关键词： Cervicothoracic spine duplication   Split cord malformation   Syrinx  

摘要： Spine duplication is a very rare condition with the literature being composed of only case reports. All previously reported cases were thoracolumbar spine duplications. Here, we report cervicothoracic spine duplication in a neurological intact male. According to our knowledge, it is the first case in the literature of cervicothoracic spine duplication. A 3-year-old patient presented to a primary physician with a complaint of short stature. He was referred to our department with suspected spinal deformity. Computerized tomography imaging revealed anterior bony structure duplication and posterior dysmorphic elements at the C5-T9 levels. Magnetic resonance imaging revealed a syrinx cavity which splits cord at the duplication level and the relation of the syrinx with posterior mediastinum through anterior bone defect. He was followed up for 10 years. In the literature, spine duplication has been classified as a severe form of split cord malformation because of the concurrence of bone duplication with split spinal cord malformation (SCM). This case presents a distinct form of SCM which shows non-duplicated dural tube as unclassified and cervicothoracic duplication level without neurological deficitis. Treatment of SCM was based on removal of splitting fibrous/osseous process. Neurologic intact spine duplication could be followed up without surgical intervention.

关键词： CXCL13   Cerebrospinal fluid   Lyme neuroborreliosis   Tick-borne encephalitis   Aseptic meningitis   recomBead assay  

摘要： The aims of the study were to determine and compare the concentration of CXCL13 in cerebrospinal fluid (CSF) of patients with Lyme neuroborreliosis (LNB) and various other neurological disorders applying a Luminex based assay and ELISA, and to find factors associated with CXCL13 concentration. CSF samples obtained from four clinically well-defined groups of patients (proven LNB, suspected LNB, tick-borne encephalitis (TBE), and aseptic meningitis/meningoencephalitis other than TBE) - 25 samples per group - were analyzed. The performance of the Luminex recomBead CXCL13 assay (Microgen, Neuried, Germany) and ELISA (Euroimmun, Lubeck, Germany) was assessed by receiver operating characteristics. CXCL13 cut-off values were presented as functions of CSF lymphocyte/monocyte counts. Demographic variables, CSF findings, and history of erythema migrans were assessed as possible predictors for CXCL13 CSF concentrations by a general linear model. The calculated cut-off values determined by the maximum of the Youden index were > 131 pg/mL for recomBead and > 259 pg/mL for the ELISA. RecomBead showed a sensitivity of 88% (68.8-97.5%) and a specificity of 94% (83.5-98.7%). For the ELISA the corresponding values were 84% (63.9-95.5%) and 98% (89.4-99.9%). The CXCL13 concentration positively correlated with CSF lymphocyte/ monocyte count and Borrelia-specific intrathecal antibody index (p<0.05). High CXCL13 concentrations were only found in the group with proven LNB. CXCL13 levels above cut-off value were established in some patients with viral meningitis/meningoencephalitis but were not detected in patients with suspected LNB without pleocytosis. Applying a linearized cut-off of the CXCL13 concentration in the CSF which is dependent on the CSF cell count is a novel approach in the laboratory diagnosis of LNB.

关键词： big data   epidemiology   10th International Classification of Diseases diagnoses   inpatients   neurological service use   outpatients   record linkage  

摘要： Background and purposeIn order to plan neurological capacities at a national level for the next decade, the current use of neurological services should be evaluated. We analyzed the utilization of neurological services in Hungary, a country with a single-payer health insurance system covering the whole population. MethodsWe created a database from medical reports submitted to the National Health Insurance Fund from all hospitals and outpatient services between 2004 and 2013. The number of subjects presenting to the neurological healthcare system and their major diagnoses by 10th International Classification of Diseases categories were analyzed. The overall healthcare service utilization of these patients was also estimated. ResultsOf the 10 million inhabitants, 2.9 million people used an inpatient or outpatient neurological service at least once over the 10-year period. Annually, 1% of the population was admitted to neurological inpatient wards and 6% of the population used some neurological outpatient service. Major reasons for using neurological services were: cerebrovascular diseases (I60-I69;1.2 million patients), episodic and paroxysmal disorders (G40-G47;1.3 million patients) and general symptoms and signs (R50-R56;1.3 million patients). The 2.9 million people had 12.7 million hospital admissions to any ward and 365.7 million outpatient visits to any specialist during the 10 years. ConclusionsThe demand for neurological services is high in Hungary;close to 30% of the population used an inpatient or outpatient neurological service at least once during this 10-year period. Results from this project provide data for international comparisons and help to ensure better informed and more focused resource allocation.

关键词： Sequencing   neurology   next generation sequencing   Analyses, Sequence   Child  

关键词： congenital amusia   stroke   auditory short-term memory   auditory working memory   delayed matching-to-sample task   fMRI  

摘要： Auditory short-term memory (STM) is a fundamental ability to make sense of auditory information as it unfolds over time. Whether separate STM systems exist for different types of auditory information (music and speech, in particular) is a matter of debate. The present paper reviews studies that have investigated both musical and verbal STM in healthy individuals and in participants with neurodevelopmental and neurological disorders. Overall, the results are in favor of only partly shared networks for musical and verbal STM. Evidence for a distinction in STM for the two materials stems from (1) behavioral studies in healthy participants, in particular from the comparison between nonmusicians and musicians;(2) behavioral studies in congenital amusia, where a selective pitch STM deficit is observed;and (3) studies in brain-damaged patients with cases of double dissociation. In this review we highlight the need for future studies comparing STM for the same perceptual dimension (e.g., pitch) in different materials (e.g., music and speech), as well as for studies aiming at a more insightful characterization of shared and distinct mechanisms for speech and music in the different components of STM, namely encoding, retention, and retrieval.

关键词： melatonin   ischemia   alzheimer   parkinson  

摘要： Melatonin is a neurohormone secreted from the pineal gland and has a wide-ranging regulatory and neuroprotective role. It has been reported that melatonin level is disturbed in some neurological conditions such as stroke, Alzheimer's disease, and Parkinson's disease, which indicates its involvement in the pathophysiology of these diseases. Its properties qualify it to be a promising potential therapeutic neuroprotective agent, with no side effects, for some neurological disorders. This review discusses and localizes the effect of melatonin in the pathophysiology of some diseases.

关键词： Optic nerve sheath diameter   Cardiac arrest   Neurological outcome   Targeted temperature management   Post-cardiac arrest syndrome   Prognostic factor  

摘要： Aim: Previous studies indicated that the optic nerve sheath diameter (ONSD) measured using brain computed tomography (CT) is a prognostic factor for poor neurological outcome after cardiac arrest. However, these studies were retrospective or included a small sample size. We performed a prospective multi-centre observational study to investigate the correlation between the ONSD on early brain CT and neurological outcomes in patients undergoing targeted temperature management (TTM). Methods: This study used data from the Korean Hypothermia Network prospective registry between November 2015 and October 2016. Out-of-cardiac arrest patients who underwent brain CT within 2 h after return of spontaneous circulation (ROSC) were included. The primary endpoint was neurological outcomes at 6 months (cerebral performance category;CPC);the secondary outcome was hospital mortality. The ONSD was measured using unenhanced brain CT images. Results: In total, 374 patients were included from 18 hospitals, and 329 underwent CT within 2 h after ROSC. Six months after cardiac arrest, good (CPC 1-2) and poor (CPC 3-5) neurological outcomes were observed in 99 (30.09%) and 230 (69.91%) patients, respectively. There was no significant difference in the ONSD between groups (good outcome group: 5.61 +/- 0.59 mm, poor outcome group: 5.69 +/- 0.79 mm;p = 0.275), nor between discharged patients who survived and those with hospital mortality (5.63 +/- 0.64 mm and 5.70 +/- 0.67 mm, respectively, p = 0.399). Conclusion: The ONSD on initial brain CT after ROSC was not correlated with neurological outcome at 6 months in patients who underwent TTM.

关键词： Bone scan   Hypocalcemia   Hypophosphatemia   Osteomalacia  

摘要： Patients with osteomalacia often visit the neurology department with conditions mimicking other myopathies. We analyzed clinical features of osteomalacia patients who visited the neurology department. These patients frequently presented with hypocalcemia, hypovitaminosis D, and pain with less severe weakness. Osteomalacia should be considered when patients present with pain and weakness. Osteomalacia is a disease of bone metabolism;however, some patients with osteomalacia initially visit the neurology department. As these patients often complain of weakness and gait disturbance, osteomalacia can be confused with other myopathies. We analyzed the clinical features of patients with osteomalacia who visited the neurology department. We retrospectively reviewed the medical records. Osteomalacia was diagnosed based on symptoms, laboratory features, and imaging results. We compared the characteristics of patients with osteomalacia who visited the neurology department with (1) those who did not visit the neurology department and (2) patients with idiopathic inflammatory myopathy. Eighteen patients with osteomalacia visited the neurology department (NR group). The common etiologies in the NR group included tumors or antiepileptic medication, whereas antiviral medication was the most common in patients who did not visit the neurology department (non-NR group). The NR group showed lower serum calcium (p = 0.004) and 25-hydroxyvitamin D (p = 0.006) levels than the non-NR group. When compared with patients with inflammatory myopathy, both groups showed proximal dominant weakness. However, pain was more common in osteomalacia than in myopathy (p = 0.008), and patients with osteomalacia showed brisk deep tendon reflex more often (p = 0.017). Serum calcium (p = 0.003) and phosphate (p < 0.001) levels were lower in osteomalacia than in myopathy. It was not uncommon for patients with osteomalacia to visit the neurology department. The clinical presentation of these patients can be m

关键词： Urgent neurological consultation population  

摘要： In the province of Ferrara, Italy, the urgent neurological consultation (UNC) cases in the population correspond to the resident outpatients who undergo a UNC in the ER of the university hospital of Ferrara (UHFe). Thanks to this health organization a retrospective survey identified 612 UNC cases (range of age 7-102 years, median 67,5 years) in the study period giving a period prevalence rate of 173 per 100,000 (95% CI 159.3-187.3) which increased with age (chi(2) for trend = 178.4 p < 0.001). The daily UNC cases (range 0-14, mean = 7.3, 95% CI 7.1-7.5) followed the Poisson distribution (goodness-of-fit test: lambda = 7.3, chi(2) = 8082, 12 freedom degrees, p > 0.70). The prevalence rate decreased with the distance between the patients' residence and the UHFe (chi(2) for trend = 82.9, p < 0.001). The commonest clinical conditions requiring UNCs were acute cerebrovascular disorders (28%), headache (14%), and vertigo (9%). The hospital admission rate was 32.5% which increased with age (chi(2) for trend = 35.8, p < 0.001). The commonest discharge diagnoses of the admitted cases were ischemic stroke (57.3%), epilepsy (7%), TIA (6%), and intraparenchymal hemorrhage (5.5%). Acute cerebrovascular disease accounted for 69% of the discharge diagnoses. The survey showed that the UNCs' demand was higher than previous Italian data confirming that acute cerebrovascular disease is the most frequent acute neurological condition requiring attention in the ER. It also suggested that the UNCs could be poorly appropriate. These findings would require the healthcare administrators attention.

关键词： Atrioesophageal Fistula   Septic emboli   Stroke  

摘要： Atrioesophageal fistula (AEF) is a rare complication of cardiac ablation for atrial fibrillation. It can present in many ways, but neurological signs and symptoms are common initial signs sometimes resulting in neurosurgeons and neurologists first evaluating patients with the condition. We present a case report of at 68-year-old female who presented with acute stroke symptoms and multifocal hemorrhages on MRI who was worked up through our neurosurgery department and diagnosed with AEF. This case highlights three clues to alert neurological clinicians to AEF as a possible diagnosis;clinical worsening of neurological symptoms in correlation to episodes of emesis, septic emboli on CT/MRI, and bacteremia caused by a gram positive oral or GI flora. If neurological clinicians encounter these red flags, an immediate CT of the chest and abdomen and consultation with cardiothoracic surgery may be life-saving.