Oral self-inflicted accidental trauma in patients with neurological disorders: a case report of dental management in infants with cerebellar hypoplasia.

Self-inflicted oral injuries, accidental or otherwise, can cause major consequences. Measures need to be taken to protect individuals from chronic self-injurious behaviour; however, there are no official guidelines on the subject. The purpose of this article is to show the case of a 1-year-old patient with neurological disorders who, following the eruption of deciduous teeth, had self-inflicted a traumatic ulcer on his tongue and lower lip. Following a multidisciplinary approach involving several operating units of our hospital to make a diagnosis, an oral device was designed to completely cover the dental elements to prevent recurrence of the trauma and to prevent further worsening of the injuries already caused. The purpose of this work is to demonstrate that although the surgical approach, such as extraction of the dental elements, may be the quickest solution in situations similar to the one presented, the high biological cost and irreversibility of the result lead to seeking alternatives and more conservative solutions such as the one described.

Historical Perspectives on the Neurologic Manifestations of Viral Pandemics.

Neurologic symptoms have been reported in over 30% of hospitalized patients with coronavirus disease 2019 (COVID-19), but the pathogenesis of these symptoms remains under investigation. Here, we place the neurologic complications of COVID-19 within the context of three historical viral pandemics that have been associated with neurologic diseases: (1) the 1918 influenza pandemic, subsequent spread of encephalitis lethargica, and lessons for the study of COVID-19-related neuroinflammation; (2) the controversial link between the 1976 influenza vaccination campaign and Guillain-Barré Syndrome and its implications for the post- and parainfectious complications of COVID-19 and COVID-19 vaccination; and (3) potential applications of scientific techniques developed in the wake of the human immunodeficiency virus pandemic to the study of postacute sequelae of COVID-19.

The Role of Aspiration Amount on Airway Protective Responses in People with Neurogenic Dysphagia.

The aim of this study was to examine relationships between the presence vs. absence of an aspiration-related airway protective response (i.e., coughing or throat clearing) with aspiration amount, trial volume, disease diagnosis, and disease duration in people with neurologic disease. A secondary analysis was completed of flexible endoscopic evaluations of swallowing (FEES) in people with neurologic disease. Thin liquid boluses with endoscopically confirmed aspiration were included. Aspiration amount was measured for each trial using the visual analysis of swallowing efficiency and safety (VASES). Statistical analyses were used to (1) compare aspiration amount between swallows with vs. without an airway protective response and (2) examine if trial volume, disease duration, and disease diagnosis were related to the presence of airway protective responses when controlling for aspiration amount. 422 aspirated swallows across 86 FEES were analyzed. Of the 59 people who aspirated more than once, 66.1% exhibited variability in the presence vs. absence of an airway protective response. Statistical analyses revealed airway protective responses were significantly related to aspiration amount (p < 0.001; Marginal R2 = 0.46) and disease duration (p = 0.036, L.R. = 4.35) but not trial volume (p = 0.428) or disease diagnosis (p = 0.103). The participants in this study were less likely to cough or throat clear when having smaller amounts of aspiration or longer disease durations. Future research is needed to examine if aspiration amount is related to airway protective responses in healthy adults and across other patient populations.

Treatment outcomes and overactive bladder symptoms after midurethral sling in female patients with or without various neurological diseases.

AIMS: We investigated surgical outcomes and changes of overactive bladder (OAB) symptoms after midurethral sling in female stress urinary incontinence (SUI) patients with or without neurological disease (ND) without spinal cord injury. METHODS: Patients who underwent midurethral sling for SUI between January 2009 and December 2018 were reviewed. Postoperative changes in OAB symptoms (de novo occurrence or resolution) within 1 year were compared in each preoperative symptom subset with a 1:1 matched analysis between non-neurological disease (NND) and ND group. RESULTS: A total of 855 patients (median follow-up: 49.8 months; mean age: 57.9 ± 9.3 years) were included. Successful SUI correction was achieved in 95.0% of NND and 93.7% of ND patients (p = .440). Among 797 patients (711 NND and 86 ND) without remnant SUI, 227 had SUI only, 198 had SUI with urgency, and 372 had mixed urinary incontinence (MUI) preoperatively. The ND patients tended to be older (62.8 ± 9.2 vs. 57.2 ± 9.0 years) and had higher proportions of diabetes (24.4% vs. 8.0%), hypertension (47.7% vs. 26.7%), and MUI (64.0% vs. 44.6%) than NND patients (p < .001, respectively). After matching age, diabetes, and hypertension, the incidence of de novo OAB was higher in ND patients (SUI only; 21.1% vs. 5.3%; p < .001) while resolution rates of urgency urinary incontinence (UUI) were similar (MUI; 57.7% vs. 53.9%; p = .414). CONCLUSIONS: Success rates of midurethral sling and resolution of UUI were comparable between NND and ND patients. In patients with neurological conditions, de novo OAB symptoms were more likely to develop.

Transition from paediatric to adult care in adolescents with neurological diseases and handicap.

The transfer of adolescents from paediatric care to adult health facilities is often difficult for the patients and their families and can lead to a breakdown in medical follow-up and therefore serious complications. Existing recommendations for the successful transition of patients with chronic disorders do not specifically address patients with handicap. Preparations for the transfer must be made well in advance. They must aim to achieve the autonomisation of adolescents by making them responsible and providing them with the knowledge that will enable them to manage their care themselves, the know-how to react appropriately if there is any change in their condition, and to move comfortably within the adult health system. This requires the active participation of the patient, his or her family and the paediatric and adult care teams. It involves multidisciplinary management plus the production and maintenance of an educational therapy programme. Finally, the identification of doctors trained in handicap, relevant sub-specialists and even expert patients could enable improvements in the continuum of complete and appropriate care for these patients within adult medicine.

Updated European Heart Rhythm Association practical guide on the use of non-vitamin-K antagonist anticoagulants in patients with non-valvular atrial fibrillation: Executive summary.

In 2013, the European Heart Rhythm Association (EHRA) published a Practical Guide on the use of non-VKA oral anticoagulants (NOACs) in patients with atrial fibrillation (AF) (Heidbuchel H, Verhamme P, Alings M, Antz M, Hacke W, Oldgren J, Sinnaeve P, Camm AJ, Kirchhof P, European Heart Rhythm A. European Heart Rhythm Association Practical Guide on the use of new oral anticoagulants in patients with non-valvular atrial fibrillation. Europace 2013;15:625-651; Heidbuchel H, Verhamme P, Alings M, Antz M, Hacke W, Oldgren J, Sinnaeve P, Camm AJ, Kirchhof P. EHRA practical guide on the use of new oral anticoagulants in patients with non-valvular atrial fibrillation: executive summary. Eur Heart J 2013;34:2094-2106). The document received widespread interest, not only from cardiologists but also from neurologists, geriatricians, and general practitioners, as became evident from the distribution of >350 000 copies of its pocket version (the EHRA Key Message Booklet) world-wide. Since 2013, numerous new studies have appeared on different aspects of NOAC therapy in AF patients. Therefore, EHRA updated the Practical Guide, including new information but also providing balanced guiding in the many areas where prospective data are still lacking. The outline of the original guide that addressed 15 clinical scenarios has been preserved, but all chapters have been rewritten. Main changes in the Update comprise a discussion on the definition of 'non-valvular AF' and eligibility for NOAC therapy, inclusion of finalized information on the recently approved edoxaban, tailored dosing information dependent on concomitant drugs, and/or clinical characteristics, an expanded chapter on neurologic scenarios (ischaemic stroke or intracranial haemorrhage under NOAC), an updated anticoagulation card and more specifics on start-up and follow-up issues. There are also many new flow charts, like on appropriate switching between anticoagulants (VKA to NOAC or vice versa), default scenarios for acute management of coronary interventions, step-down schemes for long-term combined antiplatelet-anticoagulant management in coronary heart disease, management of bleeding, and cardioversion under NOAC therapy. The Updated Guide is available in full in EP Europace (Heidbuchel H, Verhamme P, Alings M, Antz M, Diener HC, Hacke W, Oldgren J, Sinnaeve P, Camm AJ, Kirchhof P, Advisors. Updated European Heart Rhythm Association Practical Guide on the use of non-vitamin K antagonist anticoagulants in patients with non-valvular atrial fibrillation. Europace 2015;17:1467-1507), while additional resources can be found at the related ESC/EHRA website (www.NOACforAF.eu).

Treatment of perinatal viral infections to improve neurologic outcomes.

Viral infections in the fetus or newborn often involve the central nervous system (CNS) and can lead to significant morbidity and mortality. Substantial progress has been made in identifying interventions decreasing adverse neurodevelopmental outcomes in this population. This review highlights progress in treatment of important viruses affecting the CNS in these susceptible hosts, focusing on herpes simplex virus (HSV), cytomegalovirus (CMV), human immunodeficiency virus (HIV), and enteroviruses. The observation that high-dose acyclovir improves mortality in neonatal HSV disease culminated decades of antiviral research for this disease. More recently, prolonged oral acyclovir was found to improve neurologic morbidity after neonatal HSV encephalitis. Ganciclovir, and more recently its oral prodrug valganciclovir, is effective in improving hearing and neurodevelopment after congenital CMV infection. Increasing evidence suggests early control of perinatal HIV infection has implications for neurocognitive functioning into school age. Lastly, the antiviral pleconaril has been studied for nearly two decades for treating severe enteroviral infections, with newer data supporting a role for this drug in neonates. Identifying common mechanisms for pathogenesis of viral CNS disease during this critical period of brain development is an important research goal, highlighted by the recent emergence of Zika virus as a potential cause of fetal neurodevelopmental abnormalities.

Neuropsychological Assessment: Past and Future.

Neuropsychological assessment tools are the staple of our field. The development of standardized metrics sensitive to brain-behavior relationships has shaped the neuropsychological questions we can ask, our understanding of discrete brain functions, and has informed the detection and treatment of neurological disorders. We identify key turning points and innovations in neuropsychological assessment over the past 40-50 years that highlight how the tools used in common practice today came to be. Also selected for emphasis are several exciting lines of research and novel approaches that are underway to further probe and characterize brain functions to enhance diagnostic and treatment outcomes. We provide a brief historical review of different clinical neuropsychological assessment approaches (Lurian, Flexible and Fixed Batteries, Boston Process Approach) and critical developments that have influenced their interpretation (normative standards, cultural considerations, longitudinal change, common metric batteries, and translational assessment constructs). Lastly, we discuss growing trends in assessment including technological advances, efforts to integrate neuropsychology across disciplines (e.g., primary care), and changes in neuropsychological assessment infrastructure. Neuropsychological assessment has undergone massive growth in the past several decades. Nonetheless, there remain many unanswered questions and future challenges to better support measurement tools and translate assessment findings into meaningful recommendations and treatments. As technology and our understanding of brain function advance, efforts to support infrastructure for both traditional and novel assessment approaches and integration of complementary brain assessment tools from other disciplines will be integral to inform brain health treatments and promote the growth of our field. (JINS, 2017, 23, 778-790).

Segmental spinal dysgenesis: a report of early surgical intervention and outcome in a 14-month-old child.

Segmental spinal dysgenesis is a rare spinal deformity that is the result of failure of formation resulting in some cases spondyloptosis and neurological dysfunction usually at the thoracolumbar junction. There is little known concerning surgical intervention and timing in these patients. The goal of this case report is to present a case involving 14 months old diagnosed with segmental spinal dysgenesis with stenosis at the thoracolumbar junction soon after birth, treated with definitive posterior spinal fusion and subsequent follow-up.

Memory outcomes following cognitive interventions in children with neurological deficits: A review with a focus on under-studied populations.

Given the primary role of memory in children's learning and well-being, the aim of this review was to examine the outcomes of memory remediation interventions in children with neurological deficits as a function of the affected memory system and intervention method. Fifty-seven studies that evaluated the outcome of memory interventions in children were identified. Thirty-four studies met the inclusion criteria, and were included in a systematic review. Diverse rehabilitation methods for improving explicit and implicit memory in children were reviewed. The analysis indicates that teaching restoration strategies may improve, and result in the generalisation of, semantic memory and working memory performance in children older than 7 years with mild to moderate memory deficits. Factors such as longer protocols, emotional support, and personal feedback contribute to intervention efficacy. In addition, the use of compensation aids seems to be highly effective in prospective memory tasks. Finally, the review unveiled a lack of studies with young children and the absence of group interventions. These findings point to the importance of future evidence-based intervention protocols in these areas.

Managing Pulmonary Complications in Children with Neurodisabilities.

The pulmonary service at ACH is available 24 hours a day for the primary care physician who has the potential to be an important member of the team caring for these children. Initial evaluation and management utilizing the concepts presented in this article may allow the acute illness to be handled at home without the need for referral to ACH. When that is accomplished, not only does the child benefit but the entire family benefits by not having the total disruption that occurs with emergent trips to ACH and especially when hospitalization ensues. In this way the primary care physician provides a very important service not only to the sick child but also to the entire family unit. Thus the role of the primary care physician takes on special significance in the overall management of the patient.

[Preventing swallowing disorders in neurological patients]. / Prévenir les troubles de la déglutition chez les patients présentant des pathologies neurologiques.

Swallowing disorders in neurological rehabilitation are common and important as they can have harmful consequences. A multi-disciplinary hospital team was created to study ways of preventing their occurrence. This article presents the areas to focus on and the main orientations of patient management.

[Neurological sleep disorders]. / Neurologisch bedingte Schlafstörungen.

Neurological sleep disorders are common in the general population and may have a strong impact on quality of life. General practitioners play a key role in recognizing and managing sleep disorders in the general population. They should therefore be familiar with the most important neurological sleep disorders. This review provides a comprehensive overview of the most prevalent and important neurological sleep disorders, including Restless legs syndrome (with and without periodic limb movements in sleep), narcolepsy, NREM- and REM-sleep parasomnias and the complex relationship between sleep and epilepsies. Although narcolepsy is considered as a rare disease, recent discoveries in narcolepsy research provided insight in the function of brain circuitries involved in sleep wake regulation. REM sleep behavioral parasomnia (RBD) is increasingly recognized to represent an early manifestation of neurodegenerative disorders, in particular evolving synucleinopathies. Early diagnosis may thus open new perspectives for developing novel treatment options by targeting neuroprotective substances.

Protective effects of recombinant human bone morphogenetic protein-7 on focal cerebral ischemia-reperfusion injury.

This study was to investigate the protective effect of recombinant human bone morphogenetic protein-7 (rhBMP-7) on focal cerebral ischemia-reperfusion (IR) injuries and their underlying mechanisms. An intraluminal suture method was used to generate a middle cerebral artery occlusion model in rats, which was followed by reperfusion. A sham operation (SO) group underwent the procedure without occlusion, whereas an IR group and rhBMP-7 treated group (RT) underwent occlusion in the absence and presence of rhBMP-7 (250 µg/kg) administered via a femoral vein injection 30 minutes prior to reperfusion. Twenty-four hours after reperfusion, neurological function, brain water content and morphological alterations were examined. Apoptosis was detected using terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling assays, and immunohistochemical staining and Western blot assays were used to detect nuclear nuclear factor-kappa B (NF-κB) p65 expression. Compared with the SO group, IR rats showed a decrease in neurological function, an increase in brain water content, and pathological and morphological damage (p < 0.05). Higher levels of apoptosis were also detected in the infarct region area. In contrast, RT rats had reduced injury after IR. In addition, while immunohistochemical staining and western blot assays consistently detected increased expression of nuclear NF-κB after IR, these levels were reduced in the RT group. Administration of rhBMP-7 prior to reperfusion effectively inhibited the extent of IR injury by attenuating cerebral edema and ameliorating ultrastructural damage. The underlying mechanisms responsible for these observations potentially involve the inhibition of apoptosis induced by IR by rhBMP-7 via an NF-κB-related signaling cascade.

[Expert opinion on surgical care pathway management of neurologic patients from Neuro-Urology Committee of the French National Association of Urology (AFU)]. / Le parcours de l'opéré en neuro-urologie: de la programmation opératoire à la convalescence. L'avis d'expert du Comité de Neuro-Urologie de l'AFU.

The surgical care pathway of neurologic patients has two aims: preventing urinary morbidity and mortality and improving their quality of life. It requires taking into account the specificities of disabilities in domains of body functions: circulatory, ventilation and digestive physiology, motor functions, sensory functions, mental functions, and skin fragility which are responsible of dependencies in this heterogeneous group of patients. This management is necessarily multidisciplinary to be optimal and through specific clinical care pathway, providing guidance to the surgical procedure: preparation of the surgery, its realization, and post-operative rehabilitation. The indication for surgery must be coordinated and validated in neuro-urology multidisciplinary staff. Preoperative stay in a physical and rehabilitation medicine center may be useful to ensure a complete assessment and anticipate problems related to surgery. The patient will be hospitalized in the urology department in a single room suited to their disabilities and handicaps. The chronic treatments should be not modified if possible. The lack of sensitivity does not dispense anesthesia to prevent autonomic hyperreflexia, the most severe complication after high complete spinal cord injury. The laparoscopy and sub-peritoneal surgery, the early removal nasogastric tube and early refeeding make it possible to early resumption of intestinal transit. In many cases, the patients should be transferred to a physical and rehabilitation medicine during post-operative period where the nursing care will be most suitable. A quickly adapted rehabilitation must be able to reduce loss of function and physical dependence.

Brain-behavior relationship in a case of successful ECT for drug refractory catatonic OCD.

This case report describes the successful response to electroconvulsive therapy (ECT) in a patient with an unusual presentation of catatonia, whose onset occurred in the context of an extremely severe form of refractory obsessive-compulsive disorder (OCD). We correlated the clinical improvement in catatonic and OCD symptoms with specific changes in brain function as shown by regional cerebral blood flow scans, neurological soft signs examination, and neuropsychological testing. All assessments were conducted before and after the ECT course. The results strongly suggest that a right hemisphere dysfunction was the neural correlate of our patient's symptoms, and that ECT, by reverting this abnormality, may serve as an effective therapeutic approach for refractory catatonic OCD.

Smoking and endocrine disorders.

Patients suffering from endocrine disorders, which affect the function of central nervous system, need a special care and approach in the process of smoking cessation. The process of informing about negative effects of smoking addiction, including potential aggravation of the disease, has to be specially tailored to the need of the patient and the type of the disease. The informing physician should also serve as a model by not smoking and try to confirm the patient about positive benefits of quitting smoking.

Effectiveness and safety of botulinum toxin in comparison with surgery for drooling in paediatric patients with neurological disorders: a systematic review.

Different therapeutic methods for chronic drooling in paediatric patients with neurological problems have been described in the scientific literature. However, there is no consensus on the ideal strategy of treatment. The aim of this study was to compare botulinum toxin injection therapy and surgical modalities to control drooling in paediatric patients with neurological disorders. A systematic literature search was conducted on nine electronic databases for publications until April 2020. Six articles were included with a total sample of 209 patients, 67.4% (n = 141) of whom had cerebral palsy. All studies used injections of botulinum toxin type A with application to the submandibular and/or parotid salivary glands. The surgical treatments were duct ligation in the parotid and/or submandibular salivary glands, duct relocation in the submandibular salivary glands, and glandular excision of the submandibular and sublingual salivary glands. There were complications in only 16.1% (n = 27) of the sample (11 cases due to botulinum toxin application and 16 due to surgery). Drooling control was assessed by objective and subjective measures. Although surgical procedures presented a higher risk of adverse effects than botulinum toxin type A in all the studies and measurements performed, they presented larger and longer-lasting positive effects on drooling. We suggest bilateral submandibular duct relocation with bilateral sublingual gland excision or isolated bilateral submandibular duct ligation, which were the surgical techniques with the largest samples in this review. Nevertheless, further studies are necessary to compare samples with botulinum toxin type A and surgical treatment.

Vitamin B12 deficiency and phenylketonuria.

The literature regarding the vitamin B(12) status of patients with phenylketonuria was reviewed. Adequate amounts of B(12) are provided in products used in dietary treatment; however, a number of case reports and cohort studies document deficiency in those who have discontinued taking amino acid, mineral and vitamin supplements but who continue to eat only very limited amounts of natural protein. Symptoms and signs of B(12) deficiency are variable but severe deficiency may cause serious neurological disease. Nitrous oxide anaesthesia is a particular risk. It is recommended that plasma total homocysteine and plasma or urinary methylmalonate should be routinely measured, as they are more sensitive markers of deficiency than serum B(12) concentrations. Functional B(12) deficiency can occur in the presence of a normal B(12) concentration.