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Neurological Sciences : Official... May 2019Posterior reversible encephalopathy syndrome (PRES) is an encephalopathy characterized by a rapid onset of symptoms including headache, seizures, confusion, blurred...
Posterior reversible encephalopathy syndrome (PRES) is an encephalopathy characterized by a rapid onset of symptoms including headache, seizures, confusion, blurred vision, and nausea associated with a typical magnetic resonance imaging appearance of reversible subcortical vasogenic edema prominent and not exclusive of parieto-occipital lobes. Vasogenic edema is caused by a blood-brain barrier leak induced by endothelial damage or a severe arterial hypertension exceeding the limits of cerebral blood flow autoregulation. Although the exact pathophysiological mechanism is still unclear, frequent conditions that may induce PRES include severe hypertension, eclampsia/pre-eclampsia, acute kidney diseases and failure, immunosuppressive therapy, solid organ, or bone marrow transplantation. Conversely to other conditions, which may induce PRES, the link between severe infection or sepsis and PRES, often associated with gram-positive bacteria, is still poorly understood and less well known. Clinicians from multiple disciplines, such as neurologists and internists, may encounter during their profession patients with severe infection or sepsis and should consider the possible association between PRES and these conditions. We systematically reviewed the literature about this association in order to provide a helpful clinical insight of such complex pathophysiological mechanism, highlighting the importance of recognizing PRES in such a complex clinical scenario.
Topics: Humans; Infections; Posterior Leukoencephalopathy Syndrome
PubMed: 30604335
DOI: 10.1007/s10072-018-3651-4 -
Journal of Neurosurgery Oct 2018Intracranial giant cell tumors (GCTs) are extremely rare neoplasms with dismal survival and recurrence rates. The authors aimed to confirm independent adverse factors...
OBJECTIVE
Intracranial giant cell tumors (GCTs) are extremely rare neoplasms with dismal survival and recurrence rates. The authors aimed to confirm independent adverse factors for progression-free survival (PFS) and to propose an optimal treatment algorithm.
METHODS
The authors reviewed the clinical data of 43 cases of intracranial GCTs in their series. They also reviewed 90 cases of previously reported GCTs in the English language between 1982 and 2017 using Ovid MEDLINE, Embase, PubMed, and Cochrane databases with keywords of "giant cell tumor" or "osteoclastoma" and "skull," "skull base," "temporal," "frontal," "sphenoid," or "occipital." These prior publication data were processed and used according to PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Aforementioned risk factors for the authors' series and the pooled cases were evaluated in patients not lost to follow-up (m = 38 and n = 128, respectively).
RESULTS
The authors' cohort included 28 males and 15 females with a mean age of 30.5 years. Gross-total resection (GTR) was achieved in 15 (34.9%) patients. Fifteen patients (39.5%) who did not undergo GTR received postoperative radiotherapy with a mean total dose of 54.7 ± 4.1 Gy. After a mean follow-up of 71.3 months, 12 (31.6%) patients experienced recurrence, and 4 (10.5%) died of disease. The actuarial 5-year PFS and overall survival (OS) were 68.6% and 90.0% in the authors' cohort, respectively. A multivariate Cox regression analysis verified that partial resection (HR 7.909, 95% CI 2.296-27.247, p = 0.001), no radiotherapy (HR 0.114, 95% CI 0.023-0.568, p = 0.008), and Ki-67 ≥ 10% (HR 7.816, 95% CI 1.584-38.575, p = 0.012) were independent adverse factors for PFS. Among the 90 cases in the literature, GTR was achieved in 49 (54.4%) cases. Radiotherapy was administered to 33 (36.7%) patients with a mean total dose of 47.1 ± 5.6 Gy. After a mean follow-up of 31.5 months, recurrence and death occurred in 17 (18.9%) and 5 (5.6%) cases, respectively. Among the pooled cases, the 5-year PFS and OS were 69.6% and 89.2%, respectively. A multivariate model demonstrated that partial resection (HR 4.792, 95% CI 2.909-7.893, p < 0.001) and no radiotherapy (HR 0.165, 95% CI 0.065-0.423, p < 0.001) were independent adverse factors for poor PFS.
CONCLUSIONS
GTR and radiotherapy were independent favorable factors for PFS of intracranial GCTs. Based on these findings, GTR alone or GTR plus radiotherapy was advocated as an optimal treatment; otherwise, partial resection plus radiotherapy with a dose ≥ 45 Gy, if tolerable, was a secondary alternative. Lack of randomized data of the study was stressed, and future studies with larger cohorts are necessary to verify these findings.Systematic review no.: CRD42018090878 (crd.york.ac.uk/PROSPERO/).
Topics: Adult; Algorithms; Brain Neoplasms; Disease-Free Survival; Female; Giant Cell Tumors; Humans; Male; Progression-Free Survival; Retrospective Studies; Risk Factors; Young Adult
PubMed: 30497189
DOI: 10.3171/2018.4.JNS1849 -
Clinical Neurology and Neurosurgery Oct 2018The objectives of this review are to determine the level of evidence for the management of OCF, compare outcomes of different immobilisation, and to review the prognosis.
OBJECTIVES
The objectives of this review are to determine the level of evidence for the management of OCF, compare outcomes of different immobilisation, and to review the prognosis.
PATIENTS AND METHODS
A literature search was conducted using 3 databases (MEDLINE, PubMed and EMBASE). All papers between 1940 and July 2017 were screened using PRISMA guidelines. Inclusion criteria were patients with a confirmed diagnosis of occipital condyle fracture(s) on CT managed with any form of immobilisation with no age restriction. Primary outcome was clinical improvement in symptoms or Neck Disability Index. MINORS and OCEBM level was assigned to each study.
RESULTS
25 studies met the inclusion criteria. Most studies used a single form of C-spine immobilisation support (58%) with a semi rigid collar and halo device being the most common. From these studies, the average length of time for immobilisation was 11.7 weeks, 9 weeks and 8.3 weeks for halo, semi-rigid and rigid cervical collars respectively. Neuro deficit was found in 20.3% of patients. OCEBM level of evidence and MINORS score was low.
CONCLUSION
Management of OCF is associated with low level of evidence. Further studies are needed to determine optimal management of these under-diagnosed fractures.
Topics: Humans; Occipital Bone; Orthotic Devices; Prognosis; Skull Fractures; Spinal Fractures; Spine
PubMed: 30125835
DOI: 10.1016/j.clineuro.2018.08.013 -
American Journal of Medical Genetics.... Oct 2018The 22q11.2 Deletion Syndrome (22q11.2DS) is the most common microdeletion syndrome with an estimated prevalence of 1:4,000 live births. 22q11.2DS is known to have wide...
The 22q11.2 Deletion Syndrome (22q11.2DS) is the most common microdeletion syndrome with an estimated prevalence of 1:4,000 live births. 22q11.2DS is known to have wide phenotypic variability, including orthopaedic manifestations. The purpose of this systematic review is to increase the awareness of orthopaedic manifestations associated with 22q11.2DS. This systematic review was performed according to the PRISMA Guidelines. Original epidemiological studies on the prevalence of orthopaedic manifestations within 22q11.2DS were systematically searched for in PubMed and EMBASE. The included articles were scored according to a risk-of-bias tool, a best-evidence synthesis was performed and the prevalence data was extracted. Sixty-nine published manuscripts described 58 orthopaedic manifestations in a total of 6,055 patients. The prevalence of at least one cervical or occipital anomaly is 90.5-100% (strong evidence). Fourteen studies (n = 2,264) revealed moderate evidence for a wide scoliosis prevalence of 0.6-60%. Two studies demonstrated that 5-6.4% of all 22q11.2DS patients required surgical scoliosis correction. Fifteen studies (n = 2,115) reported a 1.1-13.3% prevalence of clubfoot with moderate evidence. Other reported orthopaedic manifestations are patellar dislocation (10-20%), juvenile rheumatic arthritis (3.75%), impaired growth and skeletal anomalies like polydactyly (1.0-3.7%), syndactyly (11-11.8%), butterfly vertebrae (11.1%) and 13 ribs (2-19%). Orthopaedic findings are important manifestations of the 22q11.2DS, both in bringing patients to diagnostic attention and in requiring surveillance and appropriate intervention. Data on these manifestations are scattered and incomprehensive. Routinely screening for cervical anomalies, scoliosis, and upper and lower limb malformations is recommended in this vulnerable group of patients.
Topics: Awareness; Bone Diseases; DiGeorge Syndrome; Humans; Prevalence
PubMed: 29159873
DOI: 10.1002/ajmg.a.38545 -
Neurosurgical Review Jul 2018Postoperative occipital neuralgia (PON) after upper cervical spine surgery can cause significant morbidity and may be overlooked. The causes, presentation, diagnosis,... (Meta-Analysis)
Meta-Analysis
Postoperative occipital neuralgia (PON) after upper cervical spine surgery can cause significant morbidity and may be overlooked. The causes, presentation, diagnosis, management, prognosis, and prevention of PON were reviewed following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. English-language studies and case reports published from inception to 2017 were retrieved. Data on surgical procedures, incidence, cause of PON, management, outcomes, and preventive technique were extracted. Sixteen articles, including 591 patients, were selected; 93% of the patients with PON underwent C1 lateral mass screw (C1LMS) fixation, with additional 7% who underwent occipitocervical fusion without C1 fixation. PON had an incidence that ranged from 1 to 35% and was transient in 34%, but persistent in 66%. Five articles explained the possible causes. The primary presentation was constant or paroxysmal burning pain located mainly in the occipital and upper neck area and partially extending to the vertical, retroauricular, retromandibular, and forehead zone. Treatment included medications, nerve block, revision surgery, and nerve stimulation. Two prospective studies compared the effect of C2 nerve root transection on PON. PON in upper cervical spine surgery is a debilitating complication and was most commonly encountered by patients undergoing C1LMS fixation. The etiology of PON is partially clear, and the pain could be persistent and hard to cure. Reducing the incidence of PON can be realized by improving technique. More high-quality prospective studies are needed to define the effect of C2 nerve root transection on PON.
Topics: Bone Screws; Cervical Vertebrae; Humans; Neuralgia; Neurosurgical Procedures; Pain, Postoperative
PubMed: 29116423
DOI: 10.1007/s10143-017-0923-z -
Spine Sep 2017Systematic review and meta-analysis. (Meta-Analysis)
Meta-Analysis
Does Transection of the C2 Nerve Roots During C1 Lateral Mass Screw Placement for Atlantoaxial Fixation Result in a Superior Outcome?: A Systematic Review of the Literature and Meta-analysis.
STUDY DESIGN
Systematic review and meta-analysis.
OBJECTIVE
To evaluate the clinical and functional outcomes of transection of the C2 roots during C1 lateral mass screw placement for atlantoaxial fixation.
SUMMARY OF BACKGROUND DATA
Transection of the C2 nerve roots has been recommended during atlantoaxial fixation to facilitate C1 lateral mass screw placement and possibly reduce postoperative occipital neuralgia, although this practice remains controversial.
METHODS
We searched MEDLINE, EMBASE, Web of Science, and the Cochrane Library for studies evaluating the outcomes of C1-2 fixation involving sacrifice of the C2 roots. We calculated transformed proportions with 95% confidence intervals (CI) for the outcomes of occipital neuralgia, numbness, bony fusion, and procedural morbidity. For studies comparing C2 transection with nerve sparing surgery, we performed meta-analyses for the outcomes of occipital neuralgia, occipital numbness, blood loss, and operative time.
RESULTS
Eight observational studies (N = 393) met eligibility criteria. The rate of postoperative occipital neuralgia among included studies was 0% to 25%; occipital numbness, 6.7% to100%; bony fusion, 96.7% to 100%; and procedural morbidity, 0% to 14.3%. Among comparative studies, C2 transection was associated with a higher rate of occipital numbness [odds ratio (OR) 178.6 (95% CI 26.6 to 1198.4)], lower blood loss [mean difference (MD) -195.3 mL (95% CI -317.7 to -72.8 mL)] and shorter operative times [MD -57.5 mins (95% CI -76.9 to -38.2 mins)] than when the C2 roots were spared. We found no difference in rates of occipital neuralgia [OR 1.44 (95% CI 0.45 to 4.68)].
CONCLUSION
Transection of the C2 nerve roots appears to be a viable, safe option when undertaking placement of C1 lateral mass screws. The procedure is associated with reduced operative duration and blood loss, increased rate of occipital numbness, and no change in the rate of occipital neuralgia. However, given the relatively low quality of evidence, prospective, controlled studies to evaluate this strategy are recommended.
LEVEL OF EVIDENCE
N /A.
Topics: Bone Screws; Cervical Vertebrae; Humans; Hypesthesia; Neuralgia; Postoperative Complications; Spinal Fusion; Spinal Nerve Roots; Treatment Outcome
PubMed: 28098742
DOI: 10.1097/BRS.0000000000002069 -
Headache May 2017To perform a literature review of the epidemiology, clinical presentation, diagnostic evaluation, and clinical course of occipital condyle syndrome, including a new case... (Review)
Review
OBJECTIVE
To perform a literature review of the epidemiology, clinical presentation, diagnostic evaluation, and clinical course of occipital condyle syndrome, including a new case report.
BACKGROUND
Occipital condyle syndrome (OCS) is a rare clinical syndrome, consisting of unilateral occipital headache accompanied by ipsilateral hypoglossal palsy. This headache typically radiates to the temporal region, and is triggered by contralateral head rotation. It is usually associated with skull base metastasis, often unrevealed in basic neuroimaging studies. OCS might be the first manifestation of malignancy, and its unfamiliarity can lead to a delay in the diagnosis.
METHODS
We performed a systematic literature review using PubMed and Embase for OCS, along with a new case report.
RESULTS
A total of 35 cases (mean age 59 years, range 25-77), 24 (70%) men, presented typical unilateral headache followed by ipsilateral hypoglossal palsy from 0 to 150 days after headache presentation. In 16 patients (46%), initial neuroimaging studies were normal. OCS was due to skull base metastasis in 32 cases (91%). In 18 patients (51%), OCS was the first symptom of disease.
CONCLUSIONS
OCS represents a warning sign and requires an exhaustive search for underlying neoplasm. An appropriate clinical evaluation can lead to an earlier diagnosis in patients with consistent headache.
Topics: Adult; Aged; Female; Headache; Humans; Hypoglossal Nerve Diseases; Male; Middle Aged; Occipital Bone; Skull Base Neoplasms
PubMed: 28000214
DOI: 10.1111/head.13008 -
World Neurosurgery Dec 2016The current literature regarding cranial giant cell tumor (GCT) management includes scattered case reports and small case series. We present a comprehensive literature... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
The current literature regarding cranial giant cell tumor (GCT) management includes scattered case reports and small case series. We present a comprehensive literature review and meta-analysis on this subject, along with a case report describing our management of a patient with temporal GCT.
METHODS
A systematic literature review of all reports on GCTs of the skull was performed, followed by a meta-analysis examining the effect of radiation and degree of resection on tumor recurrence.
RESULTS
Fifty-nine abstracts published between 1945 and 2015, reporting 110 cases of GCT, were reviewed. After exclusions were applied, 31 reports, covering 67 patients, were selected for meta-analysis. Average patient age was 33.7 years, and the male:female ratio was roughly 1:1. Tumor locations were temporal in 37 patients, sphenoid in 20 patients, occipital in 6 patients, frontal in 2 patients, and the temporomandibular joint in 2 patients. Treatments were surgery plus radiation in 25 patients, surgery alone in 41 patients, and radiation alone in 1 patient. In the 66 patients who underwent surgery, the degree of resection was gross total resection (GTR) in 34 patients, subtotal resection (STR) in 31 patients, and not recorded in 1 patient. The mean follow-up time was 36 months. Recurrence occurred in 8.8% of patients in the GTR group and in 32.3% of those in the STR group (odds ratio, 0.203; 95% confidence interval, 0.033-0.937; P = 0.018). The odds ratio for STR alone compared with STR plus radiation was 14.01 (P = 0.0038).
CONCLUSION
GCTs of the skull commonly affect young adults, with an equal sex distribution, and are most often centered in temporal bone. GTR is associated with the lowest recurrence rate and should be the goal of treatment. If GTR cannot be achieved, the combination of STR and radiation results in a similar recurrence rate. With the advent of denosumab, there is now a role for chemotherapy in the treatment of GCTs.
Topics: Adult; Cranial Fossa, Middle; Frontal Bone; Giant Cell Tumor of Bone; Humans; Magnetic Resonance Imaging; Male; Neoplasm Recurrence, Local; Neoplasm, Residual; Occipital Bone; Odds Ratio; Radiotherapy, Adjuvant; Skull Base Neoplasms; Sphenoid Bone; Temporal Bone; Temporomandibular Joint; Tomography, X-Ray Computed
PubMed: 27269210
DOI: 10.1016/j.wneu.2016.05.086 -
Journal of Neurosurgery. Pediatrics Feb 2016OBJECT Fusion rates are high for children undergoing posterior cervical fusion (PCF) and occipito-cervical fusion (OCF). Autologous bone has been widely used as the...
OBJECT Fusion rates are high for children undergoing posterior cervical fusion (PCF) and occipito-cervical fusion (OCF). Autologous bone has been widely used as the graft material of choice, despite the risk of donor-site morbidity associated with harvesting the bone, possibly because very low fusion rates were reported with posterior allograft cervical fusions in children several decades ago. Higher overall fusion rates using allograft in adults, associated with improvements in internal fixation techniques and the availability of osteoinductive substances such as bone morphogenetic protein (BMP), have led to heightened enthusiasm for the use of bank bone during pediatric PCF. A systematic review was performed to study factors associated with successful bone fusion, including the type of bone graft used. METHODS The authors performed a comprehensive PubMed search of English-language articles pertaining to PCF and OCF in patients less than 18 years old. Of the 561 abstracts selected, 148 articles were reviewed, resulting in 60 articles that had sufficient detail to be included in the analysis. A meta-regression analysis was performed to determine if and how age, fusion technique, levels fused, fusion substrate, BMP use, postoperative bracing, and radiographic fusion criteria were related to the pooled prevalence estimates. A systematic review of the literature was performed according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement. RESULTS A total of 604 patients met the specific inclusion and exclusion criteria. The overall fusion rate was 93%, with a mean age of 9.3 years and mean follow-up of 38.7 months. A total of 539 patients had fusion with autograft (94% fusion rate) and 65 patients with allograft (80% fusion rate). Multivariate meta-regression analysis showed that higher fusion rates were associated with OCF compared with fusions that excluded the occiput (p < 0.001), with the use of autograft instead of allograft (p < 0.001), and with the use of CT to define fusion instead of plain radiography alone. The type of internal fixation, the use of BMP, patient age, and the duration of follow-up were not found to be associated with fusion rates in the multivariate analysis. CONCLUSIONS Fusion rates for PCF are high, with higher rates of fusion seen when autograft is used as the bone substrate and when the occiput is included in the fusion construct. Further study of the use of allograft as a viable alternative to autograft bone fusion is warranted because limited data are available regarding the use of allograft in combination with more rigid internal fixation techniques and osteoinductive substances, both of which may enhance fusion rates with allograft.
PubMed: 26496632
DOI: 10.3171/2015.6.PEDS1562 -
The Journal of Maternal-fetal &... 2016To conduct a literature review to assess the effectiveness of first trimester ultrasonographic markers of spina bifida (SB) integrating data with our prospective... (Review)
Review
OBJECTIVE
To conduct a literature review to assess the effectiveness of first trimester ultrasonographic markers of spina bifida (SB) integrating data with our prospective experience.
METHODS
The analysis of the SB cases that we prospectively detected in the first trimester, between January 2012 and February 2014, and a systematic review of all the papers evaluating the effectiveness of SB ultrasonographic markers at 11-14 weeks, namely brain stem diameter (BS), fourth ventricle/intracranial translucency (IT), cisterna magna (CM), brain stem/occipital bone distance (BSOB), the ratio between BS and BSOB. Some studies assess only the effectiveness of IT, others include more parameters, and few include them all.
RESULTS
We prospectively detected four SB cases, three open (OSB) and one closed (CSB), in a low risk population undergoing first trimester screening. In the three OSB, CM (in 2/3 cases) and BSOB (3/3) distance were below the 5th percentile, BS and BS/BSOB ratio (3/3) were above the 95th percentile. In the CSB, all the measurements were within normal limits.
CONCLUSION
The literature and our data agree that most fetuses with OSB demonstrate in the first trimester positive sonographic markers in the posterior fossa, but additional prospective studies are needed to establish the best protocol for OSB screening.
Topics: Adult; Brain Stem; Cisterna Magna; Female; Fourth Ventricle; Humans; Occipital Bone; Pregnancy; Pregnancy Trimester, First; Prospective Studies; Spinal Dysraphism; Ultrasonography, Prenatal
PubMed: 26381234
DOI: 10.3109/14767058.2015.1085967