A site to transform Pubmed publications into these bibliographic reference formats: ADS, BibTeX, EndNote, ISI used by the Web of Knowledge, RIS, MEDLINE, Microsoft's Word 2007 XML.

Nervous System Diseases - Top 30 Publications

Continuing risk of meningitis due to Neisseria meningitidis serogroup C in Africa: revised recommendations from a WHO expert consultation.

Trial of Tocilizumab in Giant-Cell Arteritis.

Trial of Tocilizumab in Giant-Cell Arteritis.

Trial of Tocilizumab in Giant-Cell Arteritis.

Trial of Tocilizumab in Giant-Cell Arteritis.

Oral morphine versus ibuprofen administered at home for postoperative orthopedic pain in children: a randomized controlled trial.

Oral morphine for postoperative pain after minor pediatric surgery, while increasingly popular, is not supported by evidence. We evaluated whether oral morphine was superior to ibuprofen for at-home management of children's postoperative pain.

Muscle involvement in systemic sclerosis: points to consider in clinical trials.

SSc is clinically and pathogenetically heterogeneous. Consensus standards for trial design and outcome measures are needed. International experts experienced in SSc clinical trial design and a researcher experienced in systematic literature review screened the PubMed and Cochrane Central Register of Controlled Trials in order to develop points to consider when planning a clinical trial for muscle involvement in SSc. The experts conclude that SSc-associated muscle involvement is heterogeneous and lacks a universally accepted gold-standard for measuring therapeutic response. Although outcome studies are currently limited by the inability to clearly distinguish active, reversible muscle inflammation from irreversible muscle damage and extramuscular organ involvement, strong consideration should be given to enrolling patients with a myopathy that features several elements of likely reversibility such as muscle weakness, biopsy-proven active inflammation, an MRI indicating muscle inflammation and a baseline serum creatinine kinase above three times the upper limit of normal to prevent floor effect. Randomized controlled trials are preferred, with a duration of at least 24 weeks. Outcome measures should include a combination of elements that are likely to be reversible, such as muscle weakness, biopsy-proven active inflammation, creatinine kinase/aldolase and a quality of life questionnaire. The individual measurements might require a short pre-study for further validation. A biological sample repository is recommended.

A 63-Year-Old Woman With Neurofibromatosis Type 1 and Pulmonary Hypertension With Worsening Hypoxemia.

A 63-year-old woman with a history of neurofibromatosis type-1 (NF-1) and pulmonary arterial hypertension (PAH) thought to be secondary to the NF-1 presented with a few weeks of worsening dyspnea on exertion. She took no medications other than sildenafil for her pulmonary hypertension (PH). She denied tobacco, alcohol, and illicit or anorectic drug use. She had previously worked as a waitress. Her mother and her brother had NF-1 but no PH or lung disease.

Bowel Dysfunction Related to Spina Bifida: Keep It Simple.

Although care of urological disorders in spina bifida is well established, there is yet no agreement on a standardized approach to bowel dysfunction in this population.

Ischemic Stroke: Advances in Diagnosis and Management.

Acute ischemic stroke carries the risk of morbidity and mortality. Since the advent of intravenous thrombolysis, there have been improvements in stroke care and functional outcomes. Studies of populations once excluded from thrombolysis have begun to elucidate candidates who might benefit and thus should be engaged in the process of shared decision-making. Imaging is evolving to better target the ischemic penumbra salvageable with prompt reperfusion. Availability and use of computed tomography angiography identifies large-vessel occlusions, and new-generation endovascular therapy devices are improving outcomes in these patients. With this progress in stroke treatment, risk stratification tools and shared decision-making are fundamental.

Cerebral Venous Thrombosis: A Challenging Neurologic Diagnosis.

Headache is a common emergency department chief complaint. Although most are benign, emergency physicians must rapidly identify and manage the uncommon, sometimes subtle, presentation of headache from a life-threatening cause. Cerebral venous thrombosis imparts significant morbidity and mortality, and can be a challenging diagnosis. It most commonly occurs in those under 50 years of age with thrombosis of the cerebral veins/sinuses. Diagnosis is frequently delayed. The disease can present with 1 or more clinical syndromes, including intracranial hypertension with headaches, focal neurologic deficits, seizures, and encephalopathy. Diagnosis requires imaging. Treatment includes stabilization, management of complications, and anticoagulation.

Spontaneous Intracerebral Hemorrhage.

Although commonly arising from poorly controlled hypertension, spontaneous intracerebral hemorrhage may occur secondary to several other etiologies. Clinical presentation to the emergency department ranges from headache with vomiting to coma. In addition to managing the ABCs, the crux of emergency management lies in stopping hematoma expansion and other complications to prevent clinical deterioration. This may be achieved primarily through anticoagulation reversal, blood pressure, empiric management of intracranial pressure, and early neurosurgical consultation for posterior fossa hemorrhage. Patients must be admitted to intensive care. The effects of intracerebral hemorrhage are potentially devastating with very poor prognoses for functional outcome and mortality.

Subarachnoid Hemorrhage: Updates in Diagnosis and Management.

Subarachnoid hemorrhage (SAH) is a neurologic emergency due to bleeding into the subarachnoid space. Mortality can reach 50%. The clinical presentation is most often in the form of headache, classically defined as maximal at onset and worst of life. The most common cause is traumatic; approximately 80% of nontraumatic SAH are due to aneurysmal rupture, with the remainder from idiopathic peri-mesencephalic hemorrhage or other less common causes. Noncontrast brain computed tomography (CT) performed within 6 hours of symptom onset has sensitivity approaching 100%. Lumbar puncture may be considered after this period for definitive diagnosis if initial CT is normal.

Extracranial Cervical Artery Dissections.

Cervical artery dissections (CeAD) include both internal carotid and vertebral artery dissections. They are rare but important causes of stroke, especially in younger patients. CeAD should be considered in patients with strokelike symptoms, a new-onset headache and/or neck pain, and/or other risk factors. Early imaging with computed tomography (CT) or magnetic resonance imaging (MRI) is key to making the diagnosis. Treatment may vary depending on the extent of the dissection, timing of the dissection, and other comorbidities. The overall prognosis is good, but does depend on the initial severity of symptoms.

Nutritional optic neuropathy instead of tobacco-alcohol amblyopia.

A novel compound mutation in GLRA1 cause hyperekplexia in a Chinese boy- a case report and review of the literature.

The pathogenesis of hereditary hyperekplexia is thought to involve abnormalities in the glycinergic neurotransmission system, the most of mutations reported in GLRA1. This gene encodes the glycine receptor α1 subunit, which has an extracellular domain (ECD) and a transmembrane domain (TMD) with 4 α-helices (TM1-TM4).

Gender differences in the prevalence of congenital heart disease in Down's syndrome: a brief meta-analysis.

Down's syndrome (DS) affects one per 700 live births and congenital heart disease (CHD) occurs in 40-60% of these patients. Contributing factors to the association between DS and CHD are being unraveled. Gender could be one of them.

Severe hypokalemic paralysis and rhabdomyolysis occurring after binge eating in a young bodybuilder: Case report.

Severe hypokalemia can be a potentially life-threatening disorder and is associated with variable degrees of skeletal muscle weakness.

Chiari type I malformation with occult tethered cord syndrome in a child: A case report.

Chiari type I malformation (CM1) and occult tethered cord syndrome (OTCS) are considered to be malformations associated with subtle structural abnormalities of the terminal filum. Few studies have reported patients with CM1 and OTCS. Treatment strategy for patients of CM1 associated with OTCS is controversial.

Anti-Ma2-associated limbic encephalitis with coexisting chronic inflammatory demyelinating polyneuropathy in a patient with non-Hodgkin lymphoma: A case report.

We report the rare case of a 74-year-old man with anti-Ma2-associated paraneoplastic neurologic syndrome (PNS), and review and analyze the clinical manifestations, diagnosis, and treatment of the disease.

Conservative treatment for equinus deformity in children with cerebral palsy using an adjustable splint-assisted ankle-foot orthosis.

A novel splint, the assisting ankle-foot orthoses (AFO), was developed to provide adjustable sustained stretching to improve conservative treatment for equinus deformities in children with cerebral palsy (CP). The treatment effect was validated by follow-up visits.

Expression of matrix metalloproteinases-12 in ST-segment elevation myocardial infarction: A case-control study.

Matrix metalloproteinases-12 (MMP12) can lead to degradation of elastin resulting in plaque destabilization and rupture. MMP12 also facilitates platelet aggregation, adhesion, and granule secretion. However, evidence in the literature related to the function of MMP12 in ST-segment elevation myocardial infarction (STEMI) is little. This study investigated the expression of MMP12 in human coronary thrombus and examined the relationship between plasma MMP12 and STEMI.Arterial plasma was obtained from 46 STEMI patients and 52 stable angina pectoris (SAP) patients and 30 controls with angiographically normal coronary arteries. Coronary thrombi were obtained from 26 STEMI patients with a large thrombus burden (LTB). The expression levels of MMP12 in coronary thrombus were analyzed by immunohistochemistry and immunofluorescence, reverse transcription-polymerase chain reaction (RT-PCR), Western blotting (WB) and casein zymography. In addition, MMP12 concentration measured by enzyme-linked immunosorbent assay (ELISA) and activity measured by fluorescence resonance energy transfer (FRET) were used to assess the levels in plasma.We confirmed the expression of MMP12 in human coronary thrombus. MMP12 was secreted mainly in active form of 45 kDa in coronary thrombus. In plasma samples of the STEMI group, MMP12 concentrations were found to be higher than the SAP group (5.030 ± 2.24 pg/mL vs 3.010 ± 1.99 pg/mL, P < .05) but with lower MMP12 activity (332 ± 77 RFU vs 458 ± 91 RFU, P < .05). Also, the STEMI group demonstrated much higher MMP12 concentrations than the normal coronary artery control group (5.030 ± 2.24 pg/mL vs 1.720 ± 0.51 pg/mL, P < .05) and with lower MMP12 activity (332 ± 77 RFU vs 549 ± 112 RFU, P < .05). In addition, the STEMI group had significantly higher tissue inhibitor of metalloproteinases-1 (TIMP1) concentration (573.40 ± 270.60 pg/mL) than SAP group (384.50 ± 147.70 pg/mL) and control group (219.90 ± 154.80 pg/mL, P < .05). The imbalance in MMP12/TIMP ratio was observed in the STEMI group compared with SAP and control group (P < .05).This study demonstrated that MMP12 exists in human coronary thrombus. Patients with STEMI have elevated plasma level of MMP12 and the imbalance of MMP12/TIMP1. These data supported that MMP12 might be of potential relevance in STEMI.

Clinical and imaging features of spinal cord type of neuro Behçet disease: A case report and systematic review.

To investigate the clinical and MRI characteristics of spinal cord nerve Behçet's disease.

Cervical epidural analgesia complicated by epidural abscess: A case report and literature review.

Spinal epidural abscess is an uncommon complication in clinical practice. If the abscess is large enough, the patient will rapidly develop neurologic signs of spinal injury, and urgent neurosurgical intervention may be required.

Intensity-modulated radiation therapy for patients with 1 to 3 brain metastases in recursive partitioning analysis class 3.

The prognosis is extremely poor for patients with brain metastases in recursive partitioning analysis (RPA) class 3. It is not clear whether dose elevation for brain lesions in addition to whole-brain radiotherapy could improve survival for those patients. This study aimed to assess the efficacy and safety of dose elevation with intensity-modulated radiation therapy (IMRT) for patients with 1 to 3 brain metastases in RPA class 3.From January 2013 to December 2015, 24 patients with 1 to 3 brain metastases in RPA class 3 were included in this study. The median age was 60 (range 41-85) years and the mean graded prognostic assessment (GPA) score was 1.25 (range 0.5-2). Whole-brain radiotherapy (30 Gy) with a simultaneous integrated boost (SIB) to the brain metastases (totaling 40 Gy) was delivered in 10 fractions using IMRT technique. Survival times and overall safety were assessed. The significance of prognostic variables on survival was assessed by both univariate and multivariate analyses.All of the patients completed the planned SIB schedule. The overall response rate was 66.7%. The median survival time (MST) was 8 months for the entire group of patients. The MST was 5 months for patients with a GPA score of 0.5 to 1 (n = 11 patients) and 12 months with a GPA score of 1.5 to 2 (n = 13 patients). No acute or late toxicities greater than grade 2 were detected. Age and subsequent chemotherapy were significantly associated with MST on univariate and multivariate analyses.It is feasible to elevate radiation doses to 40 Gy using the IMRT technique in RPA class 3 patients with 1 to 3 brain metastases without serious toxicities. The preliminary results are encouraging and further studies with larger cohorts are warranted.

Ganglion cell layer complex measurements in compressive optic neuropathy.

Neuroophthalmologists and neurosurgeons are often asked by their patients what their visual prognosis will be after decompression of an optic chiasm lesion. Previous methods have been studied but have not provided consistent guidance. However, a recent algorithm which allows for retinal ganglion cell analysis from optical coherence tomography (OCT) may be more helpful.

Neuro-ophthalmic manifestations of cerebrovascular accidents.

Ocular functions can be affected in almost any type of cerebrovascular accident (CVA) creating a burden on the patient and family and limiting functionality. The present review summarizes the different ocular outcomes after stroke, divided into three categories: vision, ocular motility, and visual perception. We also discuss interventions that have been proposed to help restore vision and perception after CVA.

Association Between Interleukin-10 Receptors and the CD45-Immunophenotype of Central Nervous System Tumors: A Preliminary Study.

One of the current hypotheses assumes that brain tumors exert an immunosuppressive influence on the surrounding cellular environment. Interleukin-10 (IL-10) is one of the immunosuppressive cytokines modifying the biological activity of cancer. The aim of this study was to assess the expression of IL10R in CD45(+) cells within primary brain tumors and metastases and establish its association with tumor basic immunophenotype.

Programmed Cell Death Ligand 1 Expression in Primary Central Nervous System Lymphomas: A Clinicopathological Study.

Programmed cell death ligand 1 (PD-L1)/programmed cell death 1 (PD-1) have been shown to predict response to PD-L1/PD-1-targeted therapy. We analyzed PD-L1 expression in primary central nervous system lymphomas (PCNSLs).

Differential temperature sensitivity of synaptic and firing processes in a neural mass model of epileptic discharges explains heterogeneous response of experimental epilepsy to focal brain cooling.

Experiments with drug-induced epilepsy in rat brains and epileptic human brain region reveal that focal cooling can suppress epileptic discharges without affecting the brain's normal neurological function. Findings suggest a viable treatment for intractable epilepsy cases via an implantable cooling device. However, precise mechanisms by which cooling suppresses epileptic discharges are still not clearly understood. Cooling experiments in vitro presented evidence of reduction in neurotransmitter release from presynaptic terminals and loss of dendritic spines at post-synaptic terminals offering a possible synaptic mechanism. We show that termination of epileptic discharges is possible by introducing a homogeneous temperature factor in a neural mass model which attenuates the post-synaptic impulse responses of the neuronal populations. This result however may be expected since such attenuation leads to reduced post-synaptic potential and when the effect on inhibitory interneurons is less than on excitatory interneurons, frequency of firing of pyramidal cells is consequently reduced. While this is observed in cooling experiments in vitro, experiments in vivo exhibit persistent discharges during cooling but suppressed in magnitude. This leads us to conjecture that reduction in the frequency of discharges may be compensated through intrinsic excitability mechanisms. Such compensatory mechanism is modelled using a reciprocal temperature factor in the firing response function in the neural mass model. We demonstrate that the complete model can reproduce attenuation of both magnitude and frequency of epileptic discharges during cooling. The compensatory mechanism suggests that cooling lowers the average and the variance of the distribution of threshold potential of firing across the population. Bifurcation study with respect to the temperature parameters of the model reveals how heterogeneous response of epileptic discharges to cooling (termination or suppression only) is exhibited. Possibility of differential temperature effects on post-synaptic potential generation of different populations is also explored.