain and discomfort significantly improved at all follow-up points. EQ-5D anxiety and depression significantly improved at 1- to 3-month and 3- to 12-month follow-ups. CONCLUSIONS Resection of intradural extramedullary spine tumors appears to significantly improve patient quality of life by decreasing patient disability and pain and by improving each of the EQ-5D domains.


Animals ◽  
2021 ◽  
Vol 11 (8) ◽  
pp. 2229
Author(s):  
Adam B. O’Connell ◽  
A. Craig Irving ◽  
Paul L. Hughes ◽  
Naomi Cogger ◽  
Boyd R. Jones ◽  
...  

A study in conducted 1987 by Hughes et al., found that 39% of working sheep dogs had multifocal retinitis. One of the identified causes was ocular larval migrans, which were a result of migrating ascarid larvae. Since that paper was published, anthelmintic use in farm dogs has been highly recommended. There has been no follow-up study to determine if fundic lesions are still present. The current study aimed to investigate the prevalence of chorioretinopathy in working sheep dogs in the South-West, Waikato, New Zealand. This was a cross-sectional study of 184 working sheep dogs and 51 owners, undertaken in 2010 with owners sampled from New Zealand’s South-West Waikato and Tux North Island Dog Trial Championship. Two-way tables were used to explore the relationship between variables. Significance of association was assessed using a Chi-squared or Fisher exact test as appropriate, with a p-value of <0.05 considered significant. Overall prevalence of chorioretinopathy in the working sheep dogs was 44/184 (24%). A significantly higher prevalence of chorioretinopathy was shown in dogs with increasing age, from 2 years to >8 years (p = 0.0007) and in males (p < 0.0001). This study concluded that lesions of chorioretinopathy are still present in working sheep dogs in New Zealand.


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