摘要： Meconium stained amniotic fluid (MSAF), recently found not to be sterile as previously thought, may affect newborn brain development by several mechanism. We investigated the possible impact of MSAF on the occurrence of neurological related hospitalizations in the offspring throughout childhood.

关键词： Hypersomnolence   Narcolepsy   Hypersomnia   Orexin   Sleep disorder  

摘要： PurposeSymptomatic narcolepsy is characterized as low orexin (hypocretin) levels (110pg/ml) due to neurological diseases. However, we have experienced the cases that show symptoms similar to narcolepsy, complaining of pathological sleepiness even in cases with intermediate orexin levels (110-200pg/ml). Therefore, we reevaluated the previously reported cases to make the concept of disease to the group in which the orexin concentration is *** orexin-deficient symptomatic narcolepsy has been reported worldwide, facilities that are capable of measuring orexin are still limited. Currently, our laboratory handles with almost all the CSF samples from Asian countries for determining orexin levels. We measured over 2500 cases including 182 cases suspected of symptomatic narcolepsy/hypersomnolence. Therefore, we subdivided these cases into three groups according to CSF orexin levels, low orexin group (110pg/ml), intermediate orexin group (110-200pg/ml), and normal orexin group (>200pg/ml). We defined symptomatic hypersomnia for hypersomnolence cases with intermediate CSF orexin levels. MSLT was recommended but not necessary. Short sleep latency (<8min) without SOREMPs should be observed on MSLT when performed. We compared symptoms (excessive daytime sleepiness and cataplexy) and objective measures (mean sleep latency and number of SOREMPs in MSLT) among the three groups, attempting to characterize the symptomatic hypersomnia *** the 182 cases with clinical hypersomnolence, there were 32 cases of symptomatic narcolepsy (16%) and 14 cases of symptomatic hypersomnia (8%). Causative diseases of symptomatic narcolepsy cases consist of immune-mediated demyelinating disorders (34%) and neurodegenerations (19%), whereas those of symptomatic hypersomnia are immune-mediated demyelinating disorders (43%) and encephalopathies (14%). Significant differences were not found between orexin levels and the existence of cataplexy, SOREMPs, and short sleep

关键词： Epilepsy   Mitochondrial genome   Movement disorders   Neuromuscular disease  

摘要： The POLG gene encodes the mitochondrial DNA polymerase that is responsible for replication of the mitochondrial genome. Mutations in POLG can cause early childhood mitochondrial DNA (mtDNA) depletion syndromes or later-onset syndromes arising from mtDNA deletions. POLG mutations are the most common cause of inherited mitochondrial disorders, with as many as 2% of the population carrying these mutations. POLG-related disorders comprise a continuum of overlapping phenotypes with onset from infancy to late adulthood. The six leading disorders caused by POLG mutations are Alpers-Huttenlocher syndrome, which is one of the most severe phenotypes;childhood myocerebrohepatopathy spectrum, which presents within the first 3 years of life;myoclonic epilepsy myopathy sensory ataxia;ataxia neuropathy spectrum;autosomal recessive progressive external ophthalmoplegia;and autosomal dominant progressive external ophthalmoplegia. This Review describes the clinical features, pathophysiology, natural history and treatment of POLG-related disorders, focusing particularly on the neurological manifestations of these conditions.

关键词： Neurophysiology   Spinal cord injury  

摘要： Study design Experimental study. Objectives To investigate the role of BDNF-TrkB signaling that promotes the recovery of neurological function in rats with incomplete spinal cord injury (SCI) after treadmill training (TT). Setting Rehabilitation Medicine Center of the First Affiliated Hospital of Nanjing Medical University, Nanjing, China. Methods Forty rats were divided into five groups: (i) Sham;(ii) SCI and phosphate-buffered saline (PBS) (SCI/PBS);(iii) SCI-TT/PBS;(iv) SCI/TrkB-IgG;and (v) SCI-TT/TrkB-IgG. The intrathecal catheter and T10 contusion SCI model was established. At 7-day post SCI, the BDNF-TrkB signaling was blocked by TrkB-IgG. Exercise began at 8th day after SCI and continued for 4 weeks. The BBB scale and motor-evoked potential (MEP) were used for the evaluation of the locomotor functions. The BDNF/TrkB, PSD-95, SYP synthesis, and neuroprotective effect was determined by western blot, Nissl, or immunohistochemistry staining. Results The expression of BDNF and TrkB in the SCI-TT/PBS group was 1.46 +/- 0.09 and 1.70 +/- 0.22, respectively, higher than that in SCI/PBS group (0.51 +/- 0.04 and 0.76 +/- 0.07, respectively), relative to the Sham group. The BBB scores in the Sham, SCI/ PBS, SCI-TT/PBS, SCI/TrkB-IgG, and SCI-TT/TrkB-IgG groups were 21.00 +/- 0.00, 7.63 +/- 0.74, 12.13 +/- 1.36, 7.88 +/- 0.64, and 8.75 +/- 0.88, respectively. The percentages of MEP responders/non-responders were 100, 0, 75, 0, and 50%. The MEP latencies in Sham, SCI-TT/PBS, and SCI-TT/TrkB-IgG groups were 6.65 +/- 0.19, 13.32 +/- 2.95, and 19.55 +/- 4.55 ms, respectively. The number of NeuN(+ )neurons, the cell body area of motor neurons, PSD-95, and SYP expression in the SCI-TT/PBS group was significantly higher than that in the SCI/PBS, SCI/TrkB-IgG, and SCI-TT/TrkB-IgG groups. Conclusion The BDNF-TrkB signaling is a critical pathway in exercise training that promotes the recovery of neurological function in rats with incomplete SCI.

关键词： Foreign accent syndrome   stroke   dysarthria   cerebellum   apraxia of speech  

摘要： Foreign Accent Syndrome (FAS) is an intriguing motor speech disorder which has captured the interest of the scientific community and media for decades. At the moment, there is no comprehensive model which can account for the pathophysiology of this disorder. This paper presents a review of 112 FAS cases published between 1907 and October 2016: these were analyzed with respect to demographic characteristics, lesion location, associated neurocognitive symptoms, and comorbid speech and language disorders. The analysis revealed that organic-neurogenic FAS is more frequent in women than in men. In organic-neurogenic FAS over half of the patients acquired the foreign accent after a stroke. Their lesions are typically located in the left supratentorial regions of the brain, and generally involve the primary motor cortex and premotor cortex (BA 4 and 6), and/or the basal ganglia. Although neurocognitive data are not consistently reported, vascular FAS patients regularly suffer frontal executive dysfunctions. On the basis of a careful comparison of the cognitive and theoretical accounts of FAS, AoS and ataxic dysarthria, it is concluded that FAS should be regarded a dual component motor speech disorder in which both planning and motor execution of speech may be affected.

关键词： Neurological disorder   Alzheimer's disease   Stem cells   Basal forebrain cholinergic progenitor cells  

摘要： A neurological disorder is any disorder or abnormality in the nervous system. Among different neurological disorders, Alzheimer's disease (AD) is recognized as the sixth leading cause of death globally. Considerable research has been conducted to find pioneer treatments for this devastating disorder among which cell therapy has attracted remarkable attentions over the last decade. Up to now, targeted differentiation into specific desirable cell types has remained a major obstacle to clinical application of cell therapy. Also, potential risks including uncontrolled growth of stem cells could be disastrous. In our novel protocol, we used basal forebrain cholinergic progenitor cells (BFCN) derived from human chorion-derived mesenchymal stem cells (hC-MSCs) which made it possible to obtain high-quality population of cholinergic neurons and in vivo in much shorter time period than previous established methods. Remarkably, the transplanted progenitors fully differentiated to cholinergic neurons which in turn integrated in higher cortical networks of host brains, resulting in significant improvement in cognitive assessments. This method may have profound implications in cell therapies for any other neurodegenerative disorders.

关键词： neurology   quality of life   unmet needs  

摘要： Aim: The aim of our research was to explore the unfulfilled needs of patients with a progressive neurological disease in advanced stage of the illness within the current system of health and social care in the Czech Republic. Design and Setting: Qualitative research (grounded theory) was used to conceptualize the patterns of unmet palliative care needs in Czech Republic. Methods: The data collection method comprised individual, in-depth interviews (n = 19) and focus groups (n = 4) where a total of 52 respondents participated (patients with progressive neurological diseases [PNDs], family members, and professionals). Results: Two main categories of unfulfilled needs were determined (life with the disease, professional help), and they were described in the context of the 3 crucial themes identified in the study-the symptoms of the advanced stage of the disease resulted in substantial reduction of physical self-sufficiency, loss of autonomy, and social isolation;the level of dependence on the support and help of others increased;the patients also highlighted several problems related to health-care services. Conclusion: The unmet needs should be taken into consideration when creating the concept of the neuropalliative and rehabilitation care, including the mental health support plan, because of the emotional, behavioral, and cognitive disorders that frequently occur in the lives of a substantial amount of patients with PND.

摘要： The author comments on the consideration of neonatal neurology as a potential subspecialty of pediatric neurology in its own right. Topics covered include the failure to bridge the gap on the issue of whether there was a role for a neurologist on a neonatal unit and for a multidisciplinary approach for neonatal neurocritical care, and the need for a recognized subspecialty of neonatal neurology within paediatric neurology that is closely linked to neonatology and developmental medicine.

摘要： [10] She had brought out the criteria for early diagnosis of FA. [...]the 1970s and 1980s were the decades of nosological characterization, yet the precise cause was eluding. [20] The World Health Organization (WHO) has initiated the “United against rabies collaboration: a global catalytic platform to achieve zero human rabies death by 2030-popularly known as “zero by 30.” A person with features of encephalitis was admitted to a general hospital in North Kerala, and the neurologist who attended to him had suspected Nipah encephalitis within 12 h of admission and the blood samples confirmed the diagnosis in 24 h. The epidemic affected 19 persons within that period and 17 of them succumbed to the illness which included Mrs. Lini Puthusserry, a nurse who had attended to the first case in that epidemic. The clinical acumen of the neurologist and the availability of technology at doorstep (to carry out the laboratory tests), partnership between institutions, government and nongovernmental level, and prompt public health response enabled the state to contain the epidemic [Figure 4].

关键词： Magnetic resonance imaging   Central nervous system   Meninges   Lymphocytes  

摘要： ObjectivesBing-Neel syndrome (BNS) is a rare neurological complication of Waldenstrom's macroglobulinemia. The aim of this study is to describe the spectrum of radiological manifestations of this syndrome and their prevalence in order to facilitate its early ***-four patients with BNS were diagnosed between 1994 and 2016 in eight centres in France. We retrospectively examined the medical records of these patients as well as the corresponding literature, focusing on imaging studies. Recorded data were statistically analysed and radiological findings *** mean age of our patients was 62.4 years (35-80 years). The vast majority of patients were men, with a male to female ratio of 9:1. Findings included parenchymal or meningeal involvement or both. The most common finding was leptomeningeal infiltration, either intracranial or spinal, with a prevalence reaching 70.8%. Dural involvement was present in 37.5% of patients. In 41.7% (10/24) of patients, there was parenchymal involvement with a higher prevalence of brain comparing to medullar involvement (33.3% and 23.1% respectively). High T2 signal of the parenchyma was identified in 41.7% of patients and high signal in diffusion was evident in 25% of them. Intraorbital or periorbital involvement was also detected in four cases. A proposition regarding the appropriate imaging protocol completed our ***'s diagnosis remains challenging. Central nervous system MRI findings in the setting of known or suspected Waldenstrom's macroglobulinemia appear to be highly suggestive of BNS and appropriate imaging protocols should be implemented for their *** Points center dot Diagnosis of Bing-Neel syndrome (BNS) remains challenging and recent expert recommendations include MRI in the diagnostic criteria for the *** dot The most common radiological manifestations of BNS are leptomeningeal/dural infiltration or parenchymal involvement of brain or spinal cord, but man