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Introduction We present a case series of six treatment‐naive patients with clinical phenotypes compatible with chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy without electrodiagnostic features of demyelination but with abnormal peripheral ultrasound findings who responded to treatment. Methods All six patients underwent a complete set of ancillary investigations, including extensive nerve conduction studies. We also performed standardized nerve ultrasound of median nerves and brachial plexus as part of a larger effort to evaluate diagnostic value of sonography. Results Nerve conduction studies did not show conduction block or other signs of demyelination in any of the six patients. Sonographic nerve enlargement was present in all patients and was most prominent in proximal segments of the median nerve and brachial plexus. Treatment with intravenous immunoglobulin resulted in objective clinical improvement. Discussion Our study provides evidence that nerve ultrasound represents a useful complementary diagnostic tool for the identification of treatment‐responsive inflammatory neuropathies.

Ultrasound can be used to visualize pathology in the peripheral nerves of patients with polyneuropathy. Nerve enlargement is the most frequent pathology, but other abnormalities, including abnormal nerve echogenicity and vascularity, are also encountered. This monograph presents an overview of the role of nerve ultrasound in the evaluation and management of both inherited and acquired polyneuropathies. A description of the sonographic techniques and common abnormalities is provided, followed by a presentation of typical findings in different neuropathies. Scoring systems for characterizing the presence and pattern of nerve abnormalities as they relate to different polyneuropathies are presented. Muscle Nerve 57: 716-728, 2018.

Alemtuzumab is a lymphocyte-depleting antibody and one of the most effective treatments for relapsing multiple sclerosis. However, it also causes loss of immune-tolerance leading to secondary autoimmunity and marked anti-drug antibody responses. Although these anti-drug responses have been reported to be of no significance, we hypothesized that they will affect the depleting capacity and treatment response in some individuals. This was found following analysis of the regulatory submission of the pivotal phase III trials, which was obtained from the European Medicines Agency. At the population level there was lack of influence of 'ever-positive' alemtuzumab-specific antibody responses on lymphocyte depletion, clinical efficacy and adverse effects during the 2-year trial. This was not surprising as no one before the first infusion, and only 0·6% of people before the second-infusion, had pre-infusion, neutralizing antibodies (NAbs). However, at the individual level, NAbs led to poor lymphocyte depletion. Importantly, it was evident that 31% of people had NAbs and 75% had binding antibodies at the end of treatment-cycle 2, which suggests that problems may occur in people requiring additional alemtuzumab cycles. In addition, we also identified individuals, following 'post-marketing' alemtuzumab use, whose lymphocyte level was never effectively depleted after the first infusion cycle. Hence, although alemtuzumab depletes lymphocytes in most individuals, some people fail to deplete/deplete poorly, probably due to biological-response variation and NAbs, and this may lead to treatment failure. Monitoring depletion following infusion and assessment of the neutralizing response before re-infusion may help inform the decision to retreat or switch therapy to limit treatment failure.

Introduction Neurofibromatosis type 2 (NF2) is mainly associated with central nervous system (CNS) tumors. Peripheral nerve involvement is described in symptomatic patients, but evidence of subclinical peripheral nerve involvement is scarce. Methods We conducted a cross-sectional pilot study in 2 asymptomatic and 3 minimally symptomatic patients with NF2 to detect subclinical peripheral nerve involvement. Patients underwent clinical examination, nerve conduction studies (NCS), and high-resolution ultrasonography (HRUS). Results A total of 30 schwannomas were found, divided over 20 nerve segments (33.9% of all investigated nerve segments). All patients had at least 1 schwannoma. Schwannomas were identified with HRUS in 37% of clinically unaffected nerve segments and 50% of nerve segments with normal NCS findings. Discussion HRUS shows frequent subclinical peripheral nerve involvement in NF2. Clinicians should consider peripheral nerve involvement as a cause of weakness and sensory loss in the extremities in patients with this disease. Muscle Nerve 57: 312-316, 2018.

Introduction: We quantified intraneural blood flow (INBF) in 18 patients with end-stage kidney disease (ESKD) and examined its relationship with nerve size, neuropathy severity and nerve excitability parameters. Methods: Sonographic measurements of the median nerve were performed at the same site before and after haemodialysis. INBF was quantified by analysing power Doppler sonograms to obtain the vessel score (VSc) and maximum perfusion intensity(MPI). Corresponding median motor nerve excitability studies were performed. Neuropathy severity was assessed using total neuropathy score (TNS). Results: 39% of ESKD patients had detectable INBF compared to none in the control group (P

Introduction We aimed to identify prognostic factors in ulnar neuropathy at the elbow (UNE) and developed a scoring system to predict the prognosis. Methods We collected baseline clinical, electrophysiological, and ultrasonographical data from 2 cohorts. The outcome for all patients were determined on follow up. Prognostic factors were determined using single and multiple variable analyses. A points system was developed to determine the risk for an unfavorable outcome. Results Of 220 patients with UNE 178 (81%) could be re-evaluated. Four variables were retained in the prediction model for a points system. An unfavorable outcome was associated with right sided UNE, more severe weakness of the abductor digiti minimi (ADM), and more pronounced ulnar nerve thickening. Compound muscle action potential amplitude reduction across the elbow ≥ 16% (particularly ≥ 50%) was associated with a more favorable outcome. Discussion Outcome in UNE may be predicted by scoring 4 parameters. This article is protected by copyright. All rights reserved.

Introduction We conducted a randomized, double-blind, placebo-controlled trial to evaluate the effect of ultrasound-guided corticosteroid injection in patients with ulnar neuropathy at the elbow (UNE). Methods Fifty-five patients were randomized between an ultrasound-guided injection of 1 ml containing 40 mg methylprednisolone acetate and 10 mg lidocaine hydrochloride or a placebo injection. The primary outcome was the subjective change of symptoms after 3 months. The secondary outcomes were change in electrodiagnostic studies and ultrasonography findings. Results A success rate of 30% was found in the corticosteroid injection group versus 28% in the placebo injection group. Only the nerve cross-sectional area changed significantly in the intervention group, from a mean of 11.9 mm2 to 10.9 mm2. Conclusions We could not demonstrate a positive effect of ultrasound-guided corticosteroid injection in UNE compared with placebo. Favorable outcomes may be attributed to the natural course of UNE or the effect of patient education. Muscle Nerve 52:380–385, 2015

Introduction Ultrasound (US) measurement of the median nerve cross-sectional area (CSA) at the wrist is a useful diagnostic test for carpal tunnel syndrome (CTS). We compared median nerve normal values between samples of Indian and Dutch populations. Methods: The median nerve was examined by US at the wrist in 100 healthy volunteers in India and 137 volunteers in The Netherlands using the same protocol. Results: Median nerve CSA at the wrist (7.0 ± 1.1 mm2) in the Indian cohort was lower in comparison to the Dutch cohort (8.3 ± 1.9 mm2; P

Introduction In 30% of patients with common fibular (CF) neuropathy at the fibular head, reliable localization of the site of the lesion by means of electrodiagnostic testing is challenging. Methods We prospectively assessed proximal CF nerve cross-sectional area (CSA) measurements and at the fibular head in 87 patients with CF neuropathy and 16 with a different condition. Reference values were obtained in 64 healthy volunteers. Results Patients with CF neuropathy had a significantly larger CF nerve CSA than controls and patient controls (P 8 mm2 with a sensitivity of 90% (CI 81–95%) and a specificity of 69% (CI 58–78%). Conclusion High-resolution sonography in addition to electrodiagnostic testing improves diagnostic reliability in CF neuropathy. Muscle Nerve, 48: 171–178, 2013

Introduction: The aim of this study was to assess the presence of (sub)luxation of the ulnar nerve in patients with ulnar neuropathy at the elbow (UNE) compared with healthy controls (HC). We assessed its clinical patterns, electrodiagnostic, and sonographic characteristics. Methods: Using high-resolution sonography, we studied the incidence of (sub)luxation in a cohort of 342 patients and 70 HC. Results: Subluxation occurred in 14% and luxation in 6.7% of the UNE patients versus 5.7% and 5.7%, respectively, in HC (no significant differences). Pain at the elbow occurred more often in patients with (sub)luxation (P = 0.007). Electrodiagnostic and sonographic findings did not differ between patients with or without (sub)luxation. Conclusions: The incidence of ulnar nerve (sub)luxation between patients with UNE and HC does not differ. UNE patients with (sub)luxation do not have specific clinical or electrodiagnostic findings, apart from experiencing pain at the elbow more often. Muscle Nerve 47: 849–855, 2013

Introduction: Normative cross-sectional areas (CSAs) have been obtained for the Western population. We obtained CSAs of normal ulnar nerves at predetermined sites and correlate them with electrophysiological variables in Asian subjects. Methods: One hundred healthy volunteers, mean age 39 ± 14 years (range, 18–75 years), were recruited for the study after obtaining informed consent. The ulnar nerve was examined ultrasonographically from wrist to axilla, and CSA was measured at predetermined sites. All subjects underwent a simultaneous standardized nerve conduction study. Results: Men had larger CSAs, and CSAs increased with advancing age. There was a statistically significant correlation between CSA at the wrist and distal ulnar motor latency (P = 0.005). Conclusions: Ulnar CSA correlated with age, gender, and distal motor latency. No correlations were observed with height, weight, or body mass index. Muscle Nerve 47: 673–676, 2013

Introduction: The aim of this study was to establish the prevalence of increased intraneural vascularization detected by ultrasonography (IVUS) in patients with ulnar neuropathy at the elbow (UNE) and to determine its relationship to clinical, ultrasonographic, and electrodiagnostic findings. Methods: High-resolution ultrasonography and color Doppler imaging were performed in 137 patients with confirmed UNE, 24 patient controls, and 70 healthy controls (HCs). Results: IVUS was found in 21 (15%) of 137 patients with UNE, in 1 (4%) of 24 patient controls, and in 0 of 70 HCs (P = 0.001). Patients with IVUS were more likely to have severe weakness (P = 0.01), severe atrophy of ulnar-innervated muscles (P = 0.008), axonal damage (P = 0.001), and more pronounced nerve enlargement (P = 0.03) than those without IVUS. Conclusions: IVUS in the ulnar nerve can be detected in patients with UNE and is associated with nerve enlargement and clinical and electrodiagnostic severity. In addition, IVUS is associated with axonal damage. Muscle Nerve, 2013

Background and purpose A new treatable venous disorder, chronic cerebrospinal venous insufficiency (CCSVI), has been proposed in patients with multiple sclerosis (MS). Its relationship with iron metabolism is suggested, but has not been examined prospectively. Methods We performed extra- and transcranial echo colour Doppler (ECD) in 90 patients with MS and 41 healthy controls (HC). Indices of iron metabolism and the presence of peripheral signs of impaired venous flow were also examined. Results The ECD examination showed CCSVI in 8 (9%) of the 90 patients with MS and 0 HC (P = 0.11). The 8 CCSVI-positive MS patients were older (P = 0.02), had less often relapsing–remitting-MS (P = 0.02) and had more neurological disability assessed by expanded disability status scale (EDSS, P = 0.001) and longer duration of disease (P = 0.02) in comparison with the 82 CCSVI-negative MS patients. Multivariate analysis revealed that EDSS remained an independent factor associated with CCSVI (odds ratio 1.89, 95%CI 1.17–3.05, P-value = 0.009). CCSVI MS patients more often had bilateral telangiectasia at the legs (P = 0.008), reticular veins (P = 0.006) and venous stasis dermatitis (P = 0.004). No relationship was found between CCSVI and impaired iron metabolism in patients with MS. Conclusions CCSVI is uncommon and is a secondary epiphenomenon in MS and related to more neurological disability and the presence of varicose veins at the legs.

Introduction: From histopathological studies of peripheral nerves in leprosy, it is known that the epineurium can be thickened. We measured the epineurial thickness of the ulnar nerve by high resolution sonography (HRUS). Methods: The epineurium of the ulnar nerve was measured above the elbow on transverse scan in 25 healthy controls and 26 leprosy patients. Results: The mean epineurial thickness was 0.77 mm (95% confidence interval [CI] 0.66–0.88) in symptomatic ulnar nerves (n = 20), 0.58 mm (CI 0.51–0.65) in asymptomatic nerves (n = 30), and 0.49 mm (CI 0.44–0.54) in healthy controls (n = 25) (P = 0.0001). This thickening was related to the cross-sectional area of the ulnar nerve, but not with increased blood flow. Conclusions: The epineurium of the ulnar nerve can be measured with the use of HRUS, and it is often strikingly thickened in leprosy patients, especially in those with ulnar involvement. Muscle Nerve 46:38–41, 2012

Background and purpose: Self-reports of cognitive functioning are not always related to objective measures. We examined psychological characteristics of patients with multiple sclerosis (MS) who underestimate, overestimate or accurately estimate their executive performance. Methods: The first phase was an inventory of cognitive complaints by means of self-reported (and partner-reported) questionnaires. At the second phase (January–October 2009), 114 of the 128 participants met the inclusion and exclusion criteria and underwent cognitive and neurological assessments. Results: A total of 19% (N = 22) of participants reported subjective executive impairment, whilst 81% (N = 92) reported no subjective executive impairment. Based on Behavioural Assessment of the Dysexecutive Syndrome–Dysexecutive Questionnaire self-reports, 67% (N = 76) of the participants accurately reported no subjective executive impairment, 14% (N = 16) overestimated, and 15% underestimated (N = 17) their executive performance; 78% of the informants accurately reported no subjective executive impairment, 15% overestimated the patient’s executive performance, and 4% underestimated the patient’s executive performance. Patients with MS underestimating their executive performance were characterized by more depression (F(2,106 = 12.9, P

A young man presented with progressive motor weakness and atrophy of the ulnar muscles of his left hand over a period of more than 2 years. Electrodiagnostic studies indicated an ulnar nerve lesion, but it was not localized. High-resolution sonography of the ulnar nerve revealed an enlarged and hypoechogenic ulnar nerve at an unusual location, namely 12.5 cm proximal to the medial epicondyle. Histology showed that this was an intraneural perineurioma. High-resolution sonography of the ulnar nerve is very useful in the discovery of this unusual location of nerve pathology and may assist in its early detection.

Background: Variations in the treatment of multiple sclerosis (MS) occur. This study was performed to obtain insight into the reasons why patients with MS use or do not use immunomodulating treatment (DMT). Methods: We held a survey on the use of DMT in a group of 1572 patients with MS. Results: Response rate was 89%. Of the patients, 31% were never on DMT, 21% had stopped DMT, 30% were still on the first DMT, and 18% had changed. The treatment-naive patients were significantly older, had a longer duration of disease, and had a lower number of relapses per year (P

Introduction: Ultrasonography of the ulnar nerve has been recommended as a useful additional test in ulnar neuropathy at the elbow (UNE). Methods: We searched the literature and systemically reviewed all clinical trials in UNE. We also looked for articles about the normal sonoanatomy and spe- cific causes of UNE. Results: Seven of 14 clinical trials in UNE were suitable for further analysis. Ultrasonographic ulnar nerve size measurement appears to be a test with good diag- nostic accuracy. The most frequently reported abnormality was an increased cross-sectional area of the ulnar nerve at the elbow. However, several studies had methodological flaws. In addition, the ultrasonographic techniques and study designs dif- fered among the studies. There were a few other uncontrolled studies about the underlying causes of UNE. Discussion: The role of ultrasonography in UNE seems promising but could not be firmly established. More prospective studies are needed, and we make several recommendations for further research. Muscle Nerve 43: 627-635, 2011 The diagnosis of ulnar neuropathy at the elbow (UNE) may seem straightforward in the majority of cases. Clinicians will usually suspect an ulnar neu- ropathy when the characteristic symptoms and signs are present, and electrophysiological studies will eventually confirm the diagnosis. However, UNE has to be distinguished from other disorders and from ulnar neuropathy at other levels. Clinical signs indicating a lesion of the ulnar nerve above the level of the wrist (e.g., paresis of the flexor carpi ulnaris or flexor digitorum profundus muscles) are absent in many cases 1,2 and provocative clinical tests have a low diagnostic accuracy. 3 In addition, electrophysio- logical studies may be false negative or non-localiz- ing with sensitivities ranging from 37% to 86%. 4 Fur- thermore, the reliability of electrophysiological studies is undermined by a broad 95% confidence interval of the conduction velocity across the elbow owing to the accumulation of measurement errors. 5,6 Therefore, new laboratory tests are needed to improve the reliability of the diagnosis of UNE. 4 Ultrasonography of the ulnar nerve has been rec- ommended by several investigators as a good and reliable additional test in UNE. Using advanced ultrasonographic techniques, the ulnar nerve can be depicted with excellent resolution, and thickening may be revealed in patients with UNE. However, to our best knowledge, ultrasonography still has no de- finitive role in guidelines or practice parameters. As new and promising tests often become widely applied before they are critically appraised 7 we aimed to perform a more close and systematic review of the studies published so far. Ultrasonography in UNE requires detailed knowledge of the normal (sono-)anatomy, dynam- ics, and variations of the ulnar nerve and related structures. Therefore, we will first provide a synop- sis of the normal sonographic anatomy of the ul- nar nerve and then review ultrasonographic studies in the diagnosis of UNE and studies about underly- ing abnormalities in UNE.

BACKGROUND Clinical and experimental studies in patients with type 1 and type 2 diabetes have demonstrated changes in ion channel function and nerve structure. In this study, we investigated the relationship between axonal dysfunction and morphological change in diabetic polyneuropathy by using neuromuscular ultrasound and nerve excitability techniques. We also explored possible differences in this relationship between type 1 and type 2 diabetes. METHODS Nerve ultrasound and corresponding motor excitability studies were undertaken in 110 diabetes patients (50 type 1; 60 type 2) and 60 age-matched controls (30 for each group). Neuropathy severity was assessed by using total neuropathy score. Median and tibial nerve cross-sectional areas were measured at nonentrapment sites by using high-resolution linear probe. RESULTS Median and tibial nerve cross-sectional areas were significantly higher in diabetes patients compared with controls: type 1 (median = 7.6 ± 0.2 mm2 vs 6.3 ± 0.1 mm2 ; tibial = 14.5 ± 0.7 mm2 vs 10.8 ± 0.3 mm2 , P

Relapses, exacerbations or attacks of multiple sclerosis are the dominating feature of relapsing-remitting multiple sclerosis (MS), but are also observed in patients with secondary progressive MS. High-dose methylprednisolone is the routine therapy for relapses at present, but other treatments are also in current use. The objective of the task force was to review the literature on treatment of MS relapses to provide evidence-based treatment recommendations. Review was carried out on the literature with classification of evidence according to the EFNS guidelines for scientific task forces. Short-term, high-dose methylprednisolone treatment should be considered for the treatment of relapses of MS (level A recommendation). The optimal glucocorticoid treatment regimen, in terms of clinical efficacy and adverse events, remains to be established. A more intense, interdisciplinary rehabilitation programme should be considered as this probably further improves recovery after treatment with methylprednisolone (level B recommendation). Plasma exchange is probably efficacious in a subgroup of patients with severe relapses not responding to methylprednisolone therapy, and should be considered in this patient subgroup (level B recommendation). There is a need for further randomized, controlled trials in order to establish the optimal treatment regimen for relapses of MS.

The aim of the study was to assess the diagnostic value of short-segment nerve conduction studies (NCS) at 2-cm intervals from 4 cm above to 4 cm below the medial epicondyle in a large group of patients with ulnar neuropathy at the elbow (UNE). Furthermore, we wanted to compare electrodiagnostic and clinical findings. We evaluated 73 arms in 70 patients with UNE and observed the following abnormalities on short-segment NCS: focal conduction block (CB) in 1, focal CB with increased latency change in 34, and increased latency change alone in 25. Short-segment NCS had an additional localizing value in 28 arms of the 37 patients (76%) with motor conduction velocity (MCV) slowing across the elbow only or with nonlocalizing electrodiagnostic findings. The lesion was located above the elbow in 32 arms (53%), at the epicondyle in 16 arms (27%), and below the epicondyle in 12 (20%) of the 60 arms with focal CB or increased latency change on short-segment NCS. Patients with CB on routine and short-segment NCS had muscle weakness significantly more often than patients without CB. Thus, short-segment NCS are useful in localizing the lesion in patients with UNE and CB on routine NCS and have additional diagnostic value in patients with MCV slowing across the elbow or with nonlocalizing signs on routine nerve conduction studies. We recommend its use in all patients in whom UNE is suspected.

Introduction: The objective of this study is to obtain normative cross-sectional area (CSA) values for median nerve by ultrasound at predetermined sites and correlate them with electrophysiological variables in healthy Asian subjects. Methods: The median nerve was examined ultrasonographically in 100 healthy volunteers, mean age 39 years (range, 18–75 years). CSA of the median nerve was measured at wrist, mid-forearm, mid-arm, and axilla. All subjects underwent simultaneous standardized nerve conduction studies. Results: The mean median nerve CSAs ± SD at the distal wrist crease was 7.2 ± 1 mm2; mid-forearm 4.8 ± 0.9 mm2; mid-arm 6.1 ± 1 mm2; axilla 5.9 ± 0.9 mm2. The CSA at the wrist was the largest compared with other levels (P

Hereditary neuropathy with liability to pressure palsies (HNPP) is a disorder characterized by a tendency to develop focal neuropathies after trivial traumas. On teased nerve fiber studies, sausage-shaped myelin sheath swellings (tomacula) are found. We report the sonographic findings in a patient with genetically proven HNPP. We were able to demonstrate enlargement of several peripheral nerves, even nerves that were clinically unaffected. Enlargement was found not only at typical nerve entrapment sites but also outside these sites. This diffuse nerve enlargement may play an important role in the pathogenesis of entrapment neuropathies in HNPP patients.

The group of patients with Guillain-Barr'e syndrome (GBS) is very heterogenous with regard to antecedent infections, immunological parameters, clinical manifestations, and response to treatment. In this study, the presumed pathogenic factors anti-GM1 antibodies and Campylobacter jejuni infections were related to the clinical characteristics. Serum from 154 patients with GBS, 63 patients with other neurological diseases (OND), and 50 normal controls (NC) were tested for the presence of antibodies against GM1 and C. jejuni. Anti-GM1 antibodies were detected in 31 (20%) GBS patients, 5 (8%) OND patients, and in none of the NC. Evidence for a recent C. jejuni infection was found in 49 (32%) GBS patients and less often in OND patients (11%) or NC (8%). In GBS patients, the presence of anti-GM1 antibodies was significantly associated with C. jejuni infections. The subgroup of GBS patients with anti-GM1 antibodies suffered more often from a rapidly progressive and more severe neuropathy with predominantly distal distribution of weakness, without deficits of cranial nerves or sensory disturbances. The subgroup with C. jejuni infection also more often had a severe pure motor variant of GBS. Recovery of the patients with anti-GM1 antibodies and C. jejuni infections was not as good after plasma exchange compared with intravenous immunoglobulins.