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81. Multi-center Clinical Study on the Diagnosis and Treatment Management of Rare Neurological Disease in Children

-center Clinical Study on the Diagnosis and Treatment Management of Rare Neurological Disease in Children Estimated Study Start Date : October 30, 2019 Estimated Primary Completion Date : October 30, 2021 Estimated Study Completion Date : December 30, 2021 Resource links provided by the National Library of Medicine related topics: related topics: resources: Groups and Cohorts Go to Group/Cohort 1 spinal muscular atrophy, SMA Progressive muscular atrophy (SMA) is a group of autosomal recessive (...) adrenoleukodystrophy X-ALD is an X-linked episode of a group of diseases characterized by progressive central nervous system demyelination and adrenal insufficiency. About 1/20000 male children. Most cases of X-ALD are treated for neurological symptoms for the first time, most of them start from 3-10 years old. Early manifestations include slow mental function, decreased academic performance, lack of interest or hyperactivity, difficulty in speech, difficulty in articulation, etc. Visual impairment and progressive

2018 Clinical Trials

82. The LUCINDA Trial: LUpron Plus Cholinesterase Inhibition to Reduce Neurological Decline in Alzheimer's

) Estimated Enrollment : 50 participants Allocation: Randomized Intervention Model: Parallel Assignment Masking: Double (Participant, Investigator) Primary Purpose: Treatment Official Title: The LUCINDA Trial: LUpron Plus Cholinesterase Inhibition to Reduce Neurological Decline in Alzheimer's Estimated Study Start Date : December 2018 Estimated Primary Completion Date : December 2020 Estimated Study Completion Date : December 2020 Resource links provided by the National Library of Medicine related topics (...) The LUCINDA Trial: LUpron Plus Cholinesterase Inhibition to Reduce Neurological Decline in Alzheimer's The LUCINDA Trial: LUpron Plus Cholinesterase Inhibition to Reduce Neurological Decline in Alzheimer's - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please

2018 Clinical Trials

83. An Open-label Treatment Protocol for VTS-270 in Patients With Neurologic Manifestations of NPC

Access Expanded Access Type : Treatment IND/Protocol Official Title: An Open-label Expanded Access Treatment Protocol for VTS-270 (2-hydroxypropyl-β-cyclodextrin) in Patients With Neurologic Manifestations of Niemann-Pick Type C Disease (NPC) Resource links provided by the National Library of Medicine related topics: resources: Interventions Go to Intervention Details: Drug: VTS270 Lumber intrathecal infusion of VTS-270 Eligibility Criteria Go to Information from the National Library of Medicine (...) An Open-label Treatment Protocol for VTS-270 in Patients With Neurologic Manifestations of NPC An Open-label Treatment Protocol for VTS-270 in Patients With Neurologic Manifestations of NPC - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one

2018 Clinical Trials

84. Reliability of Hammersmith Examination in Prediction of Neurological Outcomes (REHAPENO)

(developmental milestones). Study Design Go to Layout table for study information Study Type : Observational Actual Enrollment : 50 participants Observational Model: Cohort Time Perspective: Prospective Official Title: Reliability of Hammersmith Examination in Prediction of Neurological Outcomes In Preterm Infants - Observational Study (REHAPENO) Actual Study Start Date : March 1, 2018 Actual Primary Completion Date : January 25, 2019 Actual Study Completion Date : January 25, 2019 Resource links provided (...) Reliability of Hammersmith Examination in Prediction of Neurological Outcomes (REHAPENO) Reliability of Hammersmith Examination in Prediction of Neurological Outcomes (REHAPENO) - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies

2018 Clinical Trials

85. Safety and Efficacy of Cryoablation in Management of Sialorrhea in Patients With Neurological Disorders

Safety and Efficacy of Cryoablation in Management of Sialorrhea in Patients With Neurological Disorders Safety and Efficacy of Cryoablation in Management of Sialorrhea in Patients With Neurological Disorders - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100 (...) ). Please remove one or more studies before adding more. Safety and Efficacy of Cryoablation in Management of Sialorrhea in Patients With Neurological Disorders (SECAMS) The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. of clinical studies and talk to your health care provider before participating. Read our for details. ClinicalTrials.gov Identifier

2018 Clinical Trials

86. Evaluating the Utility of a Structured Clinical Protocol for Reducing the Impact of Behavioural and Psychological Symptoms of Dementia in Progressive Neurological Diseases: A Pilot Study (PubMed)

Evaluating the Utility of a Structured Clinical Protocol for Reducing the Impact of Behavioural and Psychological Symptoms of Dementia in Progressive Neurological Diseases: A Pilot Study Behavioural and psychological symptoms of dementia (BPSD) cause significant distress to both aged care residents and staff. Despite the high prevalence of BPSD in progressive neurological diseases (PNDs) such as multiple sclerosis, Huntington's disease, and Parkinson's disease, the utility of a structured (...) alongside a statistically significant decrease in BPSD from T1 to T2. In RAC, there was no significant time effect for BPSD or staff coping; however, a medium effect size was observed for staff job stress.Staff training and clinical support in the use of a structured clinical protocol for managing BPSD were linked to reductions in staff job stress, which may in turn increase staff capacity to identify indicators of resident distress and respond accordingly. Site variation in outcomes may relate

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2018 Behavioural neurology

87. Post-malaria neurological syndrome: four cases, review of the literature and clarification of the nosological framework (PubMed)

Post-malaria neurological syndrome: four cases, review of the literature and clarification of the nosological framework Post-malaria neurological syndrome (PMNS) is a debated entity, defined by neurological complications following a post-malaria symptom-free period and a negative blood smear. Four cases of PMNS are hereby reported and a review the literature performed to clarify the nosological framework of this syndrome.A French teaching hospital infectious diseases database was investigated (...) for all PMNS cases occurring between 1999 and 2016 and the PubMed database for cases reported by other institutions after 1997. A case was defined by the de novo appearance of neurological signs following a post-malaria symptom-free period, a negative blood smear, and no bacterial or viral differential diagnoses.Four patients from the database and 48 from PubMed, including 4 following Plasmodium vivax infection were found matching the definition. In the institution, the estimated PMNS incidence rate

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2018 Malaria journal

88. Drosophila as a Model for Assessing the Function of RNA-Binding Proteins during Neurogenesis and Neurological Disease (PubMed)

circuitry. RNA-binding proteins (RBPs) play an integral role in splicing and post-transcriptional gene regulation, and mutations in RBPs have been linked with multiple neurological disorders including autism, dementia, amyotrophic lateral sclerosis (ALS), spinal muscular atrophy (SMA), Fragile X syndrome (FXS), and X-linked intellectual disability disorder. Despite their widespread nature and roles in neurological disease, the molecular mechanisms and networks of regulated target RNAs have been defined (...) Drosophila as a Model for Assessing the Function of RNA-Binding Proteins during Neurogenesis and Neurological Disease An outstanding question in developmental neurobiology is how RNA processing events contribute to the regulation of neurogenesis. RNA processing events are increasingly recognized as playing fundamental roles in regulating multiple developmental events during neurogenesis, from the asymmetric divisions of neural stem cells, to the generation of complex and diverse neurite

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2018 Journal of developmental biology

89. SMMDB: a web-accessible database for small molecule modulators and their targets involved in neurological diseases (PubMed)

status, their 2D and three-dimensional structures etc. To enrich the basic annotation of every small molecule entry present in SMMDB, it is hyperlinked to their description present in PubChem, DrugBank, PubMed and KEGG database. The annotation about their molecular targets was enriched by linking it with UniProt and GenBank and STRING database that can be utilized to study the interaction and relation between various targets involved in single neurological disease. All molecules present in the SMMDB (...) SMMDB: a web-accessible database for small molecule modulators and their targets involved in neurological diseases High-throughput screening and better understanding of small molecule's structure-activity relationship (SAR) using computational biology techniques have greatly expanded the face of drug discovery process in better discovery of therapeutics for various disease. Small Molecule Modulators Database (SMMDB) includes >1100 small molecules that have been either approved by US Food

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2018 Database: The Journal of Biological Databases and Curation

90. NeurEx: digitalized neurological examination offers a novel high‐resolution disability scale (PubMed)

NeurEx: digitalized neurological examination offers a novel high‐resolution disability scale To develop a sensitive neurological disability scale for broad utilization in clinical practice.We employed advances of mobile computing to develop an iPad-based App for convenient documentation of the neurological examination into a secure, cloud-linked database. We included features present in four traditional neuroimmunological disability scales and codified their automatic computation (...) . By combining spatial distribution of the neurological deficit with quantitative or semiquantitative rating of its severity we developed a new summary score (called NeurEx; ranging from 0 to 1349 with minimal measurable change of 0.25) and compared its performance with clinician- and App-computed traditional clinical scales.In the cross-sectional comparison of 906 neurological examinations, the variance between App-computed and clinician-scored disability scales was comparable to the variance between rating

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2018 Annals of clinical and translational neurology

91. Pilot Study of Cognitive Behavioral Therapy-Informed Physical Therapy Intervention in Functional Neurological Disorders

Behavioral Therapy-Informed Physical Therapy Intervention in Functional Neurological Disorders Estimated Study Start Date : March 8, 2019 Estimated Primary Completion Date : January 2, 2024 Estimated Study Completion Date : January 2, 2024 Resource links provided by the National Library of Medicine related topics: resources: Groups and Cohorts Go to Group/Cohort Intervention/treatment Motor Functional Neurological Disorder The cohort will consist of patients with clinically-established motor functional (...) Pilot Study of Cognitive Behavioral Therapy-Informed Physical Therapy Intervention in Functional Neurological Disorders Pilot Study of Cognitive Behavioral Therapy-Informed Physical Therapy Intervention in Functional Neurological Disorders - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum

2018 Clinical Trials

92. Do Headache Patients Require More Care in Between Visits Than Other Neurology Outpatients? (PubMed)

clinics.Patients in a headache clinic in an academic tertiary care setting require a high intensity of remote outpatient care, more so than patients in other neurology subspecialty clinics and general neurology clinic, with the exception of the neuroimmunology/MS clinic. This is to a large extent secondary to the very frequent use of secure email linked to the electronic medical record by headache patients.© 2018 American Headache Society. (...) Do Headache Patients Require More Care in Between Visits Than Other Neurology Outpatients? There is evidence that time spent in patient care in between patient visits is increasing and a contributor to physician burnout. The extent of this work on providers in the field of headache medicine is unknown.To establish whether headache outpatients require a high level of care in addition to clinic visits, based on the quantity of remote encounters per patient (phone calls and secure email

2018 Headache

93. ER Dynamics and Derangement in Neurological Diseases (PubMed)

ER Dynamics and Derangement in Neurological Diseases The endoplasmic reticulum (ER) is a morphologically dynamic organelle containing different membrane subdomains with distinct cellular functions. Numerous observations have revealed that ER stress response induced by disturbed ER homeostasis is linked to various neurological/neurodegenerative disorders. In contrast, recent findings unveil that ER structural derangements are linked to the progression of several neurological diseases (...) . The derangements involve two distinct, and likely opposing pathways. One is dysfunction of ER dynamics machinery, leading to disruption of ER network organization. Another one is facilitation of pre-existing machinery, leading to generation of markedly-ordered de novo membranous structure. Restoring the ER network can be the effective way toward the cure of ER-deranged neurological disorders.

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2018 Frontiers in neuroscience

94. An investigation into closed-loop treatment of neurological disorders based on sensing mitochondrial dysfunction (PubMed)

An investigation into closed-loop treatment of neurological disorders based on sensing mitochondrial dysfunction Dynamic feedback based closed-loop medical devices offer a number of advantages for treatment of heterogeneous neurological conditions. Closed-loop devices integrate a level of neurobiological feedback, which allows for real-time adjustments to be made with the overarching aim of improving treatment efficacy and minimizing risks for adverse events. One target which has not been (...) extensively explored as a potential feedback component in closed-loop therapies is mitochondrial function. Several neurodegenerative and psychiatric disorders including Parkinson's disease, Major Depressive disorder and Bipolar disorder have been linked to perturbations in the mitochondrial respiratory chain. This paper investigates the potential to monitor this mitochondrial function as a method of feedback for closed-loop neuromodulation treatments. A generic model of the closed-loop treatment

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2018 Journal of neuroengineering and rehabilitation

95. The spectrum of neurological disease associated with Zika and chikungunya viruses in adults in Rio de Janeiro, Brazil: A case series (PubMed)

The spectrum of neurological disease associated with Zika and chikungunya viruses in adults in Rio de Janeiro, Brazil: A case series During 2015-16 Brazil experienced the largest epidemic of Zika virus ever reported. This arthropod-borne virus (arbovirus) has been linked to Guillain-Barré syndrome (GBS) in adults but other neurological associations are uncertain. Chikungunya virus has caused outbreaks in Brazil since 2014 but associated neurological disease has rarely been reported here. We (...) investigated adults with acute neurological disorders for Zika, chikungunya and dengue, another arbovirus circulating in Brazil.We studied adults who had developed a new neurological condition following suspected Zika virus infection between 1st November 2015 and 1st June 2016. Cerebrospinal fluid (CSF), serum, and urine were tested for evidence of Zika, chikungunya, and dengue viruses.Of 35 patients studied, 22 had evidence of recent arboviral infection. Twelve had positive PCR or IgM for Zika, five

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2018 PLoS neglected tropical diseases

96. Rett syndrome: a neurological disorder with metabolic components (PubMed)

Rett syndrome: a neurological disorder with metabolic components Rett syndrome (RTT) is a neurological disorder caused by mutations in the X-linked gene methyl-CpG-binding protein 2 (MECP2), a ubiquitously expressed transcriptional regulator. Despite remarkable scientific progress since its discovery, the mechanism by which MECP2 mutations cause RTT symptoms is largely unknown. Consequently, treatment options for patients are currently limited and centred on symptom relief. Thought (...) to be an entirely neurological disorder, RTT research has focused on the role of MECP2 in the central nervous system. However, the variety of phenotypes identified in Mecp2 mutant mouse models and RTT patients implicate important roles for MeCP2 in peripheral systems. Here, we review the history of RTT, highlighting breakthroughs in the field that have led us to present day. We explore the current evidence supporting metabolic dysfunction as a component of RTT, presenting recent studies that have revealed

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2018 Open biology

97. Neurological signs as early determinants of dementia and predictors of mortality among older adults in Latin America: a 10/66 study using the NEUROEX assessment. (PubMed)

in the baseline assessment of the 10/66 study and living in Cuba, Dominican Republic, Peru, Venezuela and Mexico were analysed. Exploratory and confirmatory analysis were used to explore dimensionality of neurological symptoms. Poisson regression analyses were used to link groups of neurological signs with dementia at baseline. Cox hazard regression models were used to explore the predictive validity of neurological signs with mortality at follow up.Exploratory and confirmatory factor analyses revealed four (...) , linking neurological and gait impairments with dementia at baseline, and with mortality at follow up among older adults living in five Latin American countries.

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2018 BMC Neurology

98. Medico-economic Impact Evaluation in Telemedecine Follow-up in Complement of a Mobile Team in Patients With Neurological Disabilities (HANDI@CCESS)

disabilities is a current public health concern. New technological tools such as telemedicine can bring expertise to the place of life of people while promoting the city-hospital link. Condition or disease Intervention/treatment Phase Severe Neurological Disabilities Other: Telemedecine follow-up after an intervention at home of the mobile team Other: Mobile team follow-up after an intervention at home of the mobile team Not Applicable Study Design Go to Layout table for study information Study Type (...) Medico-economic Impact Evaluation in Telemedecine Follow-up in Complement of a Mobile Team in Patients With Neurological Disabilities (HANDI@CCESS) Medico-economic Impact Evaluation in Telemedecine Follow-up in Complement of a Mobile Team in Patients With Neurological Disabilities (HANDI@CCESS) - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies

2018 Clinical Trials

99. Emotional processing in functional neurological disorder: a review, biopsychosocial model and research agenda. (PubMed)

Emotional processing in functional neurological disorder: a review, biopsychosocial model and research agenda. Functional neurological disorder (FND) is a common and highly disabling disorder, but its aetiology remains enigmatic. Conceptually, there has been reduced emphasis on the role of psychosocial stressors in recent years, with a corresponding increase in neurobiological explanations. However, a wealth of evidence supports the role of psychosocial adversities (eg, stressful life events (...) , interpersonal difficulties) as important risk factors for FND. Therefore, there is a need to integrate psychosocial (environmental) and neurobiological factors (eg, sensorimotor and cognitive functions) in contemporary models of FND. Altered emotional processing may represent a key link between psychosocial risk factors and core features of FND. Here, we summarise and critically appraise experimental studies of emotional processing in FND using behavioural, psychophysiological and/or neuroimaging measures

2018 Neurosurgery and Psychiatry

100. Association of copeptin, a surrogate marker of arginine vasopressin, with cerebral vasospasm and delayed ischemic neurologic deficit after aneurysmal subarachnoid hemorrhage. (PubMed)

Association of copeptin, a surrogate marker of arginine vasopressin, with cerebral vasospasm and delayed ischemic neurologic deficit after aneurysmal subarachnoid hemorrhage. OBJECTIVEDelayed ischemic neurological deficit (DIND) is a leading cause of mortality and morbidity after aneurysmal subarachnoid hemorrhage (aSAH). Arginine vasopressin (AVP) is a hormone released by the posterior pituitary. It is known to cause cerebral vasoconstriction and has been implicated in hyponatremia secondary (...) to the syndrome of inappropriate antidiuretic hormone secretion. Direct measurement of AVP is limited by its short half-life. Copeptin, a cleavage product of the AVP precursor protein, was therefore used as a surrogate marker for AVP. This study aimed to investigate the temporal relationship between changes in copeptin concentrations and episodes of DIND and hyponatremia.METHODSCopeptin concentrations in cerebrospinal fluid were quantified using enzyme-linked immunosorbent assay in 19 patients: 10 patients

2018 Journal of Neurosurgery

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