关键词： Compressive myelopathy   hypertrophic pachymeningitis   immunoglobulin G4   steroid response   storiform fibrosis  

摘要： Aim: To study the clinical presentation, radiological findings, and therapy responsiveness of patients with biopsy-proven immunoglobulin G4 (IgG4) -related neurological disease. Methods: The study was conducted between January 2016 and March 2018 from the Department of Neurology and Pathology of Nizam's Institute of Medical Sciences. Patients with neurological symptoms and biopsy suggestive of IgG4-related disease (IgG4-RD) were included. These patients were studied for their demographic pattern and clinical presentation. The presence of serological markers such as vasculitic profile and IgG4 levels was analyzed. Radiological findings were studied in detail. Therapeutic agents used and the response to therapy were assessed. Results: There were six cases with IgG4-related neurological disease which were all hypertrophic pachymeningitis. The age ranged from 35 to 64 (mean = 46) years. The clinical presentation was acute in one, subacute in two, and chronic in three patients. The most common presenting symptom was headache (4), followed by gait and/or urinary disturbances (2), paraparesis (1), and diplopia (1). IgG4 levels were elevated in 50% of them. Pseudotumor-like mass and sinovenous thrombosis, not described previously, were seen in one patient. All the patients were treated with oral or intravenous steroid. Rituximab was given in three patients;azathioprine was the steroid-sparing agent in one patient. Those with acute/subacute onset of presentation had an excellent response to steroids. All the patients with a chronic duration of their symptoms received empirical anti-tuberculous therapy before a definitive diagnosis of Ig G4-RD was made. Conclusions: The characterization of patients with IgG4-related neurological disease based on the understanding of the clinical spectrum increases the confidence in the clinician to resort to early immunosuppression, thereby having prognostic implications.

摘要： In a randomised trial of 12 patients with Parkinson's disease who had excessive daytime sleepiness, sodium oxybate significantly reduced sleepiness compared with placebo, with a moderate effect on mean sleep latency in the objective multiple sleep latency test (increase of 2·9 min [95% CI 2·1 to 3·8]; p=0·002) and the subjective Epworth Sleepiness Scale score (–4·2 points [95% CI −5·3 to −3·0]; p=0·001).3 Moreover, sodium oxybate enhanced sleep itself as measured by increased slow-wave sleep duration (increase of 72·7 min [95% CI 55·7 to 89·7]; p<0·001).In elderly people without dementia, baseline excessive daytime sleepiness was significantly associated with increased amyloid β accumulation in the cingulate cortex and the parietal-precuneus lobe.5 These findings are important because elderly patients with excessive daytime sleepiness might be more vulnerable to amyloid β-associated neurodegeneration, and early identification and treatment of the sleep abnormality might slow down the accumulation of amyloid β in the brain.5 Disrupted nocturnal sleep or sleep deprivation can lead to increased amyloid β concentrations in CSF and deposition in the brain,6 but does not seem to be associated with genetic risk (APOE genotype) for Alzheimer's disease.7 These findings on the adverse consequences of disrupted nocturnal sleep and sleep deprivation on amyloid β accumulation in the brain point towards disrupted sleep as an independent risk factor in Alzheimer's disease pathogenesis.6,7 Abnormal movements during REM sleep are part of the symptomatology of REM behavioural sleep disorder and can be a prodromal symptom in α-synuclein neurodegenerative *** an elegant physiological study in mice, glutamatergic neurons in the paraventricular thalamus were shown to have an important role in the regulation of the transition from sleep to wakefulness.11 Neuronal projections from the nucleus accumbens and hypocretin neurons to the paraventricular thalamus were important to maintai

关键词： complication   injection pressure   monitor   nerve injury   regional anaesthesia  

摘要： No medical intervention is without risk, and even though generally considered safe, this also holds true for peripheral nerve blocks. Despite their clinical benefits in a variety of settings of acute and chronic pain 1, clinicians continue to be rightfully wary of nerve damage, which is thought to occur in less than 1 in 2000 patients 2. Commonly accepted aetiologies for peri‐operative nerve injury include nerve ischaemia, intraneural bleeding, compression, direct trauma and stretch 3. When nerve blocks are specifically considered, mechanical needle and injection trauma, local anaesthetic neurotoxicity, and injection pressure can contribute 4, 5. Preclinical evidence has suggested that increased injection pressure might indicate intraneural or even intrafascicular (dangerous) needle‐tip position 6. In bench studies, where injection force was measured using an in‐line manometer, Claudio et al. reported that anaesthetists were prone to use more force during injection than considered safe and that the syringe‐feel method of detecting might be unreliable 7. This has led to the market introduction of and evaluation of devices designed to detect or avoid high injection pressures 8, 9.

摘要： Prenatal diagnosis of congenital cytomegalovirus (CMV) infection is based on amniocentesis performed after 21 weeks of gestation and 7 weeks after maternal infection. However, recently, congenital CMV infection after a negative amniocentesis result has been reported. Our aim was to determine the incidence and neurological outcome of congenital CMV infection following a negative amniocentesis result.

关键词： Paraneoplastic   Neurologic   Syndrome   Presentation   Treatment   Outcome  

摘要： BackgroundGiven its rare incidence, there are few epidemiological case series on paraneoplastic neurologic syndromes (PNS).MethodsWe present a 10-year series compiled in the Section of Neuro-Oncology, Yale Cancer Center between 2002 and ***-five cases met the PNS Euro-network criteria for definitive PNS. Most (64%;16/25) had no known neoplasm. Cerebrospinal fluid pleocytosis declined logarithmically over time. Neuroimaging abnormalities were seen in 88% of cases (15/17), but with delayed onset. Therapeutic benefit correlated strongly to pre-treatment modified Rankin Scale (mRS) (p<0.01), but not with time elapsed between syndrome onset to treatment (p=0.8), first immunotherapy modality (corticosteroids: n=10;IVIG: n=10;PLEX: n=3;p=0.37), or number of immunotherapy modalities provided (p=0.17). PNS-related mortality was high (24%;6/25). Nonetheless, 16% (3/18;7 living patients censored) survived over 6 times the anticipated median expected by tumor type and *** are rare, at an estimated incidence of 3.1 cases per million-person-years. Detection of CSF pleocytosis and MRI abnormalities depend on time of analysis. While PNS-related mortality was high, immunotherapy benefit correlated strongly with pre-treatment mRS and long-term survival is possible.

关键词： Rehabilitation   Neurological disability   Natural environment   Psychosocial health   Systematic review  

摘要： Engaging with the natural environment contributes to favourable psychosocial health outcomes. A systematic review of research published before June 2017 was conducted to establish how engaging with natural environments impact the psychosocial health of people with a neurological disability. Identified studies included populations with dementia (n = 14), brain injury (n = 2), and stroke (n = 2). Evidence suggests that engaging with gardens, and gardening, can favourably impact the emotional and social health of people with dementia and, an explanatory theoretical model is proposed. Considerable research gaps exist and further research investigating the psychosocial impact of engaging with natural environments for people with different neurological conditions (for example spinal cord injury or stroke) is warranted.

关键词： Dopamine pathway   Neurotransmitter   Central nervous system   Neurodegenerative diseases  

摘要： The dopaminergic system plays important roles in neuromodulation, such as motor control, motivation, reward, cognitive function, maternal, and reproductive behaviors. Dopamine is a neurotransmitter, synthesized in both central nervous system and the periphery, that exerts its actions upon binding to G protein-coupled receptors. Dopamine receptors are widely expressed in the body and function in both the peripheral and the central nervous systems. Dopaminergic signaling pathways are crucial to the maintenance of physiological processes and an unbalanced activity may lead to dysfunctions that are related to neurodegenerative diseases. Unveiling the neurobiology and the molecular mechanisms that underlie these illnesses may contribute to the development of new therapies that could promote a better quality of life for patients worldwide. In this review, we summarize the aspects of dopamine as a catecholaminergic neurotransmitter and discuss dopamine signaling pathways elicited through dopamine receptor activation in normal brain function. Furthermore, we describe the potential involvement of these signaling pathways in evoking the onset and progression of some diseases in the nervous system, such as Parkinson's, Schizophrenia, Huntington's, Attention Deficit and Hyperactivity Disorder, and Addiction. A brief description of new dopaminergic drugs recently approved and under development treatments for these ailments is also provided.

摘要： AimTo determine whether maternal history of recurrent pregnancy loss (RPL) impacts on the long-term neurological health of the offspring. MethodA population-based cohort analysis was performed, including all singletons born between1991 and 2014 at a single regional tertiary *** morbidity up to age 18years (including cognitive, motor, and psychiatric disorders) in children born to mothers with and without a history of RPL was compared. Cumulative neurological morbidity incidence was compared with survival curves and a Weibull multivariable survival model to control for follow-up time and relevant confounders. ResultsA total of 242187 deliveries met the inclusion criteria during the study period, 5% (n=12182) of which were in mothers with RPL. Epilepsy and developmental disorders were significantly more common in the group with RPL(0.95 vs 0.74/1000 person-years [p=0.009] and 0.22 vs 0.09/1000 person-years [p<0.001]). The survival curves demonstrated significantly higher cumulative incidences of epilepsy and developmental disorders in the group with RPL. The multivariable model exhibited an independent association between maternal RPL and childhood epilepsy (adjusted hazard ratio 1.23;95% confidence interval 1.01-1.50) and developmental disorders in the offspring (adjusted hazard ratio 2.41;95% confidence interval 1.60-3.64). InterpretationA history of maternal RPL appears to be independently associated with long-term neurological morbidity of the offspring.

关键词： telemedicine   telehealth   healthcare access   rural  

摘要： Over the past 2 decades, various telehealth technologies, in particular synchronous video teleconferencing between provider and patient, have been incorporated into the practice of neurology. The practice of child neurology is now starting to take advantage of these rapidly evolving resources. This review describes the evolution of tele-neurology, starting with adult tele-stroke services and expanding to the management of both adults and children with a variety of chronic neurologic disorders including epilepsy, headache, movement disorders, and neurodevelopmental disabilities. Resources required for the development and sustainment of a child neurology telemedicine program are discussed together with requirements for licensure, and credentialing, and the importance of educating current and future neurology practitioners in how to provide this clinical service.

关键词： ATTENTION   ATTITUDES toward health   NEUROLOGICAL disorders   NEUROBIOLOGY   REINFORCEMENT (Psychology)   STRESS (Psychology)   ATTITUDES toward illness  

摘要： Functional neurological symptom disorder (FNSD) is characterized by neurological symptoms that are unexplained by other traditional neurological or medical conditions. Both physicians and patients have limited understanding of FNSD, which is often explained as a physical manifestation of psychological distress. Recently, diagnostic criteria have shifted from requiring a preceding stressor to relying on positive symptoms. Given this shift, we have provided a review of the etiology of FNSD. Predisposing factors include trauma or psychiatric symptoms, somatic symptoms, illness exposure, symptom monitoring and neurobiological factors. Neurobiological research has indicated that patients with FNSD have a decreased sense of agency and abnormal attentional focus on the affected area, both of which are modulated by beliefs and expectations about illness. Sick role and secondary gain may reinforce and maintain FNSD. The integrated etiological summary model combines research from various fields and other recent etiological models to represent the current understanding of FNSD etiology. It discusses a potential causal mechanism and informs future research and treatment.