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BACKGROUND Neuropathy is a common chronic complication in type 2 diabetes mellitus (T2DM). Statin and metformin are commonly used medications in T2DM patients, and some studies showed statin- or metformin-induced neuropathy. AIM To evaluate the incidence of neuropathy among patients with T2DM associated with statin and metformin therapies. METHODS Korean Health Insurance Review and Assessment national patient sample data from 2016 and 2017 were used. Patients with T2DM and no complications were divided into statin/metformin/statin + metformin users and non-users. Neuropathy incidence was defined by International Statistical Classification of Diseases and Related Health Problems, 10th revision codes and concomitant prescriptions for anticonvulsants or antidepressants. Logistic regression analyses were conducted to examine the associations between statin/metformin/statin + metformin therapies and the incidence of neuropathy. Propensity score (PS) matching was performed on the basis of age, sex and comorbidities. RESULTS Overall, 34964 and 35887 patients with T2DM and no complications were included in the Korean Health Insurance Review and Assessment national patient sample datasets from 2016 and 2017, respectively. Statin therapy was associated with increased risks of neuropathy in 2016 and 2017 [PS-matched odds ratio (OR) = 1.22, 95% confidence interval (CI): 1.08-1.38; PS-matched OR = 1.17, 95%CI: 1.03-1.33, respectively]. Metformin therapy was associated with reduced risks of neuropathy in 2016 and 2017 (PS-matched OR = 0.30, 95%CI: 0.21-0.42; PS-matched OR = 0.44, 95%CI: 0.32-0.60, respectively). Combined statin + metformin therapy was not significantly associated with neuropathy in 2016 or 2017 (PS-matched OR = 0.85, 95%CI: 0.61-1.19; PS-matched OR = 0.95, 95%CI: 0.66-1.38, respectively). CONCLUSION Statin therapy was associated with enhanced risk of new-onset neuropathy in patients with T2DM, but metformin therapy showed the opposite association.

Background The immunological pathophysiologies of chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN) differ considerably, but neither has been elucidated completely. Quantitative magnetic resonance imaging (MRI) techniques such as diffusion tensor imaging, T2 mapping, and fat fraction analysis may indicate in vivo pathophysiological changes in nerve architecture. Our study aimed to systematically study nerve architecture of the brachial plexus in patients with CIDP, MMN, motor neuron disease (MND) and healthy controls using these quantitative MRI techniques. Methods We enrolled patients with CIDP (n = 47), MMN (n = 29), MND (n = 40) and healthy controls (n = 10). All patients underwent MRI of the brachial plexus and we obtained diffusion parameters, T2 relaxation times and fat fraction using an automated processing pipeline. We compared these parameters between groups using a univariate general linear model. Results Fractional anisotropy was lower in patients with CIDP compared to healthy controls (p, With this study, we show that quantitative MRI techniques reveal differences in the brachial plexus between patients with CIDP, MMN, MND and healthy controls. CIDP is characterized by lower FA and higher RD than MMN, MND and healthy controls, whilst MMN is characterized by higher FA values than CIDP and MND. These differences between CIDP and MMN are the most remarkable and important finding as they emphasize important differences in the underlying pathophysiologies.

Background and purpose In its initial stages, Guillain–Barré syndrome (GBS) is difficult to identify, because diagnostic criteria may not always be fulfilled. With this retrospective study, we wanted to identify the most common electrophysiological abnormalities seen on neurophysiological examination of GBS patients and its variants in the early phases. Methods We reviewed the clinical records of patients admitted to our Neurology Unit with a confirmed diagnosis of GBS. The study sample was divided in two subgroups according to whether the neurophysiological examination was performed: within 7 days (very early group) or within 7–15 days (early group). H reflex, F waves, and motor and sensory conduction parameters were judged abnormal if they were outside the normal range for at least two nerves. We evaluated neurophysiological findings in Miller–Fisher syndrome (MFS) separately. Results The study sample comprised 36 patients. In GBS, the most frequent abnormal neurophysiological parameter was the bilateral absence of the H reflex, followed by F wave abnormalities. Motor conduction parameters were altered in less than 50% of patients, and even less common were sensory nerve action potential reduction and the "sural‐sparing" pattern. In MFS, H reflex was absent bilaterally in 100% of patients, followed by a predominant peripheral sensory involvement, whereas motor conduction parameters were frequently normal. Conclusions Bilateral absence of the H reflex is the most sensitive parameter in early diagnosis of GBS and its variants., Electrodiagnostic findings in GBS and MFS patients: percentage of patients with abnormal results in at least two nerves. When patients were excluded due to the lack of CMAPs, SNAPs or normal sural nerve SNAP, frequencies are calculated based on the number of patients reported in brackets. MCV: motor conduction velocity. DML: distal motor latency. CMAP Dur: compound motor action potential duration. CMAP A: compound motor action potential amplitude. SNAP A: sensory nerve action potential amplitude. SCV: sensory conduction velocity.

Background and purpose Chronic axonal polyneuropathy is a common, usually multifactorial, disease for which there is no treatment yet available. We investigated the association between cardiovascular health, defined by the health score of the American Heart Association, and chronic axonal polyneuropathy. Methods Between June 2013 and January 2017, we investigated participants of the Rotterdam Study, a population‐based cohort study. Participants were screened for polyneuropathy and categorized as having no, possible, probable or definite polyneuropathy. The cardiovascular health score (range 0–14; higher score reflecting better health) consisted of four health behaviours (diet, physical activity, smoking and body mass index) and three health factors (blood pressure, serum cholesterol and fasting glucose level). Results We included 1919 participants, of whom 120 (6.3%) had definite polyneuropathy. The median (interquartile range [IQR]) age was 69.0 (58.6–73.7) years and 53.4% were women. A higher cardiovascular health score was associated with a lower prevalence of definite polyneuropathy (per point increase: odds ratio [OR] 0.90, 95% confidence interval [CI] 0.84–0.96). Optimal cardiovascular health (score≥10) was strongly associated with a lower prevalence of definite polyneuropathy (OR 0.55, 95% CI 0.32–0.90). An increase in health factors and health behaviour scores separately was associated with a lower prevalence of polyneuropathy (per point increase: OR 0.82, 95% CI 0.71–0.95 and OR 0.86, 95% CI 0.78–0.96, respectively). The association between a lower cardiovascular health score and lower sural nerve amplitude was not significant after correction for covariates (difference 0.07µV, 95% CI −0.02–0.17). Conclusions Better cardiovascular health, consisting of both modifiable health behaviours and health factors, is associated with a lower prevalence of chronic axonal polyneuropathy., Chronic axonal polyneuropathy is a common, disabling disease for which there is no treatment yet available. This highlights the need to identify modifiable risk factors. In this population‐based study, we investigated the association between cardiovascular health, defined by the seven‐item American Heart Association score, and chronic axonal polyneuropathy. Better cardiovascular health, consisting of both modifiable health behaviour and health factors, is associated with lower prevalence of chronic axonal polyneuropathy in the general population. This is of interest because individuals can potentially modify their health behaviour.

This narrative review provides an overview of specific outcomes that are appropriate for chronic inflammatory demyelinating polyneuropathy monitoring during routine clinical care and highlights the minimum clinically important difference, if known. Importance Identifying clinical change in many neurologic diseases, including chronic inflammatory demyelinating polyneuropathy (CIDP), can be challenging. At the same time, how change is defined heavily influences a patient's diagnostic and treatment pathway. It can be especially problematic when equivocal subjective observations are interpreted as clinically meaningful and then used to make diagnostic and treatment decisions. Change in clinical trials is strictly defined by a preselected metric, but there is a perception that formal outcomes collection during routine clinical care is neither feasible nor necessary. Given the importance placed on how change is interpreted, there is a need to select assessments that can be applied to routine care that are representative of the neurologic disease state. Observations For an outcome measure to be useful during clinical trials, it must have good reliability, validity, be responsive to change, and have clinical meaning. To be useful during routine clinical care, the assessment must additionally be easy to collect without the need for extensive training or equipment and should provide an immediately available result that can be rapidly quantified and interpreted. Chronic inflammatory demyelinating polyneuropathy is clinically heterogeneous and so is best evaluated with a diverse group of assessment tools. Assessing strength impairment, disability, and quality of life is ideally suited for everyday practice when caring for patients with CIDP. While electrophysiologic studies, imaging, cerebrospinal fluid, and nodal/paranodal antibodies can provide diagnostic data, they are less practical and helpful longitudinal assessment tools. Conclusions and Relevance Sound clinimetric outcome measures in CIDP are widely available and have the potential to help clinicians objectify treatment response and disease progression. Such data are critically important when justifying the need for ongoing or periodic immunotherapy, documenting relapse or deterioration, or providing reassurance of disease improvement, stability, or remission.

Background and purpose Systematic research on the effect of Charcot-Marie-Tooth (CMT) disease on the outcome of pregnancy and conversely the effect of pregnancy on neuropathy is still sparse. Methods A clinical cohort study and cross-sectional study within the German CMT-NET was conducted between 2016 and 2019. Inclusion criteria were a confirmed diagnosis of CMT and at least one completed pregnancy after 1990. All participants agreed to fill in questionnaires and have their medical files reviewed. Results The study group comprised 54 women with a total of 98 pregnancies. The mean age at onset of CMT disease was 12.6 years (range 0-37 years). Fifty (92%) patients had autosomal dominant CMT; two patients each (4%) had X-linked and autosomal recessive CMT. Forty patients (74%) had a PMP22 gene duplication (CMT1A). Obstetric complications did not differ significantly from a German reference population, neither in the whole group nor in the CMT1A group. Overall there was no increased newborn morbidity and mortality. About one-third of patients reported exacerbation of CMT disease in or after pregnancy. No adverse effects of anaesthesia were reported. Most participants stressed a positive attitude and awareness of challenges associated with pregnancy. Important issues were assistance and support in caring for the family. Discussion In line with findings from our previous study undertaken in the 1990s, there were no increased complication rates for pregnancy and delivery. These results are reassuring for the vast majority of CMT patients and are important for family planning and clinical care.

Age-related muscle loss is a phenomenon that has been extensively studied in recent decades. Sarcopenia is a multisystem disease, which predisposes to muscle weakness and frailty. At around 50 years of age, an individual begins to lose muscle strength, although this becomes more evident after 70. Sarcopenia is a condition typically found in older adults but can also affect younger people. Sarcopenia is a preventable and treatable condition. In past years, methods and tools to recognize the condition early have been researched. For the development of therapeutic interventions, agreement on diagnosis is fundamental. In recent years, a possible role of ultrasonography in the diagnosis of sarcopenia has been evaluated, compared with the best-known techniques.

Background and purpose Dolichoarteriopathies of the extracranial part of the internal carotid artery (ICA) are associated with cerebrovascular events, yet information on their prevalence and risk factors remains limited. The aim of the present study therefore was to study the prevalence and risk factors of dolichoarteriopathies in a sample of patients with cerebrovascular symptoms from the Plaque At RISK (PARISK) study. Methods In a random sample of 100 patients from the PARISK study, multidetector computed tomography angiography (MDCTA) was performed as part of clinical workup. On MDCTA, we evaluated the extracranial trajectory of the ICA by measuring the length (in millimeters), the tortuosity index (TI; defined as the ICA length divided by the shortest possible distance from bifurcation to skull base), and dolichoarteriopathy type (tortuosity, coiling or kinking). Next, we investigated the association between cardiovascular risk factors and these measurements using linear and logistic regression analyses. Results The mean (standard deviation) length of the ICA was 93 (± 14) mm, with a median (interquartile range) TI of 1.2 (1.1–1.3). The overall prevalence of dolichoarteriopathies was 69%, with tortuosity being the most common (72%), followed by coiling (20%), and kinking (8%). We found that age and obesity were associated with a higher TI: difference per 10‐year increase in age: 0.05 (95% confidence interval [CI] 0.02–0.08) and 0.16 (95% CI 0.07–0.25) for obesity. Obesity and hypercholesterolemia were associated with a more severe type of dolichoarteriopathy (odds ratio [OR] 2.07 [95% CI 1.04–4.12] and OR 2.17 [95% CI 1.02–4.63], respectively). Conclusion Dolichoarteriopathies in the extracranial ICA are common in patients with cerebrovascular symptoms, and age, obesity and hypercholesterolemia may play an important role in the pathophysiology of these abnormalities., We investigated the prevalence of internal carotid artery dolichoarteriopathies using quantitative measurements and traditional visual rating in a random sample of patients with cerebrovascular symptoms who underwent multidetector computed tomography angiography. Tortuosity was the most common type of dolichoarteriopathy (72%), followed by coiling (20%), and kinking (8%). Age, obesity, and hypercholesterolemia were significantly associated with a more severe type of dolichoarteriopathy and/or tortuosity index. Therefore, these cardiovascular risk factors may play an important role in the pathophysiology of these abnormalities.

Background. Gravitational erythema is rare pathologic condition that is characterized by abnormal vascular response on venous pressure changes. It is benign disease, and it is included in differential diagnosis of vasculitis, vasculopathies, neuropathies and does not require treatment. Compression garments may be required in some cases. Clinical Case Description . The case of gravitational erythema in 13 years old patient of is presented. The disease was presented with erythematous mosaic rash on the lower limbs skin. It has appeared in 4-6 minutes after verticalisation and mostly on the lower legs. The rash was accompanied with some bluish spots disappearing after pressure. The elements of rush were itchy, there was pain in distal parts of the feet. The rash was reversed after walking and changing of limb position. Positive compression test was mentioned. Conclusion. Differential diagnosis of gravitational erythema with other clinically similar conditions remains relevant. The rare diagnosis of gravitational erythema and low awareness of doctors about it cause misdiagnosis, costly continuous examination and ineffective treatment.

Background and purpose Chronic inflammatory demyelinating polyneuropathy (CIDP) causes weakness which adversely impacts function and quality of life (QOL). CIDP often requires long-term management with intravenous or subcutaneous immunoglobulin. The Polyneuropathy and Treatment with Hizentra (R) (PATH) study showed that subcutaneous immunoglobulin (SCIG) was efficacious in CIDP maintenance. Here, patient-reported outcomes in patients on SCIG are assessed. Methods Subjects stabilized on intravenous immunoglobulin were randomly allocated to receive weekly 0.2 or 0.4 g/kg bodyweight of 20% SCIG (IgPro20) or placebo. Overall QOL/health status was assessed using the EuroQoL 5-Dimension (EQ-5D) health profile and visual analog scale, treatment satisfaction was assessed with the Treatment Satisfaction Questionnaire for Medicine (TSQM) and work-related impact was assessed with the Work Productivity and Activity Impairment Questionnaire for General Health (WPAI-GH). The EQ-5D health profile was assessed in terms of the percentage of subjects maintained or improved at week 25 of SCIG therapy on each of the EQ-5D domains versus baseline after intravenous immunoglobulin stabilization. TSQM and WPAI-GH were assessed by median score changes from baseline to week 25. Results In total, 172 subjects were randomized to placebo (n = 57), 0.2 g/kg IgPro20 (n = 57) and 0.4 g/kg IgPro20 (n = 58). Significantly higher proportions of IgPro20-treated subjects improved/maintained their health status on the EQ-5D usual activities dimension, and in additional dimensions (mobility and pain/discomfort) in sensitivity analyses. TSQM and WPAI-GH scores were more stable with IgPro20 treatment compared with placebo. Conclusions IgPro20 maintained or improved QOL in most subjects with CIDP, consistent with the PATH study findings that both IgPro20 doses were efficacious in maintaining CIDP.

Inflammatory Rasch-built overall disability scale (I-RODS) seems to be a valid activity measure for use in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Our aim was to translate and validate the I-RODS for use in CIDP patients from Serbia. Study comprised 83 patients diagnosed with CIDP. I-RODS was translated and cross-culturally validated using the standard guidelines. Following scales were also administered: Medical Research Council (MRC) sum score, Inflammatory Neuropathy Cause and Treatment (INCAT) sensory and disability scores, Krupp's Fatigue Severity Scale, Beck Depression Inventory, Visual Analogue Scale for pain, and health survey-36 (SF-36) as a quality of life measure. According to the I-RODS, significant proportion of our patients reported that "running" (51%), "dancing" (41%), and "standing for hours" (40%) were impossible tasks to perform, while "teeth brushing" (94%), "eating" (88%), and "reading a newspaper/book" (82%) were noted as the easiest items. Patients with more muscle weakness (lower MRC sum score) and more severe INCAT sensory score had lower I-RODS score (P 1 (P

Running is an athletic activity that is increasingly gaining popularity. Despite its benefits, there are many suspected risk factors for running-related overuse injuries. The objective of this study is to describe injuries and clinical symptoms observed on the sole of the foot in runners, giving special attention to the weekly running volume. The literature presented in this narrative review is based on a non-systematic search of the Medline, Google Scholar, and ResearchGate databases and focuses on foot injuries (the full spectrum of the foot pathology from bones to tendons and plantar fascia, nerve, and joint disorders) in runners, which represents an important topic for both professional and recreational runners. The weekly running distance appeared to be one of the strongest predictors for future overuse injuries. Marathon training and average weekly running of over 20 km are possible predictive factors in the development of plantar foot injuries. The plantar medial aspect of the foot is the anatomic area of the foot that most frequently experiences pain, with numerous pathologic conditions. As a result, diagnosis is always a challenging task. The ability to obtain an accurate medical history and carefully perform a physical examination, together with good knowledge of the foot anatomy and kinesiology, are also proven to be key players in ensuring proper diagnosis.

Background and purpose The aim of this study was to determine the frequency of over‐ and underdiagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and to identify related diagnostic pitfalls. Methods We conducted a retrospective study in Dutch patients referred to the Erasmus University Medical Centre Rotterdam between 2011 and 2017 with either a diagnosis of CIDP or another diagnosis that was revised to CIDP. We used the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) 2010 diagnostic criteria for CIDP to classify patients into three groups: overdiagnosis, underdiagnosis, or confirmed diagnosis of CIDP. Clinical and laboratory features and treatment history were compared between groups. Results A referral diagnosis of CIDP was revised in 32% of patients (31/96; overdiagnosis). Of 81 patients diagnosed with CIDP, 16 (20%) were referred with another diagnosis (underdiagnosis). In the overdiagnosed patients, 20% of muscle weakness was asymmetric, 48% lacked proximal muscle weakness, 29% only had distal muscle weakness, 65% did not fulfil the electrodiagnostic criteria for CIDP, 74% had an elevated cerebrospinal fluid (CSF) protein level, and 97% had another type of neuropathy. In the underdiagnosed patients, all had proximal muscle weakness, 50% had a clinically atypical CIDP, all fulfilled the electrodiagnostic criteria for CIDP, and 25% had an increased CSF protein level. Conclusion Over‐ and underdiagnosis of CIDP is common. Diagnostic pitfalls include lack of attention to proximal muscle weakness as a diagnostic hallmark of CIDP, insufficient recognition of clinical atypical phenotypes, overreliance on CSF protein levels, misinterpretation of nerve conduction studies and poor adherence to electrodiagnostic criteria, and failure to exclude other causes of polyneuropathy., Over‐ and underdiagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is common. A referral diagnosis of CIDP was revised in 32% of patients (31/96; overdiagnosis). Of 81 patients diagnosed with CIDP, 16 (20%) were referred with another diagnosis (underdiagnosis). Almost all overdiagnosed patients had other forms of neuropathy, while underdiagnosed patients were referred with various neurological disorders. Diagnostic pitfalls included lack of attention to proximal muscle weakness as a diagnostic hallmark of CIDP, insufficient recognition of clinical atypical phenotypes, overreliance on cerebrospinal fluid protein levels, misinterpretation of nerve conduction studies and poor adherence to electrodiagnostic criteria, and failure to exclude other causes of polyneuropathy.

Background and purpose Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a clinical and electrophysiological heterogeneous immune‐mediated polyneuropathy. Intravenous immunoglobulin (IVIg), corticosteroids, and plasma exchange are proven effective treatments for CIDP. The clinical response to IVIg is variable between patients and currently unexplained. Finding biomarkers related to treatment response can help to understand the diversity of CIDP and personalise treatment choice. Methods We investigated whether genetic variation between patients may explain some of these differences in treatment response. Based on previous publications, we selected six candidate genes that might affect immune and axonal functions, IVIg metabolism, and treatment response in CIDP. Genetic variants were assessed in 172 CIDP patients treated with at least one course of IVIg (2 g/kg). A response to IVIg was defined by ≥1 grade improvement on the modified Rankin Scale. Blood samples were tested for variations in CNTN2, PRF1, FCGRT, FCGR2B, GJB1, and SH2D2A genes. Results In univariate analysis, patients with the FCGR2B promoter variant 2B.4/2B.1 responded more often to IVIg than patients with the 2B.1/2B.1 variant (odds ratio [OR] = 6.9, 95% confidence interval [CI] = 1.6–30; p = 0.003). Patients with the p.(Ala91Val) variant of PRF1 were less often IVIg responsive (OR = 0.34, 95% CI = 0.13–0.91; p = 0.038). In multivariate analysis, both PRF1 and FCGR2B showed discriminative ability to predict the chance of IVIg response (area under the curve = 0.67). Conclusions Variations in PRF1 and the promoter region of FCGR2B are associated with the response to IVIg in CIDP. These findings, which require validation, are a first step towards the understanding of the heterogeneity in the treatment response in CIDP., We investigated whether genetic variation in six candidate genes was associated with the response to IVIg in CIDP patients. Variations in PRF1 and the promoter region of FCGR2B were associated with the response to IVIg in CIDP.

ObjectiveTo compare the disease course in patients with mild Guillain-Barré syndrome (GBS) who were treated with intravenous immunoglobulin (IVIg) or supportive care only.MethodsWe selected patients from the prospective observational International GBS Outcome Study (IGOS) who were able to walk independently at study entry (mild GBS), treated with one IVIg course or supportive care. The primary endpoint was the GBS disability score four weeks after study entry, assessed by multivariable ordinal regression analysis.ResultsOf 188 eligible patients, 148 (79%) were treated with IVIg and 40 (21%) with supportive care. The IVIg group was more disabled at baseline. IVIg treatment was not associated with lower GBS disability scores at 4 weeks (adjusted OR (aOR) 1.62, 95% CI 0.63 to 4.13). Nearly all secondary endpoints showed no benefit from IVIg, although the time to regain full muscle strength was shorter (28 vs 56 days, p=0.03) and reported pain at 26 weeks was lower (n=26/121, 22% vs n=12/30, 40%, p=0.04) in the IVIg treated patients. In the subanalysis with persistent mild GBS in the first 2 weeks, the aOR for a lower GBS disability score at 4 weeks was 2.32 (95% CI 0.76 to 7.13). At 1 year, 40% of all patients had residual symptoms.ConclusionIn patients with mild GBS, one course of IVIg did not improve the overall disease course. The certainty of this conclusion is limited by confounding factors, selection bias and wide confidence limits. Residual symptoms were often present after one year, indicating the need for better treatments in mild GBS.

More frequent lower dosing does not further improve the efficacy of intravenous immunoglobulin (IVIg) in stable IVIg‐dependent chronic inflammatory demyelinating polyradiculoneuropathy. More frequent lower dosing does lead to more stable serum immunoglobulin G levels but not to higher trough levels (Fig. 3)., Background and purpose High peak serum immunoglobulin G (IgG) levels may not be needed for maintenance intravenous immunoglobulin (IVIg) treatment in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and such high levels may cause side effects. More frequent lower dosing may lead to more stable IgG levels and higher trough levels, which might improve efficacy. The aim of this trial is to investigate whether high frequent low dosage IVIg treatment is more effective than low frequent high dosage IVIg treatment. Methods In this randomized placebo‐controlled crossover trial, we included patients with CIDP proven to be IVIg‐dependent and receiving an individually established stable dose and interval of IVIg maintenance treatment. In the control arm, patients received their individual IVIg dose and interval followed by a placebo infusion at half the interval. In the intervention arm, patients received half their individual dose at half the interval. After a wash‐out phase patients crossed over. The primary outcome measure was handgrip strength (assessed using a Martin Vigorimeter). Secondary outcome indicators were health‐related quality of life (36‐item Short‐Form Health Survey), disability (Inflammatory Rasch‐built Overall Disability Scale), fatigue (Rasch‐built Fatigue Severity Scale) and side effects. Results Twenty‐five patients were included and were treated at baseline with individually adjusted dosages of IVIg ranging from 20 to 80 g and intervals ranging from 14 to 35 days. Three participants did not complete the trial; the main analysis was therefore based on the 22 patients completing both treatment periods. There was no significant difference in handgrip strength change from baseline between the two treatment regimens (coefficient −2.71, 95% CI −5.4, 0.01). Furthermore, there were no significant differences in any of the secondary outcomes or side effects. Conclusions More frequent lower dosing does not further improve the efficacy of IVIg in stable IVIg‐dependent CIDP and does not result in fewer side effects.

Background and purpose Fatigue is amongst the most frequent and disabling symptoms of multiple sclerosis and a close relation between fatigue and sleep quality has been hypothesized. In this study the contribution of sleep disturbances measured by clinical and polysomnographic parameters to fatigue in multiple sclerosis was investigated. Methods This was a prospective instrumental study performed at the Neurocenter of Southern Switzerland. Demographic data and clinical characteristics including fatigue (as measured by the modified fatigue impact scale [MFIS]), neurological disability, psychiatric symptoms, medications and sleep‐related variables were collected at baseline visit and by a home full‐night polysomnography. The associations between sleep‐related variables and the MFIS were tested using partial correlations adjusted by demographic and sleep‐unrelated clinical factors. Results Seventy‐six patients were included in the study, of whom 53 (69.7%) had an MFIS ≥38 points (median 49.5, interquartile range 31.0–62.0). MFIS scores were positively associated with age, neurological disability, symptoms of depression and anxiety, and use of benzodiazepines and selective serotonin reuptake inhibitors. When adjusting for these variables, the presence of restless legs syndrome (RLS) (r = 0.37, p = 0.005) and periodic leg movements index (r = −0.33, p = 0.014) were associated with MFIS. Excessive daytime sleepiness, total sleep time, sleep efficiency, respiratory disturbances, and percentage of time spent in the different sleep stages (N1, N2, N3 and rapid eye movement) were not associated with fatigue. Conclusions Multiple sclerosis patients with a diagnosis of RLS had significantly higher global fatigue scores compared to those without RLS. Future studies should investigate whether medical treatment of RLS can ameliorate fatigue., Fatigue is amongst the most frequent and disabling symptoms of multiple sclerosis (MS), which is difficult to treat and negatively influences patients' quality of life. An investigation was done of whether clinical and polysomnographic measures of sleep quality influence fatigue in a large group of MS patients. It was observed that MS patients who also suffer from restless legs syndrome show significantly greater symptoms of fatigue, suggesting a potential causal link between these two entities and potential treatment implications.

Producción Científica, Aim of the study Systematic review and meta-analysis to assess the effectiveness of manual therapy in improving carpal tunnel syndrome (CTS) symptoms, physical function, and nerve conduction studies. Method MEDLINE, Web of Science, SCOPUS, Cochrane Library, TRIP database, and PEDro databases were searched from the inception to September 2021. PICO search strategy was used to identify randomized controlled trials applying manual therapy on patients with CTS. Eligible studies and data extraction were conducted independently by two reviewers. Methodology quality and risk of bias were assessed by PEDro scale. Outcomes assessed were pain intensity, physical func- tion, and nerve conduction studies. Results Eighty-one potential studies were identified and six studies involving 401 patients were finally included. Pain inten- sity immediately after treatment showed a pooled standard mean difference (SMD) of − 2.13 with 95% confidence interval (CI) (− 2.39, − 1.86). Physical function with Boston Carpal Tunnel Syndrome Questionnaire (BCTS-Q) showed a pooled SMD of − 1.67 with 95% CI (− 1.92, − 1.43) on symptoms severity, and a SMD of − 0.89 with 95% CI (− 1.08, − 0.70) on functional status. Nerve conduction studies showed a SMD of− 0.19 with 95% CI (−0.40, − 0.02) on motor conduction and a SMD of − 1.15 with 95% CI (− 1.36, − 0.93) on sensory conduction. Conclusions This study highlights the effectiveness of manual therapy techniques based on soft tissue and neurodynamic mobilizations, in isolation, on pain, physical function, and nerve conduction studies in patients with CTS., Publicación en abierto financiada por el Consorcio de Bibliotecas Universitarias de Castilla y León (BUCLE), con cargo al Programa Operativo 2014ES16RFOP009 FEDER 2014-2020 DE CASTILLA Y LEÓN, Actuación:20007-CL - Apoyo Consorcio BUCLE

Introduction: Numerous cases have been reported of patients with symptoms of Guillain-Barré syndrome associated with COVID-19, but much information is still lacking on this association and its implications. The objective of this review is to analyse the available evidence on this topic in the adult population. Material and methods: A systematic review was conducted of studies published on scientific databases: PubMed, Cochrane, Science Direct, MEDLINE, and WHO COVID-19 database. Results: We identified 45 studies, which were analysed and completed using the Covidence platform; the final analysis included 24 articles, with a total of 30 patients. Conclusions: We found a strong association between both conditions; furthermore, the studies analysed highlight differences in the presentation of the disease, with greater severity of symptoms in Guillain-Barre syndrome associated with COVID-19. Resumen: Introducción: Se han reportado distintos casos de pacientes con cuadro de síndrome de Guillain-Barré asociado a COVID-19, pero falta mucha información aún sobre esta asociación y sus implicancias, el objetivo de esta revisión es analizar la evidencia disponible en esta temática en la población adulta. Material y Métodos: Se realizó una revisión sistemática de estudios publicados en buscadores científicos: PubMed, Cochrane, Science direct, Medline, OMS COVID-19. Resultados: Se identificaron 45 estudios, los cuales se analizaron y completaron utilizando la plataforma Covidence, incluyendo para el análisis final 24 artículos y sumando un total de 30 pacientes. Conclusiones: Se demuestra una asociación fuerte entre ambas patologías, además los estudios analizados recalcan diferencias en la presentación de la enfermedad con mayor gravedad en los cuadros de Síndrome de Guillain-Barre asociados a COVID-19.

Background and purpose Elevated cerebrospinal fluid (CSF) total protein in patients with acute ascending paresis is indicative of Guillain–Barré syndrome (GBS). Recent studies showed that the outdated, but still widely used upper reference limit (URL) for CSF total protein of 0.45 g/L leads to false‐positive results, mainly as a result of lack of age‐adjustment. The objective of this study was to assess the frequency of increased CSF total protein in adult GBS patients according to a new age‐dependent URL. Methods Patients with GBS treated at the Medical University of Innsbruck between 2000 and 2018 were included in this study. Demographic, clinical, electrophysiological and CSF data were obtained from patients' medical charts. Frequency of increased CSF total protein depending on disease duration was compared using the conventional URL of 0.45 g/L and the age‐dependent URL. Results Ninety‐seven patients with GBS aged 57 ± 18 years, comprising 38% women, underwent CSF sampling within a median of 6 days after symptom onset. The median CSF total protein concentration was 0.65 g/L and correlated with disease duration. Overall, 74% of patients had elevated CSF total protein levels using the conventional URL, as opposed to 52% applying the age‐dependent URL. At 0–3, 4–7, 8–14 and >14 days after disease onset, elevated CSF total protein was found in 46%, 84%, 78% and 100% of patients using the conventional URL, and in 32%, 53%, 65% and 64% of patients using the age‐dependent URL. In multivariate analysis, significant predictors of elevated CSF total protein were disease duration and the demyelinating GBS variant. Similar results were obtained for CSF/serum albumin quotient (Qalb). Conclusion Fewer true‐positives for CSF total protein and Qalb must be considered in suspected GBS, especially in the early disease course., Cerebrospinal fluid (CSF) total protein and CSF/serum albumin quotient are less frequently elevated in patients with Guillain–Barré syndrome than previously reported, as a result of novel age‐dependent upper reference limits that reduce false‐positives and avoid over‐interpretation of slightly elevated protein levels in the elderly.

Herpes zoster (HZ) infection occurs in approximately 10% to 30% of individuals. Visceral neuropathies secondary to HZ can cause cystitis and urinary retention. But colonic pseudo-obstruction can also occur. Peripheral neuropathy may reveal segmental motor paresis of either upper or lower limbs, the abdominal muscles or the diaphragm. We report the case of a 62-year-old male patient who presented with abdominal distention and cutaneous vesicular eruption on the left side of the abdominal wall. Plain X-rays and computed tomography scan showed distended small bowel. A diagnosis of intestinal pseudo-obstruction was made secondary to segmental paresis of the small intestine and visceral neuropathy. Conservative management was successful and the patient was discharged uneventfully. Intestinal pseudo-obstruction ought to be considered when dealing with non-obstructive (adynamic) conditions of the digestive tract associated with HZ infection; since early recognition may help to avoid unnecessary surgery.

Patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) show varying degrees of response to intravenous immunoglobulin (IVIg) therapy. This randomised phase III study in patients with CIDP (ProCID trial) will compare the efficacy and safety of 3 different doses (0.5, 1.0, and 2.0 g/kg) of IVIg 10% (panzyga) administered every 3 weeks for 24 weeks. The primary efficacy endpoint is the rate of treatment response, defined as a decrease in adjusted inflammatory neuropathy cause and treatment disability score of ≥1 point, in the IVIg 1.0 g/kg arm at week 24. Patients with definite or probable CIDP according to European Federation of Neurological Sciences/Peripheral Nerve Society criteria with IVIg or corticosteroid dependency and active disease are eligible. All potentially eligible patients will undergo IVIg or corticosteroid dose reduction (washout phase) over ≤12 weeks or until deterioration of CIDP (active disease). Patients with deterioration during the washout phase will be randomised to receive study treatment during a dose-evaluation phase starting with a loading dose of IVIg 2.0 g/kg followed by maintenance treatment with IVIg 0.5, 1.0, or 2.0 g/kg every 3 weeks. Rescue medication (2 doses of IVIg 2.0 g/kg given 3 weeks apart) will be administered to patients in the IVIg 0.5 and 1.0 g/kg groups who deteriorate after week 3 and before week 18 or who do not improve at week 6. Safety, tolerability and quality of life will be assessed. The ProCID study will provide new information on the best maintenance dose of IVIg for patients with CIDP.

During 1993—2016 there were treated 1255 children 9 months — 14 ages old with tick-bite infections in Kirov Infectious Clinical Hospital and 1214 children from them with the verified diagnosis of Lyme disease. Damage of nervous system was detected in 98 (8.1%) patients in the forms of serous meningitis, meningoencephalitis, polyneuropathies, neuropathies, disseminated encephalomyelitis, diencephalic syndrome with impaired thermal regulation. 45.9 % of cases were mixed-infection (tick-bite encephalitis and Lyme disease).

Peripheral neuropathies associated with painful small fiber neuropathy (SFN) are complex conditions, resistant to treatment with conventional medications. Previous clinical studies strongly support the use of dietary agmatine as a safe and effective treatment for neuropathic pain. Based on this evidence, we conducted an open-label consecutive case series study to evaluate the effectiveness of agmatine in neuropathies associated with painful SFN (Study Registry: ClinicalTrials.gov, System Identifier: NCT01524666). Participants diagnosed with painful SFN and autonomic dysfunctions were treated with 2.67 g/day agmatine sulfate (AgmaSet&reg, capsules containing G-Agmatine&reg, brand of agmatine sulfate) for a period of 2 months. Before the beginning (baseline) and at the end of the treatment period, participants answered the established 12-item neuropathic pain questionnaire specifically developed to distinguish symptoms associated with neuropathy and to quantify their severity. Secondary outcomes included other treatment options and a safety assessment. Twelve patients were recruited, and 11 patients&mdash, 8 diagnosed with diabetic neuropathy, two with idiopathic neuropathy and one with inflammatory neuropathy&mdash, completed the study. All patients showed improvement in neuropathic pain to a varied extent. The average decrease in pain intensity was 26.0 rating points, corresponding to a 46.4% reduction in overall pain (p &lt, 0.00001). The results suggest that dietary agmatine sulfate has a significant effect in reducing neuropathic pain intensity associated with painful SFN resistant to treatment with conventional neuropathic pain medications. Larger randomized placebo-controlled studies are expected to establish agmatine sulfate as a preferred treatment.

Background and purpose: Hereditary transthyretin (hATTR) amyloidosis causes progressive polyneuropathy resulting from transthyretin (TTR) amyloid deposition throughout the body, including the peripheral nerves. The efficacy and safety of inotersen, an antisense oligonucleotide inhibitor of TTR protein production, were demonstrated in the pivotal NEURO-TTR study in patients with hATTR polyneuropathy. Here, the long-term efficacy and safety of inotersen are assessed in an ongoing open-label extension (OLE) study. Methods: Patients who completed NEURO-TTR were eligible to enroll in the OLE (NCT02175004). Efficacy assessments included the modified Neuropathy Impairment Score plus seven neurophysiological tests composite score (mNIS + 7), the Norfolk Quality of Life - Diabetic Neuropathy (Norfolk QOL-DN) questionnaire total score and the Short-Form 36 Health Survey (SF-36) Physical Component Summary (PCS) score. Safety and tolerability were also assessed. Results: Overall, 97% (135/139) of patients who completed NEURO-TTR enrolled in the OLE. Patients who received inotersen for 39 cumulative months in NEURO-TTR and the OLE continued to show benefit; patients who switched from placebo to inotersen in the OLE demonstrated improvement or stabilization of neurological disease progression by mNIS + 7, Norfolk QOL-DN and SF-36 PCS. No new safety concerns were identified. There was no evidence of increased risk for grade 4 thrombocytopenia or severe renal events with increased duration of inotersen exposure. Conclusion: Inotersen slowed disease progression and reduced deterioration of quality of life in patients with hATTR polyneuropathy. Early treatment with inotersen resulted in greater long-term disease stabilization than delayed initiation. Routine platelet and renal safety monitoring were effective; no new safety signals were observed. info:eu-repo/semantics/publishedVersion

Neurologic manifestations of Zika infection must be adequately recognized and treated; our study methods can be used for monitoring and warning systems., Evidence is increasing that Zika virus can cause extensive damage to the central nervous system, affecting both fetuses and adults. We sought to identify traces of possible clinical manifestations of nervous system diseases among the registers of hospital admissions recorded in the Brazilian Unified Health System. Time series of several diagnoses from the International Classification of Diseases, 10th Revision, were analyzed by using control diagrams, during January 2008–February 2016. Beginning in mid-2014, we observed an unprecedented and significant rise in the hospitalization rate for congenital malformations of the nervous system, Guillain-Barré syndrome, encephalitis, myelitis, and encephalomyelitis. These conditions are compatible with viral infection and inflammation-associated manifestations and may have been due to the entrance of Zika virus into Brazil. These findings show the necessity of adequately diagnosing and treating suspected cases of Zika virus infection and also that health surveillance systems can be improved by using routine data.

Background: Due to incidental occurrence of ectropion as a late complication of cheek advancement flaps, this study investigated the long-term effects of these flaps for post-Mohs' reconstruction of the cheek aesthetic. Methods: All the patients who underwent a cheek advancement flap in the Catharina Hospital Eindhoven between January 2006 and January 2013 where included and assessed by means of a retrospective chart review and a survey about the long-term outcome and patient satisfaction. Results: A retrospective chart review was performed on all 54 eligible patients, and 41 (76%) of these patients participated in the study. The mean follow-up was 3.5 years (SD = 2.0, range = 1-7 years). Early complications were ectropion (6%), infection (2%), dog-ears (1%), haematoma (4%), and distal tip necrosis (2%). Late outcome and complications were sensory neuropathies (41%), late ectropion (7%), hypopigmentation of scars (29%), contractures (27%), and abnormal hair distribution (17%). Patients rated their reconstruction as good or excellent in 87% of cases. Conclusions: The cheek advancement flap is a suitable technique for reconstruction of large cheek skin defects after excision of skin malignancies. However, patients should be informed that long-term complications, including ectropion, can occur. Additional follow-up might lead to an early detection of these late effects.

International audience; Homozygous frameshift variants in CNTNAP1 have recently been reported in patients with arthrogryposis and abnormal axon myelination. In two brothers with severe congenital hypotonia and foot deformities, we identified compound heterozygous variants in CNTNAP1, reporting the first causative missense variant, p.(Cys323Arg). Motor nerve conductions were markedly decreased. Nerve microscopical lesions confirmed a severe hypomyelinating process and showed loss of attachment sites of the myelin loops on the axons, which could be a characteristic of Caspr loss-of-function. We discuss the pathophysiology of the myelination process and we propose to consider this disorder as a congenital hypomyelinating neuropathy.

We report a rare case of a dual neuro-vascular variation, which was observed in the right extremity of male cadaver. About an inch inferior to the elbow joint, three branches arose from the median nerve. These were the anterior interosseous branch, a Martin-Gruber branch (MGB) and a muscular branch. The MGB coursed infero-medially to join with the ulnar nerve by running posterior to the ulnar artery. It was surprising to observe that the MGB passed between the ulnar artery and its venae comitantes. There was an acute angulation of the MGB here, suggesting entrapment at this site.

International audience; BACKGROUND: Fingolimod is approved for the treatment of relapsing-remitting multiple sclerosis and was effective in experimental autoimmune neuritis in rats, a possible model for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We aimed to evaluate the efficacy of fingolimod in delaying disability progression in patients with CIDP who withdrew from currently effective treatments (intravenous immunoglobulin [IVIg] or corticosteroids). METHODS: This double-blind, multicentre, randomised, placebo-controlled, parallel-group, event-driven study was done at 48 neurology centres in Australia, Canada, Israel, Japan, the USA, and nine countries in Europe. Participants with CIDP who were receiving IVIg or corticosteroids were randomly assigned (1:1) to once-daily oral fingolimod 0\textperiodcentered5 mg or placebo. Owing to the event-driven design, treatment duration was flexible and could be up to 4\textperiodcentered5 years. Randomisation was done with an automated interactive voice response-web response system and was stratified by Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale scores. Previous IVIg treatment was discontinued after one final course ending the day before the first dose of fingolimod or placebo was given, whereas corticosteroids were tapered off over 8 weeks after randomisation. The primary endpoint was time to first confirmed worsening (>=1 point increase on the adjusted INCAT disability scale score versus baseline) and was assessed in the full analysis set, which consisted of all patients who underwent randomisation and had at least one efficacy assessment for the primary analysis. The survival distribution functions of time to first worsening were estimated within each treatment group according to the Kaplan-Meier survival distribution function and compared with a stratified log-rank test. The trial is registered with ClinicalTrials.gov, number NCT01625182. FINDINGS: Of 106 participants randomly assigned between Jan 24, 2013, and March 10, 2016, 54 received fingolimod (41 who had been receiving IVIg and 13 who had been receiving corticosteroids) and 52 received placebo (41 who had been receiving IVIg and 11 who had been receiving corticosteroids). The trial ended for futility as recommended by an independent data monitoring committee after an interim analysis when 44 confirmed worsening events had occurred. At the end of the study, the survival estimate of the proportion of participants free from confirmed worsening was not significantly different between the fingolimod group (42%, 95% CI 23-60) and the placebo group (43%, 28-59; p=0\textperiodcentered91). Adverse events occurred in 41 (76%) participants receiving fingolimod and 44 (85%) on placebo, and serious adverse events occurred in nine (17%) and four (8%) patients, respectively. The most common adverse events with fingolimod were headache (12 [22%] patients), hypertension (ten [19%]), and extremity pain (seven [13%]). Adverse events leading to study discontinuation occurred in seven (13%) participants on fingolimod and none on placebo. INTERPRETATION: Fingolimod 0\textperiodcentered5 mg once-daily was not better than placebo for the treatment of CIDP. Future trial designs should take account of the possibility that if IVIg is stopped abruptly, some patients might relapse soon afterwards whereas others might remain in remission. FUNDING: Novartis Pharma.

Background: Nerve conduction studies (NCSs) are essential for diagnosing various kinds of focal and diffuse neuropathies. Due to the paucity of local NCS data, electrodiagnostic laboratories in Punjab rely on values from Western and other Indian studies. Aim: This study was conducted to provide normative data for median nerve conduction parameters (motor and sensory) in Punjabi populace. Materials and Methods: A cross-sectional study was done on 290 participants (150 males and 140 females), aged 17–21 years, as per standardized protocol. The data were analyzed separately for both genders using SPSS version 20. It consisted of distal latencies and conduction velocities of motor and sensory divisions of median nerve. Student's unpaired t-test was used for statistical analysis. Results: There was no effect of gender on any of the median nerve conduction parameters. Height and weight had nonsignificant negative and positive correlation, respectively (P > 0.05), with conduction velocity in both motor and sensory median nerves. For median motor nerve, the values of distal latency and conduction velocity in males were 2.9 ± 0.16 ms and 60.25 ± 2.99 m/s, respectively, whereas, in females, they were 2.6 ± 0.43 ms and 59.83 ± 2.82 m/s. Similarly, for median sensory nerve, the latency and velocity values in males were 2.8 ± 0.56 ms and 54.81 ± 3.70 m/s, whereas, in females, they were 2.4 ± 0.33 ms and 54.56 ± 3.65 m/s, respectively. Conclusion: The data in this study compared favorably with already existing data. It would help the local electrodiagnostic laboratories in assessing the median nerve abnormalities with greater accuracy in this population subset.

The primary aim of this paper was to assess the relevance of high-frequency ultra-sound examination in qualifying patients for either surgical or conservative treatment of peripheral entrapment neuropathies. The study was conducted in a group of 55 patients aged 7-83 (mean age 43.6), including 28 males and 27 females, who in 2009-2011 were referred to an ultrasound examination due to a clinical suspicion of entrapment neuropathies. For the purposes of the analysis, the patients were divided into four groups: carpal tunnel syndrome (1), ulnar nerve entrapment (2) (cubital tunnel syndrome and Guyon's canal syndrome), posterior interosseous nerve syndrome (3) and other entrapment neuropathies (4). The cases of isolated idiopathic carpal tunnel syndrome were excluded from the analysis. All patients underwent the interview, physical examination and ultrasound examination. Ultrasound examinations were performed with Esaote MyLab 50 and MyLab 60 systems using high-frequency broadband linear transducers: 6-18 MHz. Sixty-seven percent of patients (37 persons) underwent a neurophysiological test. Nerve echostructure, its hyperemia as well as nerve cross-sectional area or, in the case of small nerves, diameter were assessed in all patients. Furthermore, the following were assessed in individual groups: notch sign in group 1, nerve instability in a dynamic ultrasound examination in group 2, nerve angulation in a dynamic ultrasound examination and tenderness on nerve compression at the site of the visualized pathology in group 3. The analyses of the collected material were performed by means of descriptive statistics. The results of clinical and surgical verification were consistent with ultrasound findings in 96.4%. The results indicate that high-frequency ultrasonography is a valuable method in qualifying patients for various types of treatment of peripheral neuropathies resulting from compression.Podstawowym celem pracy była ocena przydatności badania ultrasonograficznego z zastosowaniem głowic wysokiej częstotliwości w kwalifikowaniu do leczenia operacyjnego albo zachowawczego neuropatii obwodowych o charakterze uciskowym. Materiał pracy stanowiła grupa 55 osób w wieku 7–83 lata (średnia wieku 43,6 roku), w tym 28 mężczyzn i 27 kobiet, kierowanych w latach 2009–2011 na badanie ultrasonograficzne z klinicznym podejrzeniem neuropatii uciskowych, na potrzeby analizy podzielonych na cztery grupy: zespół kanału nadgarstka (1), zespół ucisku nerwu łokciowego (2) (ucisk na poziomie rowka nerwu łokciowego oraz ucisk na poziomie kanału Guyona), zespół ucisku nerwu międzykostnego tylnego (3), inne neuropatie uciskowe (4). Z analizowanej grupy wyłączono przypadki izolowanego idiopatycznego zespołu kanału nadgarstka. U wszystkich pacjentów przeprowadzono badanie podmiotowe, przedmiotowe oraz badanie ultrasonograficzne. Badania ultrasonograficzne przeprowadzono aparatami Esaote MyLab 50 oraz MyLab 60 z zastosowaniem szerokopasmowych głowic liniowych o wysokich częstotliwościach: 6–18 MHz. U 67% pacjentów (37 osób) wykonano badanie neurofizjologiczne. U wszystkich pacjentów oceniano echostrukturę nerwu i jego przekrwienie oraz określano pole powierzchni przekroju nerwu albo – w przypadku drobnych nerwów – średnicę. Dodatkowo w grupie 1 oceniano objaw wcięcia, w grupie 2 – niestabilność nerwu w dynamicznym badaniu ultrasonograficznym, w grupie 3 – zaginanie kątowe nerwu w dynamicznym badaniu ultrasonograficznym oraz tkliwość w czasie ucisku nerwu w miejscu uwidocznionej patologii. Analizy zebranego materiału dokonano za pomocą statystyki opisowej. W odniesieniu do weryfikacji klinicznej i operacyjnej zgodność z rozpoznaniem ultrasonograficznym osiągnięto w 96,4% przypadków. Uzyskane wyniki wskazują, że badanie ultrasonograficzne z zastosowaniem głowic wysokiej częstotliwości jest cenną metodą w kwalifikowaniu do rodzaju leczenia neuropatii obwodowych o charakterze uciskowym.

AimsMyopathy or neuropathy has been associated with lamivudine/telbivudine therapy in hepatitis B patients. We aim to describe the pathological changes of lamivudine/telbivudine-associated neuromyopathy.MethodsWe retrospectively recruited six patients who were diagnosed with nucleotide analogues-associated myopathy or neuropathy. Muscle and nerve biopsy were performed, and the specimens were prepared for the light microscopy and electron microscopy. Genomic DNA was extracted from frozen muscle specimens, and the mitochondrial DNA (mtDNA) content was quantified by real-time PCR.ResultsRecovery of the myopathy can be achieved after the discontinuation or changing the drugs to entecavir. Muscle and nerve biopsy revealed similar changes under either the light or electronic microscopy in all the subjects. Quantitative real-time PCR revealed decrease of mtDNA content in the affected muscle.ConclusionsMtDNA depletion results in mitochondrial dysfunction in the lamivudine/telbivudine-associated neuromyopathy. Myopathy was characterised by mitochondrial dysfunction accompanied with neurogenic damage due to axonal neuropathy. Ultrastructure changes of mitochondria included vacuolisation, simplification of the cristae and homogenised matrix.

Transplantation of mesenchymal stromal cells (MSCs) to sites of experimentally-created nerve injury in laboratory animals has shown promising results in restoring nerve function. This approach for nerve regeneration has not been reported in horses. In this study, we first evaluated the in vitro ability of equine, bone marrow-derived MSCs (EBM-MSCs) to trans-differentiate into Schwann-like cells (SLCs) and subsequently tested the MSCs, in vivo for their potential to regenerate a transected nerve after implantation. The EBM-MSCs from 3 equine donors were differentiated into SCLs for 7 days, in vitro, in the presence of specialized differentiation medium and evaluated for morphological characteristics, by using confocal microscopy, and for protein characteristics, by using selected Schwann-cell markers (GFAP and S100b). The EBM-MSCs were then implanted into the fascia surrounding the ramus communicans of one fore limb of 3 healthy horses after a portion of this nerve was excised. The excised portion of the nerve was examined histologically at the time of transection, and stumps of the nerve were examined histologically at day 45 after transplantation. The EBM-MSCs from all donors demonstrated morphological and protein characteristics of those of Schwann cells 7 days after differentiation. Nerves implanted with EBM-MSCs after nerve transection did not show evidence of nerve regeneration at day 45. Examination of peripheral nerves collected 45 days after injury and stem cell treatment revealed no histological differences between nerves treated with MSCs and those treated with isotonic saline solution (controls). The optimal delivery of MSCs and the model suitable to study the efficacy of MSCs in nerve regeneration should be investigated.

Suprascapular nerve entrapment was first described in 1959 by Kopell and Thompson. Although rare, this condition is among the causes of poorly explained shoulder pain in patients with manifestations suggesting a rotator-cuff tear but normal tendons by imaging studies. Suprascapular nerve entrapment may cause 2% of all cases of chronic shoulder pain. Among the many reported causes of suprascapular nerve entrapment, the most common are para-labral cysts, usually in the spinoglenoid notch, and microtrauma in elite athletes. The potential relevance of concomitant rotator-cuff tears remains debated. Less common causes include tumours, scapular fractures, and direct trauma involving traction. Early diagnosis and treatment are crucial to avoid the development of irreversible muscle wasting. Endoscopic surgery to treat the various causes of suprascapular nerve compression has superseded open nerve release.

F-waves are low amplitude responses produced by antidromic activation of motoneurons. They may not appear after each stimulus and are inherently variable in latency, amplitude, and configuration. Meaningful analysis of F-waves requires an appreciation of these characteristics of F-waves as well as an understanding of their physiology. These features of F-waves as well as their physiology are reviewed. This is important since F-waves are one of the most frequently used studies in clinical neurophysiology and much of the controversies surrounding the use of F-waves relates to a failure to adequately consider the requirements of F-wave analysis. These requirements include the number of F-waves that need to be recorded, the parameters that should be evaluated, and the muscle from which the F-waves are recorded. If analyzed correctly, current reports would indicate that F-waves are the most sensitive and reliable nerve conduction study for evaluating polyneuropathies, can be abnormal in focal proximal nerve dysfunction, can be at least as sensitive as needle electromyography for defining lumbosacral radiculopathies, and can provide a meaningful physiological window into disorders of the central nervous system. Reports supporting these statements and their clinical relevance are discussed.

La polyarthrite rhumatoïde est le rhumatisme inflammatoire chronique le plus fréquent. Ses complications neurologiques sont dominées par les neuropathies périphériques, cliniquement asymptomatiques, mais peuvent être détectées sur l’électromyogramme (EMG). Nous rapportons l’observation d’une patiente qui a présenté une neuropathie sensitivo-motrice aux deux membres inférieurs associée à une sciatique de topographie L5 d’origine rhumatoïde.Key words: Sciatique non discale, polyarthrite rhumatoïde, neuropathies

Background Charcot–Marie–Tooth 1A (CMT1A) patients show a reduction of spontaneous activities of daily living measured by means of questionnaires or pedometers, which are quite inaccurate compared to recent measurement techniques. Aim The study aimed at quantifying daily living activities in CMT1A patients by means of inertial sensors, which give information not only on the amount but also on the intensity of these activities. Materials and methods Time and count (amount), and velocity and power (intensity) of 24 h daily living activities were measured in eight patients (20–48 years; Barthel >90; Tinetti >20) and eight healthy individuals, matched for age and gender, by means of a wearable inertial sensor device. Results There were no differences between patients and controls in the 24-h distance covered and count of steps. However, count of step climbing and sit to stand were lower in patients than in controls (139.93 ± 141.66 vs. 341.06 ± 164.07 n and 58.23 ± 7.82 vs. 65.81 ± 4.75 n, respectively; P

Background Alpha-synuclein (α-syn) is abundantly expressed in the central nervous system and involved in the regulation of neurotransmission. Insoluble fibrils of phosphorylated α-synuclein (p-α-syn) have been implicated in several neurodegenerative diseases (e.g. Parkinson's disease, Alzheimer's disease). The aim of the study was to determine the gene expression pattern and localization of α-syn/p-α-syn in the human enteric nervous system (ENS). Methods Human colonic specimens (n = 13, 15–83 years) were processed for α-syn and p-α-syn immunohistochemistry. Colocalization of α-syn was assessed by dual-labeling with pan-neuronal markers (PGP 9.5, HuC/D). For qPCR studies, tissue was obtained from full-thickness sections, tunica muscularis, submucosa, mucosa, and laser-microdissected (LMD) enteric ganglia. Results Highest α-syn levels were detectable within the tunica muscularis and submucosa. Ganglia isolated by LMD showed high expression of α-syn mRNA. All myenteric and submucosal ganglia and nerve fibers were immunoreactive for α-syn. Dual-labeling revealed colocalization of α-syn with both pan-neuronal markers. p-α-syn immunoreactivity was consistently observed in specimens from adults with increasing age. Conclusions α-syn is abundantly expressed in all nerve plexus of the human ENS including both neuronal somata and processes. The presence of p-α-syn within the ENS is a regular finding in adults with increasing age and may not be regarded as pathological correlate. The data provide a basis to unravel the functions of α-syn and to evaluate altered α-syn in enteric neuropathies and α-synucleinopathies of the CNS with gastrointestinal manifestations.

We describe two sibs with chorioretinal dystrophy, hypogonadotrophic hypogonadism, and cerebellar ataxia, Boucher-Neuhäuser syndrome, a rare but distinct pleiotropic single gene disorder with an autosomal recessive pattern of inheritance. The cases presented illustrate that this syndrome is still poorly recognised. We provide a review and analysis of previously reported cases and the differential diagnosis, which might aid in the identification of additional cases.

ContextGastroparesis is defined by delayed gastric emptying without mechanical obstruction of the gastroduodenal junction, which has been increasingly investigated. Nevertheless, knowledge on the relationships between etiology, symptoms and degree of delayed gastric emptying is limited.ObjectivesThe demographic, clinical and etiological features of Brazilian patients with gastroparesis were studied and the relationships between these findings and the severity of gastric emptying were determined.MethodThis is a retrospective study of medical records of 41 patients with symptoms suggestive of gastroparesis admitted between 1998 and 2011, who had evidence of abnormally delayed gastric emptying on abdominal scintigraphy. Cases with idiopathic gastroparesis were compared with those of patients with neurologic disorders or diabetes mellitus, in whom autonomic neuropathy is likely to occur.ResultsThe majority of the patients were women (75.6%) with a median age of 41 years and a long-term condition (median: 15 years). Twelve patients (29.3%) had a body mass index of less than 20 kg/m2. The most common presenting symptoms were dyspepsia (53.6%), nausea and vomiting (46.3%), weight loss (41.4%) and abdominal pain (24.3%). Regarding etiology, 16 patients had digestive disorders including idiopathic gastroparesis (n = 12), 12 had postoperative conditions, 11 had diseases of the nervous system, five had diabetes mellitus and in three cases gastroparesis was associated to a variety of conditions. In the majority of patients (65.8%) gastric emptying was severely delayed. There was no association between etiology of gastroparesis, type of presenting symptoms and the degree of delay in gastric emptying. Gastroparesis patients with proven (neurological conditions) or presumed (diabetes) nervous system involvements were significantly younger (P= 0.001), had more recent symptom onset (P= 0:03) and a trend towards more severe gastric empty (P = 0:06). There were no significant differences between this subgroup of patients and that comprising cases of idiopathic gastroparesis regarding any of the variables studied.ConclusionsThe demographic, clinical and etiological characteristics of Brazilian patients with gastroparesis are quite varied, but there is a predominance of women with long-standing symptoms and marked delay in gastric emptying. The type of presenting symptoms and the degree of delay in gastric emptying do not predict the etiology of gastroparesis. However, severely delayed gastric emptying in younger patients with recent symptom onset should raise the suspicion of impaired neural control of gastro-duodenal motility. ContextoA gastroparesia é uma condição clínica definida por retarde anormal do esvaziamento gástrico, sem obstrução mecânica antro-piloro-duodenal, que vem sendo crescentemente estudada. No entanto, as relações entre etiologia, sintomas e grau de retarde do esvaziamento gástrico na gastroparesia são ainda pouco conhecidas.ObjetivosEstudar as características demográficas, clínicas e etiológicas e o grau de retarde do esvaziamento gástrico em pacientes brasileiros com gastroparesia e comparar os casos idiopáticos com aqueles com doenças do sistema nervoso ou diabete melito, em que é alta a probabilidade de ocorrência de neuropatia autonômica.MétodoEstudo retrospectivo dos prontuários de 41 casos com sintomas sugestivos de gastroparesia admitidos entre 1998 e 2011, que tiveram comprovação de retarde anormal do esvaziamento gástrico em exame cintigráfico específico, que foi também revisto.ResultadosDentre os 41 casos, 31 (75,6%) eram mulheres; a mediana de idade foi de 41 anos e predominou quadro clínico de longa duração (mediana: 15 anos). Doze (29,26%) pacientes apresentavam índice de massa corporal inferior ao limite de 20 kg/m2. Os sintomas mais frequentes foram a dispepsia (53,6%), náuseas e vômitos (46,3%), perda de peso (41,4%) e dor abdominal (24,3%). Quanto à etiologia, 16 pacientes tinham afecções digestivas, incluindo a gastroparesia idiopática (n = 12), 12 eram de estados pós-operatórios, 11 tinham doenças do sistema nervoso, 5 tinham diabete melito e 3 eram de etiologias variadas. A maioria dos pacientes (65,8%) apresentou retarde do esvaziamento gástrico intenso ou muito intenso. Não houve qualquer associação entre etiologia da gastroparesia, sintomas apresentados e grau de retarde do esvaziamento gástrico. Nos casos com comprometimento neurológico comprovado ou presumido (diabéticos), os pacientes eram significativamente mais jovens (P = 0.001), com início mais recente dos sintomas (P = 0.03) e com tendência a retarde mais acentuado do esvaziamento gástrico (P = 0.06). Não houve diferença significativa entre este subgrupo e os casos de gastroparesia idiopática quanto a nenhuma das variáveis estudadas.ConclusõesAs características clínicas e demográficas dos casos de gastroparesia são variadas, mas há predomínio de mulheres com quadro clínico de longa duração. Os sintomas apresentados e o grau de retarde do esvaziamento gástrico não predizem a etiologia da gastroparesia. No entanto, retarde muito intenso do esvaziamento gástrico em pacientes mais jovens e com sintomas de início mais recente devem levantar a suspeita de prejuízo ao controle neural da motilidade gástrica. Pacientes com gastroparesia idiopática tem características clínicas e grau de retarde do esvaziamento gástrico semelhantes aos de casos com comprometimento neurológico comprovado ou presumido.

Molecular pathology of benign prostatic hyperplasia is multifactorial and involves endocrine, biochemical, immunological interactions. The mechanisms involved in the onset and progression of benign prostatic hyperplasia are: infections, older than 50 years, imbalances in hormones and neurotransmitters, inflammation, oxidative stress. It is estimated that an infectious etiology can be a cause of wrong immune response directed at the prostate. Inflammatory neuropathies often occur after infections with various microorganisms. It is also known, that the presence of microorganisms is heterogeneous in patients with benign prostatic hyperplasia. In this paper we documented the antibody profile of anti-GM1, -GM2, -GM3, -GD1a, -GD1b, -GT1b, -GQ1b IgG and IgM type in 30 patients with benign prostatic hyperplasia and in 30 controls. The results showed an increasing anti-GD1a and anti-GQ1b titer in patients with benign prostatic hyperplasia compared to control. The authors suggest that a careful monitoring of the patients developing an endogenous anti-gangliosidic immune response is required in order to assess these antibodies as potential risk factors for neuropathy in patients with benign prostatic hyperplasia.