关键词： Traumatic   Vertebral artery   Rupture   Intraspinal hematoma  

摘要： Traumatic vertebral artery rupture is extremely rare. Herein, we report a case of traumatic vertebral artery rupture causing extensive intraspinal hematoma and progressive neurological symptoms.

关键词： Amyotrophic lateral sclerosis   Multiple sclerosis   Parkinson's disease   Stroke   Oropharyngeal dysphagia  

摘要： Neurological diseases are frequently associated with swallowing disorders and malnutrition. Moreover, patients with neurological diseases are at increased risk of micronutrient deficiency and dehydration. On the other hand, nutritional factors may be involved in the pathogenesis of neurological diseases. Multiple causes for the development of malnutrition in patients with neurological diseases are known including oropharyngeal dysphagia, impaired consciousness, perception deficits, cognitive dysfunction, and increased needs. The present evidence- and consensus-based guideline addresses clinical questions on best medical nutrition therapy in patients with neurological diseases. Among them, management of oropharyngeal dysphagia plays a pivotal role. The guideline has been written by a multidisciplinary team and offers 88 recommendations for use in clinical practice for amyotrophic lateral sclerosis, Parkinson's disease, stroke and multiple sclerosis. (C) 2017 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.

关键词： healthcare   telemedicine   telestroke   stroke   head and spinal injury  

摘要： Background:Equality in healthcare between urban and rural areas is problematic in France. Telemedicine networks are expected to improve equality in expertise assessment. We aimed to evaluate the use and impact of a regional rural French telemedicine network, dedicated to medical and surgical neurological emergencies, on interhospital patient ***:Eight community hospital emergency departments were remotely connected to the only university hospital in Franche-Comte, France. We prospectively obtained data from all patients consecutively admitted to emergency care departments in the region and who received medical or neurosurgical expertise by telemedicine from January 2002 to December 2015. The reasons for requesting expertise, number of requested neurological opinions, and interhospital patient transfers were analyzed. Economic savings were determined by estimating the cost of avoided ***:A total of 23,710 patients had telemedicine consultations in the region. The network was used by every community hospital (independently of the existence of local neurological teams). These consultations were overwhelmingly for cases of stroke (30%) and head or spinal injuries (36%). Cerebral tumors represented 9% of teleconsultations. In 2015, 75% of patients admitted to the remote hospitals that did not have onsite neurological expertise nevertheless received neurovascular tele-expertise. The rate of thrombolyzed patients dramatically increased within 13 years regionally (9.9%) and 33.5% of thrombolyses were performed by telemedicine. The number of patients examined by telemedicine and admitted for head or spinal injuries also increased over the 13-year period (12% vs. 21%). Secondary interhospital transfers were halved for both pathologies. The estimated saving is approximate to Euro3.5 ***:Telemedicine networks facilitate acute-phase neurological assessment and prevent unnecessary secondary interhospital transfers.

关键词： Glucocerebrosidase   GBA1   Dystonia   Parkinson's disease   Gaucher disease  

摘要： Background: Neuronopathic Gaucher disease type 3 (GD3) is frequent in northern Sweden, whereas GD1 is found throughout the country. In a nation-wide study, we examined neurological manifestations and clinical course in 12 patients with GD3 and 13 patients with GD1. Methods: The patients were evaluated by standardized neurological assessments. Every sixth month, the GD3 patients were rated with the modified Severity Scoring Tool. At baseline and at the 3 years follow-up, patients underwent University of Pennsylvania Smell Identification Test, Montreal Cognitive Assessment and Hospital Anxiety and Depression Scale. When clinical signs were present, additional examinations were undertaken. Results: Marked clinical heterogeneity was evident in both GD3 and GD1 groups. Several GD3 patients had a hitherto unreported rapid and repetitive dystonia-like hyperkinetic movement disorder. Most patients with GD3 have abnormalities of horizontal gaze, ataxia and focal epilepsy, some also had cognitive impairment, anxiety and hyposmia. Six GD3 patients, all homoallelic for L444P GBA1 mutations, have lived beyond 40 years of age;and none has developed Parkinsonism. Two of the GD1 patients suffer from Parkinsonism;mild to complete hyposmia was present in six GD3 and five GD1 patients. Neither the group of GD3 nor GD1 patients had detectable progression of their neurological manifestations. Conclusions: These middle-aged and older Swedish GD3 or GD1 patients are clinically stable over time. However, we have identified unusual clinical features, discordant phenotypes and a hyperkinetic dystonia-like movement disorder which appears unique to this Swedish disease variant and expands the phenotype for GD. (C) 2016 Elsevier Inc. All rights reserved.

摘要： The article discusses a study on whether there is a need to screen N-methyl-D-aspartate receptor antibody in patients with ovarian teratomas and without symptoms of autoimmune encephalitis. Topics mentioned include the link between NMDAR encephalitis and OT, a recommendation on the need to exclude the presence of OT in patient with NMDAR encephalitis, and the improvement of clinical management of OT patients after screening for elevated NMDAR antibodies.

关键词： ARYL hydrocarbon receptors   POLYCHLORINATED biphenyls   MICE as laboratory animals   NEUROLOGICAL disorders   PARKINSON'S disease  

摘要： Polychlorinated biphenyls (PCBs) are persistent organic pollutants that remain a human health concern with newly discovered sources of contamination and ongoing bioaccumulation and biomagnification. Children exposed during early brain development are at highest risk of neurological deficits, but highly exposed adults reportedly have an increased risk of Parkinson's disease. Our previous studies found allelic differences in the aryl hydrocarbon receptor and cytochrome P450 1A2 (CYP1A2) affect sensitivity to developmental PCB exposure, resulting in cognitive deficits and motor dysfunction. High-affinity Ahr (b) Cyp1a2(-/-) mice were most sensitive compared with poor-affinity Ahr (d) Cyp1a2(-/-) and wild-type Ahr (b) Cyp1a2(+/+) mice. Our follow-up studies assessed biochemical, histological, and gene expression changes to identify the brain regions and pathways affected. We also measured PCB and metabolite levels in tissues to determine if genotype altered toxicokinetics. We found evidence of AHR-mediated toxicity with reduced thymus and spleen weights and significantly reduced thyroxine at P14 in PCB-exposed pups. In the brain, the greatest changes were seen in the cerebellum where a foliation defect was over-represented in Cyp1a2(-/-) mice. In contrast, we found no difference in tyrosine hydroxylase immunostaining in the striatum. Gene expression patterns varied across the three genotypes, but there was clear evidence of AHR activation. Distribution of parent PCB congeners also varied by genotype with strikingly high levels of PCB 77 in poor-affinity Ahr (d) Cyp1a2(-/-) while Ahr (b) Cyp1a2(+/+) mice effectively sequestered coplanar PCBs in the liver. Together, our data suggest that the AHR pathway plays a role in developmental PCB neurotoxicity, but we found little evidence that developmental exposure is a risk factor for Parkinson's disease.

关键词： GH   GnRH   neuroprotection   neuroregeneration   neurotrophic   therapy   CNS   extrapituitary   extrahypothalamic  

摘要： This brief review of the neurological effects of growth hormone (GH) and gonadotropin-releasing hormone (GnRH) in the brain, particularly in the cerebral cortex, hypothalamus, hippocampus, cerebellum, spinal cord, neural retina, and brain tumors, summarizes recent information about their therapeutic potential as treatments for different neuropathologies and neurodegenerative processes. The effect of GH and GnRH (by independent administration) has been associated with beneficial impacts in patients with brain trauma and spinal cord injuries. Both GH and GnRH have demonstrated potent neurotrophic, neuroprotective, and neuroregenerative action. Positive behavioral and cognitive effects are also associated with GH and GnRH administration. Increasing evidence suggests the possibility of a multifactorial therapy that includes both GH and GnRH.

摘要： Background: Fatigue in post-polio syndrome (PPS) has been shown to affect quality of life adversely. There is currently no disease-specific measure of fatigue for PPS. Objective: To develop a scale to measure fatigue in PPS that meets rigorous psychometric standards. Design: Qualitative followed by validation and test-retest studies. Setting: Polio clinic followed by national questionnaire studies. Participants: A total of 45 participants from polio clinic for qualitative;319 participants from clinic or self-referral for validation study, of whom 87 completed the retest questionnaire. Methods: Draft questionnaire items on PPS fatigue were derived from transcripts of qualitative interviews. After cognitive debriefing, the draft measure was administered by mail along with comparator questionnaires to a new sample. Main Outcome Measurements: Draft PPS fatigue measure, Fatigue Severity Scale, and visual analog scale for fatigue. Results: Analysis of 271 of 319 (85%) questionnaires identified a 2-factor solution (RMSEA 0.058). For the physical subscale, a 20-item scale showed good fit (chi(2) P = .189), strict unidimensionality (t-test 5.17%), and reliability 0.91. For the cognitive subscale, a 7-item scale showed excellent fit (chi(2) P = .917), strict unidimensionality (t-test 5.2%), and reliability 0.89. Evidence of a "difficulty factor" emerged also supporting a total score that showed good fit (chi(2) P = .151), strict unidimensionality (t-test 0.4%), and reliability consistent with group use at 0.73. Test-retest correlations for all scales were greater than 0.85. Standard error of measurement on metric ranges was 5.4 for total, 2.9 for physical, and 1.69 for cognitive domains. With the latent estimate of the total score transformed to a 0-100 scale, the mean score was 49.5 (SD 6.9). Spearman correlations with the Fatigue Severity Scale and visual analog scale were 0.60 and 0.55, respectively. Conclusions: A patient-derived Neurological Fatigue Index for PPS, with p

关键词： arboviruses   ganglionopathy   Guillain-Barre syndrome   sensory neuronopathy   Zika virus  

关键词： dentistry   DPSCs   neurological diseases   neurons   regenerative medicine  

摘要： Medical research in regenerative medicine has brought promising perspectives for the use of stem cells in clinical trials. Stem cells are undifferentiated cells capable of multilineage differentiation and available in numerous sources in the human body. Dental pulp constitutes an attractive source of these cells since collecting mesenchymal stem cells from this site is a noninvasive practice that can be performed after a common surgical extraction of supernumerary or wisdom teeth. Thus, tissue sacrifice is very low and several cytotypes can be obtained owing to these cells' multipotency, in addition to the fact that they can be cryopreserved and stored for long periods. Mesenchymal stem cells have high proliferation rates, making them favorable for clinical application. These multipotent cells, present in biological waste, constitute an appropriate resource in the treatment of many neurological diseases.