Gastrointestinal symptom burden in diabetic autonomic and peripheral neuropathy - A Danes cohort study.

OBJECTIVE: We investigated associations between gastrointestinal symptoms - evaluated as a combined weighted symptom score (CWSS) - Diabetic autonomic neuropathy (DAN), and distal symmetrical polyneuropathy (DSPN) in type 1 and type 2 diabetes. RESEARCH DESIGN AND METHODS: Cross-sectional study in a tertiary outpatient clinic. CWSS was calculated based on questionnaires: gastroparesis composite symptom index (GCSI) and gastrointestinal symptom rating score (GSRS). DAN and DSPN were addressed using the composite autonomic symptom score 31 (COMPASS-31) questionnaire, cardiac autonomic reflex tests (CARTs), electrochemical skin conductance (ESC), vibration perception threshold (VPT), Michigan Neuropathy Screening Instrument (MNSI), pain- and thermal sensation. Analyses were adjusted for age, sex, diabetes duration, smoking, LDL-cholesterol, HbA1C and systolic blood pressure. Type 1 and type 2 diabetes were evaluated separately. RESULTS: We included 566 with type 1 diabetes and 377 with type 2 diabetes. Mean ± SD age was 58 ± 15 years and 565 (59.9 %) were women. A high CWSS was present in 143 (25 %) with type 1 and 142 (38 %) with type 2 diabetes. The odds of DAN by COMPASS-31 (p < 0.001) were higher in the high score group. For type 1 diabetes, odds of cardiac autonomic neuropathy were higher in the high CWSS group. The odds of DSPN by VPT and MNSI in type 1 diabetes, and by ESC, VPT and pain sensation in type 2 diabetes were higher in the high CWSS group. CONCLUSIONS: A high symptom score was associated with neuropathy by COMPASS-31 and vibration perception. Gastrointestinal symptom burden associated inconsistently with other neuropathy tests between diabetes types.

Quantitative assessment of plantar pressure patterns in relation to foot deformities in people with hereditary motor and sensory neuropathies.

BACKGROUND: Hereditary motor and sensory neuropathies (HMSN), also known as Charcot-Marie-Tooth disease, are characterized by affected peripheral nerves. This often results in foot deformities that can be classified into four categories: (1) plantar flexed first metatarsal, neutral hindfoot, (2) plantar flexed first metatarsal, correctable hindfoot varus, (3) plantar flexed first metatarsal, uncorrectable hindfoot varus, and (4) hindfoot valgus. To improve management and for the evaluation of surgical interventions, a quantitative evaluation of foot function is required. The first aim of this study was to provide insight into plantar pressure of people with HMSN in relation to foot deformities. The second aim was to propose a quantitative outcome measure for the evaluation of surgical interventions based on plantar pressure. METHODS: In this historic cohort study, plantar pressure measurements of 52 people with HMSN and 586 healthy controls were evaluated. In addition to the evaluation of complete plantar pressure patterns, root mean square deviations (RMSD) of plantar pressure patterns from the mean plantar pressure pattern of healthy controls were calculated as a measure of abnormality. Furthermore, center of pressure trajectories were calculated to investigate temporal characteristics. Additionally, plantar pressure ratios of the lateral foot, toes, first metatarsal head, second/third metatarsal heads, fifth metatarsal head, and midfoot were calculated to measure overloading of foot areas. RESULTS: Larger RMSD values were found for all foot deformity categories compared to healthy controls (p < 0.001). Evaluation of the complete plantar pressure patterns revealed differences in plantar pressure between people with HMSN and healthy controls underneath the rearfoot, lateral foot, and second/third metatarsal heads. Center of pressure trajectories differed between people with HMSN and healthy controls in the medio-lateral and anterior-posterior direction. The plantar pressure ratios, and especially the fifth metatarsal head pressure ratio, differed between healthy controls and people with HMSN (p < 0.05) and between the four foot deformity categories (p < 0.05). CONCLUSIONS: Spatially and temporally distinct plantar pressure patterns were found for the four foot deformity categories in people with HMSN. We suggest to consider the RMSD in combination with the fifth metatarsal head pressure ratio as outcome measures for the evaluation of surgical interventions in people with HMSN.

Amazon Amandaba-Prevalence, Risk Factors and Self-Care Perception Associated with Diabetic Peripheral Neuropathy in Patients with Type 2 Diabetes: A Cross-Sectional Study.

BACKGROUND: Diabetic peripheral neuropathy (DPN) is one of the most common complications of type 2 diabetes mellitus. There is a gradual loss of protective sensation in the skin and the function of the foot joints, increasing the risk of injury as the disease progresses. The objective of this study was to verify whether socioeconomic factors, health risk factors, and self-care are associated with DPN. METHODS: Observational cross-sectional with 228 individuals of ≥30 years in Family Health Strategies in a city in the eastern Amazon, in northern Brazil, using questionnaires containing socioeconomic information, clinical and laboratory parameters, the Summary of Diabetes Self-Care Activities Questionnaire, and the Michigan Neuropathy Screening Instrument. RESULTS: The prevalence of DPN was 66.6%. The presence of neuropathy is associated with male gender, dyslipidemia, and increased microalbuminuria. Logistic regression analysis revealed male subjects' increased BMI and altered HDL levels were associated with DPN. CONCLUSIONS: In men with altered BMI, and dysregulation in biochemical parameters, neuropathy is more prevalent.

Proposal for the functional assessment of acute inflammatory neuropathy (FAAIN) in Guillain-Barré syndrome.

BACKGROUND: Guillain Barré syndrome (GBS) functional assessment is necessary in clinical practice, research and clinical trials. Existing instruments are not sensitive to change and are not applicable to the current GBS clinical spectrum. OBJECTIVE: To construct a functional assessment for acute inflammatory neuropathies (FAAIN-GBS), inclusive for current GBS spectrum that assesses extension and intensity separately. METHODS: FAAIN-GBS subscales were constructed. Its structure and interpretation were defined. It was validated using data from medical record of 167 GBS patients admitted to the Institute of Neurology and Neurosurgery. Cronbach α was used for items reduction and reliability analysis. Bartlett sphericity test was performed. Exploratory factor analysis (EFA) of the main components, with varimax rotation, was applied to evaluate dimensionality and content validity. Hughes scale was used as gold standard for criterion validity. Sensitivity, specificity and area under the receiver operating characteristic curves (AUROC), were calculated. Construct validity was assessed by confirmatory factor analysis (CFA). RESULTS: FAAIN-GBS is made up of two subscales (extension and intensity). The final score is obtained by averaging both dimensions. Internal consistency was acceptable (Cronbach 0.745). EFA showed three dimensions: intensity, spinal extension and cranial extension. Spearman correlation between FAAIN-GBS and Hughes scale was 0.463. Sensitivity (0.714) and specificity (0.986) values showed the good behavior of the scale; AUROC was 0.93. CONCLUSION: FAAIN-GBS was constructed and a first step of validation was made, showing good internal consistency and validity. New prospective studies with large populations will be necessary to perfect this instrument that could be useful in neurological practice.

Expert opinion on monitoring symptomatic hereditary transthyretin-mediated amyloidosis and assessment of disease progression.

BACKGROUND: Hereditary transthyretin-mediated amyloidosis, also known as ATTRv amyloidosis (v for variant), is a rare, autosomal dominant, fatal disease, in which systemic amyloid progressively impairs multiple organs, leading to disability and death. The recent approval of disease-modifying therapies offers the hope of stabilization or eventual reversal of disease progression, and yet highlights a lack of disease-management guidance. A multidisciplinary panel of expert clinicians from France and the US came to consensus on monitoring the disease and identifying progression through a clinical opinion questionnaire, a roundtable meeting, and multiple rounds of feedback. MONITORING DISEASE AND PROGRESSION: A multidisciplinary team should monitor ATTRv amyloidosis disease course by assessing potential target organs at baseline and during follow-up for signs and symptoms of somatic and autonomic neuropathy, cardiac dysfunction and restrictive cardiomyopathy, and other manifestations. Variability in penetrance, symptoms, and course of ATTRv amyloidosis requires that all patients, regardless of variant status, undergo regular and standardized assessment in all these categories. Progression in ATTRv amyloidosis may be indicated by: worsening of several existing quantifiable symptoms or signs; the appearance of a new symptom; or the worsening of a single symptom that results in a meaningful functional impairment. CONCLUSIONS: We suggest that a multisystem approach to monitoring the signs and symptoms of ATTRv amyloidosis best captures the course of the disease. We hope this work will help form the basis of further, consensus-based guidance for the treatment of ATTRv amyloidosis.

Electrodiagnostic Assessment of Uncommon Mononeuropathies.

Carpal tunnel syndrome, ulnar neuropathy at the elbow, and peroneal neuropathy are the most common mononeuropathies; however, other individual nerves may also be injured by various processes. These uncommon mononeuropathies may be less readily diagnosed owing to unfamiliarity with the presentations and vague symptoms. Electrodiagnostic studies are essential in the evaluation of uncommon mononeuropathies and can assist in localization and prognostication. However, they can also be challenging; stimulation at the proximal sites is difficult and well-validated reference values are not available. This article reviews the electrodiagnostic assessment of several uncommon upper and lower extremities mononeuropathies.

[Entrapment neuropathies: a contemporary approach to pathophysiology, clinical assessment, and management : German version]. / Nervenkompressionssyndrome ­ eine aktuelle Betrachtung von Pathophysiologie, klinischer Untersuchung und Management : Deutsche Fassung.

Entrapment neuropathies such as carpal tunnel syndrome, radiculopathies, or radicular pain are the most common peripheral neuropathies and also the most common cause for neuropathic pain. Despite their high prevalence, they often remain challenging to diagnose and manage in a clinical setting. Summarising the evidence from both preclinical and clinical studies, this review provides an update on the aetiology and pathophysiology of entrapment neuropathies. Potenzial mechanisms are put in perspective with clinical findings. The contemporary assessment is discussed and diagnostic pitfalls highlighted. The evidence for the noninvasive and surgical management of common entrapment neuropathies is summarised and future areas of research are identified.

Condutas clínicas e barreiras no tratamento da polineuropatia amiloidótica familiar associada à transtirretina (PAF-TTR) no Brasil / Clinical practice and barriers in familial transthyretinrelated amyloidotic polyneuropathy (FAP) care in Brazil

Objetivo: Identificar as condutas terapêuticas e a variabilidade na prática clínica, assim como necessidades não atendidas e barreiras para a adequada assistência a pacientes com polineuropatia amiloidótica familiar relacionada à transtirretina (PAF-TTR), no Brasil. Métodos: Estudo transversal, por meio de questionário semiestruturado on-line enviado por e-mail. Foram incluídos médicos com experiência no manejo clínico-assistencial de pacientes com PAF-TTR no Brasil. O questionário foi composto por 30 questões envolvendo características gerais da população brasileira com PAF-TTR, características das escolhas terapêuticas e da falha, definições de progressão de doença e estadiamento, e métodos para mensuração do impacto na qualidade de vida. Resultados: Seis profissionais responderam ao inquérito. Quanto ao diagnóstico e à classificação da doença, houve consenso quanto ao uso de quadro clínico associado a testagem genética para o diagnóstico, e foram considerados adequados os critérios de Coutinho e do Ministério da Saúde, apesar de serem pouco úteis na avaliação da progressão da doença. Entre os especialistas, 83,3% entendem que a terapia atualmente disponível no Sistema Único de Saúde (SUS) atende às necessidades dos pacientes no estágio I da doença, entretanto todos os especialistas apontam necessidades assistenciais não atendidas, uma vez que esse medicamento não possui benefício definido para os estágios II e III da doença. A progressão da doença é definida como qualquer novo sintoma ou piora daqueles preexistentes, não sendo necessária modificação no estágio da doença para caracterizar tal evento. Conclusões: A condução deste estudo permitiu a identificação de aspectos importantes para auxiliar no entendimento da prática clínico-assistencial no país e das necessidades em saúde desses pacientes

Objective: To identify therapeutic approaches and variability in clinical practice, as well as unmet needs and barriers to adequate care for patients with familial transthyretin-related amyloidotic polyneuropathy (FAP) in Brazil. Methods: Cross-sectional study using an online semi-structured questionnaire sent by email. Physicians with experience in the clinical-care management of patients with FAP in Brazil were included. The questionnaire consisted of 30 questions involving general characteristics of the Brazilian population with FAP; characteristics of therapeutic choices and failure; definitions of disease progression and staging; and methods for measuring the impact on quality of life. Results: Six professionals responded to the survey. As for the diagnosis and classification of the disease, there was a consensus regarding the use of a clinical picture associated with genetic testing for the diagnosis and, as appropriate, the criteria proposed by Coutinho and the Ministry of Health, although not very useful in evaluating the progression of the disease. 83.3% of experts understand that the therapy currently available in the SUS meets the needs of patients in stage I of the disease, however, all experts point out unmet care needs, since this drug has no defined benefit for stages II and III of the disease. Disease progression is defined as any new symptom or worsening of pre-existing ones, and no change in the stage of the disease is necessary to characterize such an event. Conclusions: The conduction of this study allowed to identify important aspects to a better understanding of the clinical care practice in the country and unmet needs of these patients

Hospitalization Costs of Lower Limb Ulcerations and Amputations in Patients with Diabetes in Romania.

In this retrospective case-control study conducted in Cluj-Napoca, Romania, we assessed the effect of ulcerations/amputations on hospitalization costs of patients with diabetes. Patients with (Group 1) or without (Group 2) ulcerations/amputations (case-control ratio 3:1) admitted to a single diabetes center between 2012-2017 were included. The effects of hospitalization days, age, duration of diabetes, body mass index and glycated hemoglobin (HbA1c) on total costs was explored using a multivariate linear regression analysis, enter model. Overall, 876 patients were included (Group 1: 682, 323 [47.4%] with amputations; Group 2: 194). Median (interquartile range) total expenses in Group 1 were 40% higher compared to Group 2 (€724 [504; 1186] vs €517 [362; 645], p < 0.001). Significant differences were observed between hospitalization costs (p < 0.001), cost of food (p < 0.001), medication (p = 0.044), drugs administered at the emergency room/intensive care unit (p < 0.001) and other expenses (p = 0.003). Hospitalization costs represented 80.5% of total expenses in Group 1 and 76.3% in Group 2. In multivariate analysis, hospitalization days influenced significantly the total costs in both groups (p < 0.001); in Group 2, the effect of HbA1c was also significant (p = 0.021). Diabetic foot ulcers and subsequent amputations most likely impose a significant economic burden on the Romanian public healthcare system.

Advances in imaging technologies for the assessment of peripheral neuropathies in rheumatoid arthritis.

Peripheral neuropathy in patients with rheumatoid arthritis is associated with a maladaptive autoimmune response that may cause chronic pain and disability. Nerve conduction studies are the routine method performed when rheumatologists presume its presence. However, this approach is invasive, may not reveal subtle malfunctions in the early stages of the disease, and does not expose abnormalities in structures surrounding the nerves and muscles, limiting the possibility of a timely diagnosis. This work aims to present a narrative review of new technologies for the clinical assessment of peripheral neuropathy in Rheumatoid Arthritis. Through a bibliographic search carried out in five repositories, from 1990 to 2020, we identified three technologies that could detect peripheral nerve lesions and perform quantitative evaluations: (1) magnetic resonance neurography, (2) functional magnetic resonance imaging, and (3) high-resolution ultrasonography of peripheral nerves. We found these tools can overcome the main constraints imposed by the previous electrophysiologic methods, enabling early diagnosis.

Comparação da dor e qualidade de vida entre indivíduos com e sem neuropatia diabética / Comparison of pain and quality of life between individuals with and without diabetic neuropathy / Comparación del dolor y la calidad de vida entre individuos con y sin neuropatía diabética

Objetivo: comparar a dor e a qualidade de vida de indivíduos com e sem neuropatia diabética. Método: estudo transversal realizado com 251 participantes com diabetes mellitus tipo 2. Utilizou-se a escala Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) para avaliar neuropatia diabética, características da dor, perda de sensibilidade protetora (PSP) e avaliar a qualidade de vida pelo Short-Form 6 Dimensions-Brasil/SF-6D. Realizou-se análise estatística descritiva. Resultados: 16,3% apresentaram neuropatia, 97,6% queixaram-se de dor, sendo a maioria crônica e nos pés ou panturrilhas. 51,2% dos neuropáticos tiveram PSP no teste do monofilamento (p=0,001). Os descritores de dor mais referidos pelos neuropáticos: queimação (p=0,004), formigamento (p=0,002) e alfinetada e/ou agulhada (p=0,003) e os domínios de qualidade de vida afetados foram: dor, saúde mental e vitalidade. Conclusão: aqueles com neuropatia têm maior intensidade de dor, acordam à noite e apresentam alteração na sensibilidade dos pés, que pode ser rastreada na atenção primária.

Objective: to compare pain and quality of life in individuals with and without diabetic neuropathy. Method: a cross-sectional study with 251 participants with type 2 diabetes mellitus. The Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) scale was used to assess diabetic neuropathy, pain characteristics, loss of protective sensitivity (LPS) and to assess the quality of life by Short-Form 6 Dimensions-Brasil/SF-6D. Descriptive statistical analysis was performed. Results: among the participants, 16.3% had neuropathy, 97.6% complained of pain, most of them chronic and in the feet or calves. Also, 51.2% of neuropathic patients had LPS in the monofilament test (p=0.001). The pain descriptors most frequently reported by neuropathic patients were: burning (p=0.004), tingling (p=0.002), and pinprick and/or needling (p=0.003). The affected quality of life domains were: pain, mental health, and vitality. Conclusion: those with neuropathy have greater pain intensity, wake up at night, and have altered foot sensitivity, which can be tracked in primary care.

Objetivo: comparar el dolor y la calidad de vida en individuos con y sin neuropatía diabética. Método: estudio transversal con 251 participantes con diabetes mellitus tipo 2. Se utilizó la escala Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) para evaluar la neuropatía diabética, las características del dolor, la pérdida de sensibilidad protectora (PSP) y para evaluar la calidad de vida por Short-Form 6 Dimensiones-Brasil/SF-6D. Se realizó análisis estadístico descriptivo. Resultados: el 16,3% presentaba neuropatía, el 97,6% se quejaba de dolor, la mayoría crónico y en pies o pantorrillas. El 51,2% de los pacientes neuropáticos tenían PSP en la prueba de monofilamento (p = 0,001). Los descriptores de dolor informados con mayor frecuencia por los pacientes neuropáticos fueron: ardor (p = 0,004), hormigueo (p = 0,002) y pinchazo y/o punción (p = 0,003) y los dominios de calidad de vida afectada fueron: dolor, salud mental y vitalidad. Conclusión: las personas con neuropatía tienen mayor intensidad de dolor, se despiertan por la noche y tienen alteración de la sensibilidad de los pies, lo que se puede rastrear en atención primaria.

Possible implication of undescribed SMN1-SMN2 genotype in chronic EMG-pattern of SMA with transitory acute denervation.

Spinal muscular atrophy (SMA) refers to a group of genetic neuromuscular disorders affecting lower motor neurons causative of numerous phenotypes. To date, according to the age of onset, maximum muscular activity achieved, and life expectation four types of SMA are recognized, all caused by mutations in the SMN1 gene with SMN2 copy number influencing disease severity. Herein, we describe the case of a 31-year-old young male with normal psychomotor development who has experienced fatigue, cramps, and muscle fasciculations in the lower limbs for a period of 2 months. Based on electrophysiological and clinical findings we performed SMA genetic, clinical exome and RNA expression of candidate genes which led us to suggest SMN1-SMN2 genes [(2+0) and (0+0)] combination as possibly being implicated in the phenotype.

Estimating the impact of COVID-19 pandemic on services provided by Italian Neuromuscular Centers: an Italian Association of Myology survey of the acute phase.

INTRODUCTION: Since February 2020, the outbreak of COVID-19 in Italy has forced the health care system to undergo profound rearrangements in its services and facilities, especially in the worst-hit areas in Northern Italy. In this setting, inpatient and outpatient services had to rethink and reorganize their activities to meet the needs of patients during the "lockdown". The Italian Association of Myology developed a survey to estimate the impact of these changes on patients affected by neuromuscular disorders and on specialized neuromuscular centers during the acute phase of COVID-19 pandemic. METHODS: We developed an electronic survey that was sent to neuromuscular centers affiliated with the Italian Association of Myology, assessing changes in pharmacological therapies provision, outpatient clinical and instrumental services, support services (physiotherapy, nursing care, psychological support) and clinical trials. RESULTS: 40% of surveyed neuromuscular centers reported a reduction in outpatient visit and examinations (44.5% of centers in Northern regions; 25% of centers in Central regions; 50% of centers in Southern regions). Twenty-two% of centers postponed in-hospital administration of therapies for neuromuscular diseases (23.4% in Northern regions; 13.0% in Central regions; 20% in Southern regions). Diagnostic and support services (physiotherapy, nursing care, psychological support) were suspended in 57% of centers (66/43/44% in Northern, Central and Southern centers respectively) Overall, the most affected services were rehabilitative services and on-site outpatient visits, which were suspended in 93% of centers. Strategies adopted by neuromuscular centers to overcome these changes included maintaining urgent on-site visits, addressing patients to available services and promoting remote contact and telemedicine. CONCLUSIONS: Overall, COVID-19 pandemic resulted in a significant disruption of clinical and support services for patients with neuromuscular diseases. Despite the efforts to provide telemedicine consults to patients, this option could be promoted and improved further. A close collaboration between the different neuromuscular centers and service providers as well as further implementation of telehealth platforms are necessary to ensure quality care to NMD patients in the near future and in case of recurrent pandemic waves.

Entrapment neuropathies: a contemporary approach to pathophysiology, clinical assessment, and management.

Entrapment neuropathies such as carpal tunnel syndrome, radiculopathies, or radicular pain are the most common peripheral neuropathies and also the most common cause for neuropathic pain. Despite their high prevalence, they often remain challenging to diagnose and manage in a clinical setting. Summarising the evidence from both preclinical and clinical studies, this review provides an update on the aetiology and pathophysiology of entrapment neuropathies. Potential mechanisms are put in perspective with clinical findings. The contemporary assessment is discussed and diagnostic pitfalls highlighted. The evidence for the noninvasive and surgical management of common entrapment neuropathies is summarised and future areas of research are identified.

Evaluation of the risk of diabetic peripheral neuropathy: Design and validation of the NeuDiaCan nursing screening procedure. / Valoración del riesgo de neuropatía periférica diabética: diseño y validación del procedimiento para cribado enfermero NeuDiaCan.

OBJECTIVE: To design and validate a nursing screening procedure for diabetic peripheral neuropathy in primary care. METHODS: The study was carried out in three phases. 1)Construction of an item bank to form the procedure with an exit score describing the patient's clinical situation. 2)Test and reduction of the initial tentative procedure on a sample of 50 patients using community nurse consultations, eliminating the components with low inter-intra nurse reliability. 3)Validation of the version of the procedure obtained in the previous step on a sample of 106 patients. Calculation of validity and reliability by eliminating components with low criterion validity with respect to the results of the diagnostic electromyography used as a reference standard. Cut-off points were estimated for the use of the procedure as a screening tool, predictive values, performance, internal consistency and inter-nurse reliability. RESULTS: The initial tentative procedure consisted of 12 components that were reduced to 10. In the process of validation of this second version the procedure was simplified again, eventually comprising 6 components, with a cut-off point of 2.5 in its output scale, the point at which it reaches adequate values of sensitivity and negative predictors to be used as a screening instrument. For this cut-off point the inter-intra nurse reliability, criterion validity and predictive validity reached acceptable values. CONCLUSIONS: NeuDiaCan as a nursing screening procedure for diabetic peripheral neuropathy in primary care is valid, reliable and easy to use.

Health-care use and information needs of children with neonatal brachial plexus palsy: A cross-sectional survey among 465 Dutch patients.

To investigate health-care use (HCU) and information needs of children aged 0-18 years with neonatal brachial plexus palsy (NBPP), a cross-sectional study was performed. Patients and/or parents seen in our NBPP clinic were invited to complete a survey comprising questions on HCU due to NBPP and current information needs. Outcomes were described for three age-groups (0-1/2-9/10-18 years), based on follow-up status (early/late/no-discharge). Four hundred sixty-five parents/patients participated (59 in the 0-1, 226 in the 2-9, and 180 in the 10-18-year group). Two hundred ninety-three patients had C5-C6 lesions, 193 were discharged from follow-up, 83 of whom categorized as 'early discharged' (<1 year of age). Over the past year, 198 patients had contact with the expert team (49 in the 0-1, 81 in the 2-9, and 68 in the 10-18-year group) and 288 with at least one other health-care professional (53 in the 0-1, 133 in the 2-9, and 102 in the 10-18-year group). Of the 83 patients discharged early, 34 reported health-care use. Two hundred twenty-eight participants reported current information needs of whom 23 were discharged early. HCU and information needs of Dutch children with NBPP remains considerable even in children who were discharged. Stricter follow-up and information provision for these patients is needed.

Quantitative assessment of diabetic amyotrophy using magnetic resonance neurography-a case-control analysis.

OBJECTIVES: To quantitatively characterize diabetic amyotrophy (DA), or diabetic lumbosacral radiculoplexopathy, and compare with controls using magnetic resonance neurography (MRN). METHODS: Forty controls and 23 DA cases were analyzed qualitatively and quantitatively. Cross-sectional areas (CSAs) of bilateral L3 through S2 lumbosacral nerve roots, femoral nerves, and sciatic nerves (proximal and distal measurements) were measured. A linear model was used to assess the nerve location and case/control effect on angle-corrected CSAs. Intra- and inter-reader analysis was performed using intraclass correlation (ICC). RESULTS: In DA cases, abnormalities of the lumbosacral nerve roots, sciatic, femoral, and obturator nerves were seen in 21/23, 16/23, 21/23, and 9/23, respectively. Denervation abnormalities of multiple abdominopelvic muscles were seen. Quantitatively, the CSA of all measured LS plexus nerve roots and bilateral femoral nerves were significantly larger in DA cases vs. controls by 45% (95% CI, (30%, 49%); p < 0.001). The ICC was moderate for inter-rater analysis = 0.547 (95% CI, 0.456-0.626) and excellent for intra-rater analysis = 0.90 (95% CI, 0.89-92). CONCLUSIONS: Multifocal neuromuscular lesions related to diabetic amyotrophy were qualitatively and quantitatively detected on MRN. Qualitative abnormalities distinguished cases from controls, and nerve CSAs of cases were significantly larger than those of controls. Therefore, MRN may be employed as a non-invasive diagnostic tool for the evaluation of diabetic amyotrophy. KEY POINTS: • Qualitative abnormalities of lumbosacral nerve roots, their peripheral branches, and muscles are seen in DA. • The lumbosacral nerve roots and their peripheral branches in diabetic amyotrophy cases are significantly larger in cross-sectional area than non-diabetic subjects by 45% (95 CI, 30%, 49%; p < 0.001). • The ICC was moderate for inter-rater analysis = 0.547 (95% CI, 0.456-0.626) and excellent for intra-rater analysis = 0.90 (95% CI, 0.89-92).

Update on the Impact, Diagnosis and Management of Cardiovascular Autonomic Neuropathy in Diabetes: What Is Defined, What Is New, and What Is Unmet.

The burden of diabetic cardiovascular autonomic neuropathy (CAN) is expected to increase due to the diabetes epidemic and its early and widespread appearance. CAN has a definite prognostic role for mortality and cardiovascular morbidity. Putative mechanisms for this are tachycardia, QT interval prolongation, orthostatic hypotension, reverse dipping, and impaired heart rate variability, while emerging mechanisms like inflammation support the pervasiveness of autonomic dysfunction. Efforts to overcome CAN under-diagnosis are on the table: by promoting screening for symptoms and signs; by simplifying cardiovascular reflex tests; and by selecting the candidates for screening. CAN assessment allows for treatment of its manifestations, cardiovascular risk stratification, and tailoring therapeutic targets. Risk factors for CAN are mainly glycaemic control in type 1 diabetes mellitus (T1DM) and, in addition, hypertension, dyslipidaemia, and obesity in type 2 diabetes mellitus (T2DM), while preliminary data regard glycaemic variability, vitamin B12 and D changes, oxidative stress, inflammation, and genetic biomarkers. Glycaemic control prevents CAN in T1DM, whereas multifactorial intervention might be effective in T2DM. Lifestyle intervention improves autonomic function mostly in pre-diabetes. While there is no conclusive evidence for a disease-modifying therapy, treatment of CAN manifestations is available. The modulation of autonomic function by SGLT2i represents a promising research field with possible clinical relevance.

Diagnostic accuracy of upper limb neurodynamic tests for the assessment of peripheral neuropathic pain: A systematic review.

BACKGROUND: Upper limb neurodynamic tests (ULNTs) are used to identify a neuropathic pain component in patients' presenting with arm and/or neck pain. Clinical tests with established diagnostic accuracy are required to not only to inform clinical management but also minimise costs associated with expensive medical investigations. OBJECTIVE: To evaluate the role of ULNTs in assessment of peripheral neuropathic pain and to inform their value in clinical practice when assessing patients with arm and/or neck symptoms. DESIGN: Systematic review was undertaken according to published guidelines, and reported in line with PRISMA-DTA. METHOD: Key databases were searched up to 21/11/2017. INCLUSION CRITERIA: Patient population experiencing arm and/or neck symptoms with suspected peripheral neuropathic involvement, studies that compared ULNT to a reference standard, any study design using primary diagnostic accuracy data. Two reviewers independently assessed risk of bias (ROB) using QUADAS-2. The overall quality of evidence was evaluated using GRADE. RESULTS: Of eight included studies (n = 579), four were assessed as low ROB, although all had concerns regarding applicability. For carpal tunnel syndrome, ULNT1 sensitivity values ranged 0.4-0.93, specificity 0.13-0.93, positive likelihood ratio 0.86-3.67 and negative likelihood ratio 0.5-1.9. For cervical radiculopathy ULNT1 and the combined use of four ULNTs had sensitivity of 0.97 (95%CI 0.85-1.00) whereas the ULNT3 was the most specific (0.87, 95%CI 0.62-0.98). Positive likelihood ratio ranged 0.58-5.68 and negative likelihood ratio 0.12-1.62. CONCLUSION: Based on the available evidence ULNTs cannot be utilised as a stand-alone test for the diagnosis of CTS. Limited evidence suggests that ULNTs may be clinically relevant for the diagnosis of CR, but only as a "ruling out" strategy. However, the overall quality of the body of evidence after applying the GRADE approach was low to very low across studies. Further higher quality research is needed to establish firm conclusions.

Degree of risk for foot ulcer due to diabetes: nursing assessment / Grado de riesgo para las úlceras en los pies por diabetes: evaluación de enfermería / Grau de risco para úlceras nos pés por diabetes: avaliação de enfermagem

ABSTRACT Objective: To classify the level of risk for foot ulcers in people with diabetes mellitus and identify their main predictive risk factors. Method: Exploratory, descriptive study, in which patients were assessed in a municipal ambulatory of São Paulo through nursing consultation, following the guidelines of the International Consensus on the Diabetic Foot. Data were descriptively analyzed. Results: The analyzed population was composed of 50 longevous and retired people, with household income of up to two minimum wages, with dermato-neurofunctional risk factors and unfavorable clinical indicators, and 66% had Risk 1; 16% Risk 2; 6% Risk 3 and 12% Risk 4. Of this analyzed total, 96% never had their feet examined with the Semmes-Weinstein monofilament. Conclusion: The data found indicate the importance of careful feet examination in people with diabetes by the nursing staff to identify future risks of ulcers and, thus, prevent them.

RESUMEN Objetivo: Clasificar el grado de riesgo para las ulceraciones en los pies de las personas con diabetes mellitus e identificar sus principales factores de riesgo predictivos. Método: Estudio exploratorio, descriptivo, en el cual los pacientes fueron evaluados en un ambulatorio municipal de São Paulo por medio de la consulta de enfermería, según las directrices del International Consensus on the Diabetic Foot. Los datos fueron analizados descriptivamente. Resultados: La población analizada fue de 50 personas, longevos jóvenes, jubilados, con ingresos familiares de hasta dos salarios mínimos, con factores de riesgo dermato-neurofuncionales e indicadores clínicos desfavorables, siendo que el 66% presentó riesgo 1; el 16% de riesgo 2; el 6% riesgo 3; y el 12% de riesgo 4. De ese total analizado, el 96% nunca tuvo los pies examinados con el monofilamento de Semmes-Weinstein. Conclusión: Los datos encontrados apuntan la importancia de la evaluación cuidadosa de los pies de las personas con diabetes por la enfermería para identificar los riesgos futuros de ulceraciones y, de esta forma, prevenirlos.

RESUMO Objetivo: Classificar o grau de risco para ulcerações nos pés de pessoas com diabetes mellitus e identificar seus principais fatores de risco preditivos. Método: Estudo exploratório, descritivo, onde os pacientes foram avaliados em um ambulatório municipal de São Paulo por meio da consulta de enfermagem, segundo diretrizes do International Consensus on the Diabetic Foot. Os dados foram analisados descritivamente. Resultados: a população analisada foi de 50 pessoas, longevos jovens, aposentados, renda familiar de até dois salários mínimos, com fatores de risco dermato-neuro-funcionais e indicadores clínicos desfavoráveis, sendo que 66% apresentaram risco 1; 16% risco 2; 6% risco 3 e 12% risco 4. Dentre estes, 96% nunca tiveram seus pés examinados com o monofilamento de Semmes Weinstein. Conclusão: Os dados encontrados apontam a importância da avaliação criteriosa dos pés das pessoas com diabetes pela enfermagem para identificar os riscos futuros de ulcerações, e desta forma trabalhar a prevenção dos mesmos.