关键词： Paraneoplastic syndromes   Motor neuron syndrome   Amyotrophic lateral sclerosis   Anti-Ma   Myelopathy   Radiculopathy  

摘要： ObjectiveTo present clinical, radiological, and pathological features of a cohort of patients with motor neuron involvement in association with anti-Ma2 antibodies (Ma2-Ab).MethodsRetrospective case-series of patients with definite paraneoplastic neurological syndrome (PNS) and Ma2-Ab, and cases identified from a review of the *** 33 Ma2-Ab patients referred between 2002 and 2016, we retrospectively identified three patients (9.1%) with a motor neuron syndrome (MNS). Seven additional cases were retrieved among the 75 Ma2-patients reported in the literature (9.3%). A total of ten patients are, therefore, described herein. MNS was evident as combined upper and lower MNS in four patients, isolated upper MNS in two, and isolated lower MNS in one;three patients were diagnosed with myeloradiculopathy. The most common MNS signs/symptoms were: hyperreflexia (80%), proximal weakness (60%), proximal upper-limb fasciculations (50%), head drop (40%), and dysarthria/dysphagia (30%). Brain MRI abnormalities included bilateral pyramidal tract T2-weighted/FLAIR hyperintensities (three patients). Spine MRI found bilateral, symmetric, T2-weighted signal abnormalities in the anterior horn in two patients. CSF examination was abnormal in nine patients. Cancer was found in seven patients (four testicular, two lung, and one mesothelioma). Eight patients underwent first-line immunotherapy. Second-line immunotherapy was adopted in all our patients and in none of those identified in the literature. Motor improvement was observed in 33% of our patients, and 20% in the literature *** neuron involvement could complicate Ma2-Ab-associated PNS in almost 10% of patients and must be carefully studied to adapt treatment. This disorder differs from amyotrophic lateral sclerosis.

关键词： Functional neurosurgery   History   Mexico  

摘要： From the predominance of the pre-Columbian cultures to the present, an interest has existed in Mexico in brain structure and function, in both normal and pathological conditions. This interest has resulted in both medical and surgical attempts to treat some neurological conditions. Eighty years ago, with the influence of American and European neurosurgeons, modern Mexican neurosurgery emerged. Since then, it has progressively grown and diversified into several high specialties. At present, functional neurosurgery in Mexico constitutes, similar to that in other countries, a recognized surgical subspecialty that provides valuable information to treat brain dysfunctions such as movement disorders, pain, epilepsy, and other conditions. In the present report, we have briefly described the main historical aspects of the emergence and development of functional neurosurgery in Mexico.

关键词： Amyotrophic lateral sclerosis   Drug delivery   Neural stem cells   Neurological models  

摘要： Despite decades of research, pharmacological therapies for spinal cord motor pathologies are limited. Alternatives using macromolecular, viral, or cell-based therapies show early promise. However, introducing these substances into the spinal cord, past the blood-brain barrier, without causing injury is challenging. We describe a technique for intraspinal injection targeting the lumbar ventral horn in rodents. This technique preserves motor performance and has a proven track record of translation into phase 1 and 2 clinical trials in amyotrophic lateral sclerosis (ALS) patients. The procedure, in brief, involves exposure of the thoracolumbar spine and dissection of paraspinous muscles over the target vertebrae. Following laminectomy, the spine is affixed to a stereotactic frame, permitting precise and reproducible injection throughout the lumbar spine. We have used this protocol to inject various stem cell types, primarily human spinal stem cells (HSSCs);however, the injection is adaptable to any candidate therapeutic cell, virus, or macromolecule product. In addition to a detailed procedure, we provide stereotactic coordinates that assist in targeting of the lumbar spine and instructional videos. The protocol takes similar to 2 h per animal.

关键词： Alkaline phosphatase   Hypophosphatasia   Neurological symptoms   PLP   TNSALP   Vitamin B6  

摘要： Hypophosphatasia (HPP) typically manifests with fractures, tooth loss, and muscle pain. Although mental health diagnoses and neurological symptoms have not been previously well documented in HPP, they occur commonly. The recognition of non-traditional symptoms may improve patient satisfaction, preempt costly evaluation and misdiagnosis, and lead to further treatment *** (HPP) is an inborn error of metabolism due to deficiency of tissue non-specific alkaline phosphatase (TNSALP). It is traditionally characterized by rickets in children and osteomalacia in adults, along with fractures, tooth loss, and muscle pain. Neurological symptoms and mental health diagnoses have not been widely reported, and we therefore report their prevalence in a cohort of patients with *** retrospective chart review was performed on a series of 82 HPP patients. Patient charts were reviewed to identify the possible presence and onset of 13 common neurological *** age was 36years (2 to 79). Seventeen had adult onset HPP (>18years) and 65 had pediatric onset HPP (<18years). Median time from symptom onset to HPP diagnosis was 8years (0 to 67). Seventy-four percent had a family history of bone disease, while 17% had a family history of neurologic disease. Bone problems occurred in 89%, dental problems in 77%, and muscle problems in 66%. Fatigue occurred in 66%, headache in 61%, sleep disturbance in 51%, gait change in 44%, vertigo in 43%, depression in 39%, anxiety in 35%, neuropathy in 35%, and hearing loss in 33%.ConclusionsThe extra-skeletal manifestations of HPP, specifically neurological symptoms, have not been previously well documented. However, mental health diagnoses and neurological symptoms such as headache and sleep disturbance occur commonly in patients with HPP. The recognition of non-traditional symptoms in HPP may improve patient satisfaction, preempt costly evaluation and misdiagnosis, and may lead to further treatment

摘要： Aim This study aimed to review the psychometric properties and clinical application of parent-report instruments that assess feeding difficulties in children with neurological impairments. Method Papers were identified through five electronic databases based on 15 keywords and were included if they met the following criteria: published in English, described the implementation of parent-report instruments, and included children with neurological impairments (either in the report or a related study population). Results In total, 1220 relevant abstracts were screened and 22 full-text articles were evaluated. The following six parent-report instruments met the inclusion criteria: (1) Screening Tool of Feeding Problems applied to children, (2) Paediatric Eating Assessment Tool, (3) Paediatric Assessment Scale for Severe Feeding Problems, (4) Montreal Children's Hospital Feeding Scale, (5) Children's Eating Behaviour Inventory, and (6) Behavioural Paediatric Feeding Assessment Scale (BPFAS). Based on comprehensive psychometric testing and consistently good results, the BPFAS was considered the most valid and reliable instrument. The BPFAS also showed good clinical applicability because it was readily available, required a short administration time, and used a simple scoring system. Interpretation We reviewed the available parent-report instruments for assessing feeding difficulties in children with neurological impairments. The BPFAS had the best psychometric properties and clinical applicability. What this paper adds Six parent-report instruments were suitable for assessing feeding in children with neurological impairments. The Behavioural Paediatric Feeding Assessment Scale (BPFAS) has the strongest psychometric properties. The BPFAS also has good clinical applicability.

关键词： Nervous System Diseases   Autophagy  

关键词： economic evaluation   neurological disease   stem cells   stroke  

摘要： Objectives: To examine economic evaluation studies of stem cell therapies (SCTs) in neurological disorders and to provide an overview of the quality of the economic evidence available on this topic. Methods: The review examined studies that performed an economic evaluation of the use of stem cells in adult patients with neurological diseases and that were published in English during the period 2007 to 2017. Data analyzed and reported included study population, disease indication, main analytical approaches for the economic analysis and perspective, key assumptions made or tested in sensitivity analyses, cost outcomes, estimates of incremental cost effectiveness, and approaches to quantifying decision uncertainty. Results: A total of three studies reporting on the findings of the economic evaluation of the use of SCT in stroke, Parkinson disease, and secondary progressive multiple sclerosis, respectively, were identified. All three studies conducted a cost-utility analysis using decision-analytic models and reported an incremental cost per quality-adjusted life-years gained incremental cost-effectiveness ratio) versus standard care. These studies reported meaningful cost savings in stroke, Parkinson disease, and secondary progressive multiple sclerosis in the base-case scenarios. Conclusions: Despite significant progress in clinical research in the use of SCT in neurological diseases, economic evaluation of these therapies is still at a nascent stage. Given the early stage of research inputs (clinical and cost outcomes data) into the models per se, further research is urgently needed to enable meaningful assessment of the cost effectiveness of these advanced therapies and to ensure sustainable access for population groups most likely to benefit in clinical practice.

关键词： Residency training   Graduate medical education   Primary care   education   Neurology   education  

摘要： ObjectiveA survey of recently certified psychiatrists was conducted to obtain their feedback about the contribution of the primary care and neurology components of residency training to their professional development and to their current needs as *** 22-item survey was developed based on issues discussed at a forum on residency competence requirements and administered electronically to four cohorts of recently certified *** response rate was 17% (1049/6083). Overall, the respondents described both their primary care and neurology experiences as helping them accomplish several goals for their professional development. The majority were satisfied with their primary care training and felt well-prepared to enter practice. The most common suggestions for improving the primary care component were better integration with psychiatry and providing longitudinal experiences and more outpatient experience. They were somewhat less satisfied with their neurology training, and only about half felt well-prepared for the neurologic aspects of psychiatry practice. The most common suggestions for improving neurology training were to provide more time in neurology with experiences that were more relevant to psychiatry such as outpatient and consultation experiences. Some also thought longitudinal experiences would be *** psychiatrists were generally satisfied with the primary care and neurology components of residency training and felt that they had contributed to their professional development. Their suggestions for improvement contribute to the rich discussion among training directors and other psychiatry educators about these components of residency training.

摘要： Table 2 (page 100): The heading 'Drug free (n=98) Medicated (n =35)' should instead read 'Medicated (n=98) Drug free (n=35)'. The authors would like to apologise for any inconvenience caused.