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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

Background: We sought to determine the usefulness of sonography in the detection of nerve involvement in patients with vasculitic neuropathy. Methods: We enrolled 16 consecutive patients with vasculitic neuropathy (11 systemic vasculitis and 5 single organ peripheral nerve vasculitis), who met the diagnostic criteria of the Peripheral Nerve Society, and 16 disease controls with noninflammatory axonal polyneuropathy (10 cryptogenic, 4 metabolic, 2 hereditary). Patients underwent standardized nerve conduction studies and assessment of muscle strength (Medical Research Council scale), in addition to sonography of large arm and leg nerves, and brachial plexus. Nerves were evaluated bilaterally at predetermined sites for nerve size (cross-sectional area) and presence of hypervascularization. Results: We found enlarged nerves at common sites of nerve compression in all vasculitic and control patients. Multifocal enlargement in arm nerves, proximal to common sites of nerve compression, was sensitive (94%) and specific (88%) for vasculitic neuropathy. Sonography showed nerve enlargement in 51% of clinically or electrodiagnostically unaffected nerves. Sonography of the brachial plexus was normal. We found hypervascularization in 3 patients with systemic vasculitis. Conclusions: Sonographic enlargement of arm nerves proximal to sites of nerve compression with sparing of the brachial plexus may indicate a pattern characteristic of patients with vasculitic neuropathy. Sonography may represent a sensitive and specific technique for the detection of inflammatory neuropathy. Classification of evidence: This study provides Class III evidence that sonographic enlargement of arm nerves proximal to sites of nerve compression accurately identifies patients with vasculitic neuropathy.

Objective To investigate development of sonographic abnormalities and applications of high-resolution ultrasonography (HRUS) in neurofibromatosis type 1 (NF1). Methods Sixteen asymptomatic or minimally symptomatic NF1 patients underwent HRUS at inclusion and 1 year follow-up. Upper and lower extremity nerves were investigated. Peripheral nerve involvement was graded. Results Plexiform neurofibromas (PNFs) were found in 7 patients (43.8%) at inclusion and 10 (62.5%) at follow-up. All initially identified PNFs were also found at follow-up; additional PNFs were found by extended longitudinal assessment at follow-up. All 3 patients with minor and 7 patients with severe peripheral nerve involvement had similar involvement at follow-up. Mean nerve size change was −0.2 mm 2 (±1.6) and 0.3 mm 2 (±6.2) in patients with minor and severe involvement. Mean PNF size change was −0.1 mm 2 (±9.9). Conclusions HRUS allows qualitative assessment of peripheral nerves, which makes it advantageous as initial imaging technique in suspected neuropathy. Patients with minimal nerve involvement remained so, and might therefore require less follow-up for malignant peripheral nerve sheath tumor (MPNSTs) development. Measured change in PNF size was highly variable. Repeating an extensive standardized HRUS protocol during follow-up thus seems less useful to screen for MPNSTs. Significance HRUS has potential applications as diagnostic and screening tool in NF1.

Item does not contain fulltext Over the last two decades, dozens of applications have emerged for ultrasonography in neuromuscular disorders. We wanted to measure its impact on practice in laboratories where the technique is in frequent use. After identifying experts in neuromuscular ultrasound and electrodiagnosis, we assessed their use of ultrasonography for different indications and their expectations for its future evolution. We then identified the earliest papers to provide convincing evidence of the utility of ultrasound for particular indications and analyzed the relationship of their date of publication with expert usage. We found that experts use ultrasonography often for inflammatory, hereditary, traumatic, compressive and neoplastic neuropathies, and somewhat less often for neuronopathies and myopathies. Usage significantly correlated with the timing of key publications in the field. We review these findings and the extensive evidence supporting the value of neuromuscular ultrasound. Advancement of the field of clinical neurophysiology depends on widespread translation of these findings.

Objective Wartenberg’s migrant sensory neuritis (WMSN) is a rare, patchy, pure sensory neuropathy of unknown etiology. High-resolution ultrasonography (HRUS) is an emerging diagnostic technique for neuropathies, but it has not been applied in WMSN. In this study we aimed to determine HRUS abnormalities in WMSN. Methods We performed a case-control study of 8 newly diagnosed patients with WMSN and 22 treatment-naive disease controls (16 patients with pure sensory axonal neuropathy and 6 with pure sensory chronic inflammatory demyelinating polyneuropathy (CIDP) or Lewis-Sumner syndrome (LSS)). All patients underwent routine diagnostic evaluations and a predefined HRUS protocol. Results We found multifocal nerve enlargement in all 8 WMSN patients. The median nerve in the upper arm and the sural nerve were significantly larger in WMSN than in axonal controls (p = 0.01 and p = 0.04). In CIDP/LSS, sonographic enlargement was more extensive. Furthermore we found brachial plexus involvement in 3 of 8 (38%) WMSN patients. Conclusion HRUS showed enlargement of multiple nerves in all WMSN patients even if clinical testing and NCS were normal. Significance The feature of multifocal nerve enlargement may be of additional value in establishing the diagnosis of WMSN and may support the suggestion of an auto-immune etiology.

Objective To investigate the prevalence, severity and relation to fluid retention of self-reported pregnancy-related carpal tunnel syndrome (CTS) symptoms in a large sample of pregnant women. Design A prospective longitudinal cohort study. Setting Dutch women who became pregnant between January 2013 and January 2014 in the southeast of The Netherlands. Population or sample A total of 639 Dutch pregnant women. Methods Baseline characteristics were assessed at 12 weeks’ gestation. CTS symptoms were assessed using the Boston Carpal Tunnel Questionnaire (BCTQ) at 32 weeks and during the first postpartum week regarding the last weeks of pregnancy. Fluid retention, sleeping problems and depressive symptoms (using the Edinburgh Depression Scale) were assessed at several time points during pregnancy. Main outcome measures BCTQ scores, fluid retention and sleeping problems. Results Of the 639 women, 219 (34%) reported CTS symptoms during pregnancy. Total mean scores on the BCTQ were significantly higher after 32 weeks’ than up to 32 weeks’ gestation. Most women experienced mild to moderate symptoms. Pregnant women with CTS symptoms reported significantly higher levels of fluid retention during gestation compared with pregnant women without CTS symptoms [F = 60.6, df (1598), P

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.

Carpal tunnel syndrome (CTS) is a common, compressive nerve-entrapment disorder with symptoms of numbness, paresthesia, and pain. Carpal tunnel release surgery is the only known long-term effective treatment. However, surgery is invasive and up to 30% of patients report recurrence or persistence of symptoms or suffer from post-surgical complications. A promising non-surgical treatment for CTS is mechanical wrist traction. The purpose of this study was to evaluate clinical outcomes following mechanical traction in patients with CTS compared to care as usual. Adult patients (N = 181, mean age 58.1 (13.0) years, 67% women) with electrodiagnostically confirmed CTS were recruited from an outpatient neurology clinic in the Netherlands between October 2013 and April 2015. After baseline assessments, patients were randomized to either the intervention group (12 treatments with mechanical traction, twice a week for a period of 6 weeks) or “care as usual”. The main clinical outcome measure was surgery during 6 months’ follow-up. In addition, symptom severity was measured using the Boston Carpal Tunnel Questionnaire (BCTQ) at baseline, 3, and 6 months’ follow-up. Baseline characteristics and severity of CTS symptoms at follow-up were compared between the intervention and care-as-usual groups using a t test and χ 2 tests. Time to event (surgery) between the groups was analyzed using Kaplan-Meier survival analysis and Cox proportional hazards analysis. The intervention group had fewer surgeries (28%) compared to the care-as-usual group (43%) during follow-up (χ2 1 = 4.40, p = .036). Analyses of the survival curves revealed a statistically significant difference between the groups over time (log-rank test χ 2 1 = 6.94, p = .008). At 6 months’ follow-up, symptom severity and functional status scores had significantly decreased from baseline in both groups (p

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.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).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.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.

Objective:To determine ultrasonographic peripheral nerve involvement in patients with asymptomatic neurofibromatosis type 1 (NF1).Methods:Thirteen asymptomatic and 4 minimally symptomatic patients with NF1 were included in this cross-sectional pilot study to detect asymptomatic abnormalities of the brachial plexus and upper and lower extremity nerves. Patients underwent clinical examination, nerve conduction studies (NCS), and high-resolution ultrasonography (HRUS).Results:HRUS showed abnormalities in 16 patients (94.1%). Neurofibromas were identified in 10 patients (58.8%): localized neurofibromas were found in 3 patients (17.6%), plexiform neurofibromas in 3 (17.6%), and both in 4 (23.5%). In 6 patients (35.3%), only nerve enlargement without an abnormal fascicular pattern was observed. Severe involvement of the peripheral nervous system with multiple plexiform neurofibromas was observed in 7 patients (41.2%), while 4 patients (23.5%) had no or only minor involvement. Both NCS and HRUS were performed on 73 individual nerve segments. In 5.5%, abnormalities were found with both tests; in 50.7%, only with HRUS; and in 1.4%, only with NCS.Conclusions:HRUS frequently showed subclinical involvement of the peripheral nerves in NF1, also when NCS were normal. HRUS findings ranged from normal to widespread peripheral nerve involvement. Because the presence of plexiform neurofibromas and the benign tumor load are risk factors for the development of a malignant peripheral nerve sheath tumor, HRUS may be a useful tool to identify a subgroup of patients who could benefit from regular follow-up.

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 We aimed to determine whether nerve ultrasound can improve detection of potentially treatable chronic inflammatory neuropathies in patients that have normal nerve conduction study (NCS). Diagnostic consensus criteria for inflammatory neuropathies primarily rely on NCS results that indicate multifocal demyelination. However, additional supportive criteria are developed to identify these treatable inflammatory neuropathies. In this study we examined the additional value of sonography in the detection of treatable inflammatory neuropathies. Methods We analysed 240 incident patients with clinical suspicion of chronic inflammatory neuropathy; work-up consisted of routine ancillary investigations recommended in the diagnostic consensus guidelines and a standardized sonographic protocol assessing arm nerves and brachial plexus. All patients without any demyelinating features on NCS, but with sonographic abnormalities fitting inflammatory neuropathy and a clinical phenotype totally compatible with chronic inflammatory demyelinating polyneuropathy (CIDP), Lewis Sumner Syndrome (LSS), or multifocal motor neuropathy (MMN), received standard treatment with IVIg. Treatment effect was evaluated by routine clinical examination and hand grip or myometry. Results We found 17 patients (7%) with a clinical phenotype compatible with CIDP (n = 10), LSS (n = 1) and MMN (n = 6, with 3 positive for anti-GM1 auto-antibodies), sonographic nerve enlargement of proximal median nerve segments and/or cervical (nerve) roots, yet negative NCS (2 only axonal loss, 15 normal). An abnormal MRI of brachial plexus was found in 9/17 patients (53%) and an elevated protein content of cerebrospinal fluid in 9/17 patients (53%). All 17 patients showed objective improvement on IVIG treatment. Conclusion Our study suggests that nerve ultrasound may help to improve detection of treatment-responsive chronic neuropathies, even in patients not fulfilling the diagnostic NCS and other supportive criteria, including MRI of the brachial plexus. Therefore, future revisions of the diagnostic consensus criteria may include sonography as complementary technique to NCS and MRI.

IntroductionAnimal and human studies of diabetes have shown significant alterations in nerve blood flow (NBF), which may also play a role in the development of neuropathy. However, the non-invasive assessment of NBF in human subjects has remained elusive until the recent technological advancements in ultrasound (US) technology. We undertook sonographic assessment of NBF in 75 patients with type 2 diabetes and correlated the findings with neuropathy severity scores and electrophysiological parameters.MethodsBlinded median and tibial nerve ultrasound scans were performed at non-entrapment sites using a high-resolution linear probe. NBF was quantified using power Doppler techniques to obtain the vessel score (VSc) and maximum perfusion intensity (MPI). Routine nerve conduction studies were performed, and neuropathy severity was assessed using the total neuropathy score (TNS). Aged- and gender-matched controls were enrolled.ResultsDiabetic nerves had higher rates of NBF detection (28%) compared to the control group (p2 vs. 7.2 ± 0.1 mm2, p2 vs. 12. 8 ±0.5 mm2, pConclusionsPeripheral nerve hypervascularity is detectable by US in moderate to severe diabetic neuropathy with prominent sensory dysfunction. Consistent with previous studies, the sonographic detection of NBF is a pathological finding.

Objective Detection of nerve enlargement in polyneuropathies by sonography is a new research area. No systematic investigation has been done yet in chronic inflammatory demyelinating polyneuropathy (CIDP). Therefore we investigated this in CIDP. Methods Eleven patients with CIDP fulfilling the international criteria on CIDP underwent ultrasonographic examination of the median, ulnar, fibular and posterior tibial nerves and sometimes the brachial plexus bilaterally, using a standardized protocol. We assessed presence of nerve thickening and increased nerve vascularization. Results In 7 of the 11 patients multiple nerve enlargements were detected: ulnar nerve 7, fibular nerve 5, posterior tibial nerve 4 and median nerve in 4 patients. The number of enlarged nerves was related with the MRC sum-score (p = 0.03) and the total protein in the cerebrospinal fluid (CSF) at diagnosis (p = 0.02). Increased vascularization was seen in 6 of the 11 patients: 4 in one nerve and in 2 in multiple nerves. The number of nerves with increased vascularization was associated with the number of enlarged nerves (p = 0.01) and total protein in the CSF (p = 0.006). Conclusion Multiple nerve enlargements occur in CIDP showing a relation with a lower MRC sum-score, increased nerve vascularization and a higher total protein of the CSF. Significance Our findings of nerve enlargement and increased nerve vascularization may be tools to monitor disease activity in CIDP, but further studies are needed.

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 In this study the authors explored how people with recently diagnosed multiple sclerosis (MS) experience their disease within their family lives. Ten people in various stages of the cycle of family life (leaving home, finding a partner, raising children, parenting adolescents, launching children) who had been diagnosed with MS were interviewed in half-structured conversational interviews. METHOD Transcriptions were analyzed following a phenomenological approach. RESULTS Five themes were found: (a) dwindling capacity for housekeeping and childcare (b) struggling to ask for or to accept help, (c) countering awkward attitudes toward my illness, (d) suspecting family members of concealing their, and (e) watching family members wrestle with your illness. DISCUSSION The participants described that their illness affected their ability to care for their family and home as they used to. Only a couple of studies have addressed the first person perspective of patients on family and MS. The study expands on these studies by exploring not previously examined perspectives on leaving home, finding a partner, parenting adolescents, and launching children. The findings on family and MS, approached as elements of the first person perspective of MS patients, may guide future research. Given the pivotal role of worries on family in patient experience of MS, we argue that acknowledgment of family as a constitutive element of the patient perspective should be integrated in regular MS care. The authors suggest that the clinical handling of MS as a family issue needs to be done thoughtfully and with attention to the specifics of each unique family situation. (PsycINFO Database Record

Objective We explored the nerve ultrasound (US) characteristics of 15 patients with end-stage kidney disease (ESKD) and correlated these findings with clinical severity and electrophysiological parameters of neuropathy. Methods 15 ESKD patients on thrice-weekly high-flux haemodialysis and 15 healthy controls were enrolled. Sonographic and electrophysiologic studies were conducted before and after a single session of haemodialysis. Serial measurements of median nerve cross-sectional area (CSA) and hypoechoic fraction (HF) were performed at the same non-entrapment site in the mid-forearm. Neuropathy severity was quantified using the total neuropathy score (TNS). Results 86.7% of the ESKD cohort had neuropathy (TNS>1). ESKD patients had significantly higher baseline CSA (8.9±1.2mm 2 vs 7.5±1.0mm 2 , p p p 2 ; p p + after dialysis ( r =0.782, p Conclusions This study shows that peripheral nerves in ESKD patients are larger and more hypoechoic and that these morphological abnormalities may be reversed by dialysis. Significance US may be useful as an early marker of neuropathy in ESKD.

A 63-year-old man reported numbness in his left first, second and third digits for 2 years. Initially, he found that ‘shaking out’ his left hand helped, but this benefit did not last. He underwent decompression of left-sided carpal tunnel syndrome 2 years earlier and his symptoms worsened after surgery, with more sensory loss and pain. He worked as a carpenter, and had injured his left thumb with nails on multiple occasions without feeling pain. On examination, he had an enlarged left thumb (figure 1), though he appeared unaware of this; his other digits were normal in size. The median nerve was palpable at the wrist, but Tinel's test was negative. There was a mild weakness of abductor pollicis brevis and opponens brevis, with reduced sensation over the fingertips …

Objective:To determine the diagnostic value of high-resolution ultrasound (HRUS) for detection of chronic inflammatory demyelinating polyneuropathy (CIDP), Lewis-Sumner syndrome (LSS), and multifocal motor neuropathy (MMN).Methods:Between January 2013 and January 2015, we enrolled 75 consecutive treatment-naive patients with chronic inflammatory neuropathies and 70 disease controls. We performed extensive nerve conduction and standardized HRUS studies bilaterally of large arm and leg nerves and brachial plexus. We determined optimal sonographic cutoff values of nerve size and used receiver operating characteristic analysis and logistic regression models to identify nerve combinations with optimal diagnostic performance.Results:Enlargement of median nerve at forearm >10 mm2, upper arm >13 mm2, and any trunk of brachial plexus >8 mm2 was 99% specific for chronic inflammatory neuropathies. A shortened HRUS protocol for detecting this abnormal nerve enlargement showed high sensitivity (83%–95%), positive predictive value (100%), and negative predictive value (98%) in discriminating CIDP, LSS, and MMN from clinical mimics.Conclusions:Sonographic enlargement of proximal median nerve segments in the arms and brachial plexus is a key feature of chronic inflammatory neuropathies, which helps to reliably distinguish them from axonal neuropathies and amyotrophic lateral sclerosis.Classification of evidence:This study provides Class II evidence that, in absence of clinical features that suggest a hereditary demyelinating neuropathy, sonographic enlargement of proximal median nerve segments and brachial plexus accurately identifies patients with chronic inflammatory neuropathies.

In this study we aimed to identify prognostic factors of ulnar neuropathy at the elbow (UNE) and developed a scoring system to establish the prognosis.We collected baseline clinical, electrophysiologic, and ultrasonographic data from 2 cohorts. The outcomes 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.Of the 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. A compound muscle action potential amplitude reduction across the elbow of ≥16% (particularly if ≥ 50%) was associated with a more favorable outcome.Outcome in UNE may be predicted by scoring 4 parameters. Muscle Nerve 55: 698-705, 2017.

Peter Joseph Jongen,1,2 Ludovicus G Sinnige,3 Björn M van Geel,4 Freek Verheul,5 Wim I Verhagen,6 Ruud A van der Kruijk,7 Reinoud Haverkamp,8 Hans M Schrijver,9 Jacoba C Baart,10 Leo H Visser,11 Edo P Arnoldus,12 Herman Jacobus Gilhuis,13 Paul Pop,14 Monique Booy,15 Marco Heerings,16 AntonKool,17 Esther van Noort17 1Department of Community and Occupational Medicine, University Medical Center Groningen, University of Groningen, Groningen, 2MS4 Research Institute, Nijmegen, 3Multiple Sclerosis Centre Leeuwarden, Medical Centre Leeuwarden, Leeuwarden, 4Department of Neurology, Medical Centre Alkmaar, Alkmaar, 5Department of Neurology, Groene Hart Hospital, Gouda, 6Department of Neurology, Canisius Wilhelmina Hospital, Nijmegen, 7Department of Neurology, Slingeland Hospital, Doetinchem, 8Department of Neurology, Zuwe Hofpoort Hospital, Woerden, 9Multiple Sclerosis Centre, Westfries Gasthuis, Hoorn, 10Department of Neurology, Ziekenhuisgroep Twente, Almelo-Hengelo, 11Multiple Sclerosis Centre Midden Brabant, St Elisabeth Hospital, Tilburg, 12Multiple Sclerosis Centre Midden Brabant, Tweesteden Hospital, 13Department of Neurology, Reinier de Graaf Gasthuis, Delft, 14Department of Neurology, Viecuri Medical Centre, Venlo-Venray, 15Multiple Sclerosis Centre, Amphia Hospital, Breda, 16MH Advies en organisatiebureau, Assen, 17Curavista bv, Geertruidenberg, the Netherlands Background: MSmonitor is an interactive web-based program for self-management and integrated, multidisciplinary care in multiple sclerosis.Methods: To assess the utilization and valuation by persons with multiple sclerosis, we held an online survey among those who had used the program for at least 1 year. We evaluated the utilization and meaningfulness of the program’s elements, perceived use of data by neurologists and nurses, and appreciation of care, self-management, and satisfaction.Results: Fifty-five persons completed the questionnaire (estimated response rate 40%). The Multiple Sclerosis Impact Profile (MSIP), Medication and Adherence Inventory, Activities Diary, and electronic consultation (e-consult) were used by 40%, 55%, 47%, and 44% of respondents and were considered meaningful by 83%, 81%, 54%, and 88%, respectively. During out-patient consultations, nurses reportedly used the MSmonitor data three to six times more frequently than neurologists. As to nursing care, more symptoms were dealt with (according to 54% of respondents), symptoms were better discussed (69%), and the overall quality of care had improved (60%) since the use of the program. As to neurological care, these figures were 24%, 31%, and 27%, respectively. In 46% of the respondents, the insight into their symptoms and disabilities had increased since the use of the program; the MSIP, Activities Diary, and e-consult had contributed most to this improvement. The overall satisfaction with the program was 3.5 out of 5, and 73% of the respondents would recommend the program to other persons with multiple sclerosis.Conclusion: A survey among persons with multiple sclerosis using the MSmonitor program showed that the MSIP, Medication and Adherence Inventory, Activities Diary, and e-consult were frequently used and that the MSIP, Medication and Adherence Inventory, and e-consult were appreciated the most. Moreover, the quality of nursing care, but not so neurological care, had improved, which may relate to nurses making more frequent use of the MSmonitor data than neurologists. Keywords: patient-reported outcome, impact, adherence, diary, inventory, e-consult

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

OBJECTIVE To assess the relationship between the cross-sectional area (CSA) of the ulnar nerve by ultrasound (US) with clinical and electrophysiologic findings in Hansen ulnar neuropathy. METHODS Twenty-one patients (42 arms) with Hansen disease (mean age 30.0 ± 12.97, range 13-61 years, borderline tuberculoid 29%, borderline lepromatous, 19% lepromatous leprosy 42%, and pure neuritic type 10%) were examined clinically for ulnar sensory and motor weakness. The ulnar nerve was ultrasonographically examined from the wrist to the axilla, and CSA was measured at the level of maximum enlargement. Ulnar sensory nerve conduction was recorded orthodromically with ring electrodes placed at the fifth digit and amplitude of sensory nerve action potential (SNAP) recorded 3 cm proximal to the distal wrist crease. Motor conduction velocity (MCV) was recorded at the wrist-below the elbow, below the elbow-above the elbow, and above the elbow-axilla segments. RESULTS Out of the 42 arms with Hansen disease, 76% had clinically motor weakness, and 43% had sensory loss in the upper limbs innervated by the ulnar nerve. As compared with healthy subjects, the patients with Hansen ulnar neuropathy had a statistically significant reduction in SNAP (P ≤ 0.0001) and MCV (P ≤ 0.0001). It was observed that the maximum enlargement of the ulnar nerve in all the patients was a few centimeters above the elbow segment. The mean CSA of ulnar nerve above the medial epicondyle was 18 ± 15 mm as compared with controls 4.83 ± 1.12 mm (P < 0.0001). In addition to nerve thickening, US depicted abnormality in morphology. In 55%, the nerve was hypoechoic, and in 7.1%, the nerve pattern was oligofascicular. Color Doppler (CD) flow signals were observed in all the nerves with loss of fascicular pattern and in 40% of the nerves that were hypoechoic. A statistically significant correlation was found between CSA of ulnar nerve above the medial epicondyle vs. MCV at BE-AE and compound muscle action potentials (CMAP) above the elbow in the patients with clinical motor weakness (r = -0.55, P < 0.001) and (r = -0.57, P < 0.001), respectively. There was no statistical significant correlation between CSA and SNAP in the patients with (r = -0.52, P = 0.23) and without (r = -0.07, P = 0.83) sensory loss. CONCLUSIONS In leprosy patients, a positive correlation exits between the presence of motor weaknesses of the ulnar nerve innervated muscles, sonographically thickening of the ulnar nerve, and motor conduction slowing of the ulnar nerve at the BE-AE segment. In addition, US provided information on nerve morphologic alterations regarding the echo texture and location of nerve enlargement.

A new venous disorder, chronic cerebrospinal venous insufficiency (CCSVI), has been proposed in patients with multiple sclerosis (MS). It is a vascular condition characterized by an impaired cerebrospinal venous drainage due to obstructions in the main extracranial cerebrovenous outflow routes (i.e. internal jugular veins [IJV] and/or azygos veins). In this review, the studies which assessed the prevalence of CCSVI in MS by echo colour Doppler (ECD) will be discussed. The technical aspects of determination of the five CCSVI criteria: (1) reflux in the IJV and/or vertebral veins in supine and upright position, (2) reflux in the deep cerebral veins, (3) high-resolution B-mode proximal IJV stenosis, (4) flow not Doppler detectable in IJVs and/or vertebral veins (VVs) and (5) reverted postural control of the main cerebrovenous outflow pathway are described in detail. We conclude that so far there are many studies with contradictory results, and as yet a strong scientific base to support the evidence for a causative relationship of CCSVI and MS is lacking. Recent studies call into question the validity of using ECD as a proper and reliable test for the diagnosis of CCSVI. One explanation for the variety in interpretation of the individual CCSVI criteria, with the wide-ranging percentages CCSVI, could be the different methods by using ECD to determine various criteria.

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

Introduction Nerve ultrasound is a relatively new diagnostic tool in peripheral neuropathies. Some studies investigated inter-observer variability of this technique, but had several limitations such as only including healthy controls, studying few sites or still images, or being single center. This multicenter study systematically investigated inter-observer variability of ultrasound at multiple sites in healthy and diseased nerves to gain more insight on its applicability in daily clinical practice. Methods We enrolled 20 chronic inflammatory neuropathy patients and 10 healthy controls in 3 different centers. All participants underwent an extensive nerve ultrasound protocol, including cross-sectional area measurements of median, ulnar, fibular, tibial, and sural nerves, and brachial plexus. Ultrasound was performed blinded by a local investigator and an investigator visiting all centers. In 5 patients ultrasound was performed on two different types of sonographic device. All investigators had at least one year of experience with nerve ultrasound. Results Overall, mean difference between investigators was −0.11 mm2 (95%-CI −0.23 to 0.00 mm2). The investigated nerve site had significant influence on the mean difference in a multilevel mixed model, but only at the ulnar sulcus there was significant deviation from 0 mm2 (−0.46 mm2). No significant effect of different hospitals or devices was found. Intraclass correlation coefficients (ICCs) were 0.48–0.96 in arm nerves compared to 0.46–0.61 in large leg nerves. Standard deviation (SD) for difference between investigators was 1.0–1.7 mm2 in arm nerve sites compared to 1.5–3.1 mm2 in brachial plexus, posterior tibial, and fibular nerve sites. Conclusion Sonographic measurement of nerve size was reliable with a low mean difference between investigators and high ICCs, especially in arm nerves. Still, solitary abnormal measurements at sites with a high variation in difference (high SD) should be interpreted with care. Different devices and a multicenter setting had no significant influence on the mean difference. This indicates that nerve ultrasound studies can reliably obtain nerve size data that may be pooled and generalized to daily clinical practice.

Carpal tunnel syndrome (CTS) is a common entrapment neuropathy. Electrodiagnostic testing (EMG) is used to confirm the diagnosis. It is not known what the diagnostic accuracy of high-resolution sonography is in comparison to EMG.The aim of this study was to compare the diagnostic accuracy of both tests in CTS patients.A prospective cohort of 207 patients with possible CTS underwent high-resolution sonography and EMG. The diagnosis of CTS was based on clinical signs and symptoms. The cross-sectional area of the median nerve at the carpal tunnel inlet and at the distal one-third level of the forearm was assessed by an investigator, blinded to the clinical and EMG data. Normal sonographic values were obtained from 137 controls. All patients and 40 controls underwent a standardised nerve conduction study. The kappa coefficient was used to evaluate the relationship between sonography, EMG and clinical diagnosis.The cross-sectional area at the distal one-third of the forearm was not significantly different between the controls and patients (p = 0.59), whereas the cross-sectional area at the carpal tunnel inlet was significantly increased in the patient group (p0.0001). The kappa coefficient for EMG using the median-ulnar distal sensory latency difference versus clinical evaluation was 0.64 and, for sonography, this coefficient was 0.69; these were not statistically different (p = 0.37). Combining the two tests resulted in a kappa coefficient of 0.72, which was not significantly different from sonography alone (p = 0.73).In patients with a clinical diagnosis of CTS, the accuracy of sonography is similar to that for EMG. Sonography is probably preferable because it is painless, easily accessible and preferred by the patients.

Based on approximately eight years of data collection with the nationwide Computer Registry of All Myopathies and Polyneuropathies (CRAMP) in the Netherlands, recent epidemiologic information for thirty neuromuscular disorders is presented. This overview includes age and gender data for a number of neuromuscular disorders that are either relatively frequently seen in the neuromuscular clinic, or have a particular phenotype. Since 2004, over 20,000 individuals with a neuromuscular disorder were registered in CRAMP; 56% men and 44% women. The number per diagnosis varied from nine persons with Emery Dreifuss muscular dystrophy to 2057 persons with amyotrophic lateral sclerosis. Proportions of men ranged from 38% with post-polio syndrome to 68% with progressive spinal muscular atrophy, excluding X-chromosome linked disorders. Inclusion body myositis showed the highest median age at diagnosis of 70 years. These data may be helpful in the diagnostic process in clinical practice and trial readiness. (C) 2016 Elsevier B.V. All rights reserved.

We quantified intraneural blood flow (INBF) using perfusion measurement software (PixelFlux), and compared it with the qualitative method of counting blood vessels (vessel score) in a cohort of carpal tunnel syndrome (CTS) patients. METHODS Forty-seven patients (67 wrists) with a clinical and electrophysiological diagnosis of CTS, and 20 healthy controls (40 wrists) were enrolled. Median nerve ultrasound (US) was performed at the carpal tunnel inlet to measure the cross-sectional area (CSA) and vessel score. Power Doppler sonograms from nerves with detectable INBF were processed with PixelFlux to obtain the maximum perfusion intensity (MPI). RESULTS Forty-nine percent of CTS patients had detectable INBF compared with none in the control group (P

Background Job loss is common in multiple sclerosis (MS) and is known to exert a negative effect on quality of life. The process leading up to job loss typically includes negative work events, productivity losses and a need for accommodations. By using active coping strategies job loss may be prevented or delayed. Objective Our goal was to examine negative work events and accommodations in relation to coping strategies in employed relapsing–remitting MS patients. Methods Ninety-seven MS patients (77% females; 21–59 years old) completed questionnaires concerning the patient’s work situation, coping strategies, demographics, physical, psychological and cognitive functioning. Forward binary logistic regression analyses were conducted to examine coping strategies and other (disease) characteristics predictive of reported negative work events and accommodations. Results Nineteen per cent of the employed MS patients reported one or more negative work events, associated with a higher use of emotion-oriented coping and more absenteeism. Seventy-three per cent reported using one or more work accommodations, associated with a higher educational level and more presenteeism. MS patients reporting physical changes to the workplace employed more emotion-oriented coping, while flexible scheduling was associated with task-oriented coping. Conclusion Emotion-oriented and task-oriented coping strategies are associated with negative work events and the use of accommodations.

Contains fulltext : 172833.pdf (Publisher’s version ) (Open Access) BACKGROUND: In persons with multiple sclerosis (MS) self-efficacy positively affects health-related quality of life (HRQoL) and physical activity. In a previous study we observed that 6 months after an intensive 3-day social cognitive treatment (Can Do treatment) with the participation of support partners, self-efficacy and HRQoL had improved in persons with relapsing remitting MS (RRMS). Given the chronic nature of the disease, it is important to know whether these beneficial changes may last. METHODS: Can Do treatment was given to 60 persons with MS and their support partners. At baseline and 12 months after treatment self-efficacy control, self-efficacy function, physical and mental HRQoL, anxiety, depression and fatigue were assessed via self-report questionnaires. Differences were tested via a paired t test. RESULTS: Of the 57 persons with MS that completed the baseline assessment and the 3-day treatment, 38 filled in the 12th month questionnaires (response rate 66.7 %), 22 with RRMS and 14 with progressive MS. In the RR group self-efficacy control had increased by 20.2 % and physical HRQoL by 15.0 %, and depression and anxiety had decreased by 29.8 and 25.9 %, respectively (all P < 0.05); the changes in mental HRQoL (+17 %) and fatigue (-20 %) failed to be statistically significant (P = 0.087, P = 0.080, respectively). In the progressive group no changes suggestive of improvement were seen. CONCLUSIONS: The findings suggest that a 3-day intensive social cognitive treatment (Can Do treatment) with the participation of support partners may have long lasting beneficial effects on the self-efficacy and HRQoL in persons with RRMS; and that improvements in anxiety and depression, not seen in the 6-month study, may yet develop at 12 months.

Contains fulltext : 51646.pdf (Publisher’s version ) (Closed access) Each of the various neuromuscular diseases is rare. Consequently, solid epidemiological data are not available and it is often difficult to find sufficient patients for studies. For this reason, the Dutch neuromuscular database, CRAMP (Computer Registry of All Myopathies and Polyneuropathies), was developed in 2004 by the Dutch Neuromuscular Research Support Centre, to store information on patient characteristics and diagnoses (based on Rowland and McLeod's classification) in a uniform and easily retrievable manner. Care was taken to preserve data confidentiality. It is envisaged that CRAMP will prove particularly useful for studies in which multicentre collaboration is needed to recruit a sufficiently large number of patients. More than 10,000 patients with neuromuscular diseases (4,837 female, 5,476 male) have been registered since 2004, half of whom (n=5059) have peripheral nerve disorders.

BackgroundMultiple Sclerosis (MS) is the most common cause of neurological disability in young and middle-aged adults. At this stage in life most people are in the midst of their working career. The majority of MS patients are unable to retain employment within 10 years from disease onset. Leading up to unemployment, many may experience a reduction in hours or work responsibilities and increased time missed from work. The MS@Work study examines various factors that may influence work participation in relapsing-remitting MS patients, including disease-related factors, the working environment and personal factors.Methods/designThe MS@Work study is a multicenter, 3-year prospective observational study on work participation in patients with relapsing-remitting MS. We aim to include 350 patients through 15–18 MS outpatient clinics in the Netherlands. Eligible participants are 18 years and older, and either currently employed or within three years since their last employment. At baseline and after 1, 2 and 3 years, the participants are asked to complete online questionnaires (including questions on work participation, work problems and accommodations, cognitive and physical ability, anxiety, depression, psychosocial stress, quality of life, fatigue, empathy, personality traits and coping strategies) and undergo cognitive and neurological examinations. After six months, patients are requested to only complete online questionnaires. Patient perspectives on maintaining and improving work participation and reasons to stop working are gathered through semi-structured interviews in a sub-group of patients.DiscussionProspective studies with long-term follow-up on work participation in MS are rare, or take into account a limited number of factors. The MS@Work study provides a 3-year follow-up on various factors that may influence work participation in patients with relapsing-remitting MS. We aim to identify factors that relate to job loss and to provide information about preventative measures for physicians, psychologists and other professionals working in the field of occupational health.Keywords: Multiple Sclerosis, Work participation, Absenteeism, Cognitive abilities, Psychological factors, Fatigue, Physical disability

In persons with multiple sclerosis (MS) a lowered self-efficacy negatively affects physical activities. Against this background we studied the relationship between self-efficacy and cognitive performance in the early stages of MS. Thirty-three patients with Clinically Isolated Syndrome (CIS) and early Relapsing Remitting MS (eRRMS) were assessed for self-efficacy (MSSES-18), cognition (CDR System), fatigue (MFIS-5), depressive symptoms (BDI), disease impact (MSIS-29), and disability (EDSS). Correlative analyses were performed between self-efficacy and cognitive scores, and stepwise regression analyses identified predictors of cognition and self-efficacy. Good correlations existed between total self-efficacy and Power of Attention (r= 0.65;P< 0.001), Reaction Time Variability (r= 0.57;P< 0.001), and Speed of Memory (r= 0.53;P< 0.01), and between control self-efficacy and Reaction Time Variability (r= 0.55;P< 0.01). Total self-efficacy predicted 40% of Power of Attention, 34% of Reaction Time Variability, and 40% of Speed of Memory variabilities. Disease impact predicted 65% of total self-efficacy and 58% of control self-efficacy variabilities. The findings may suggest that in persons with CIS and eRRMS self-efficacy may positively affect cognitive performance and that prevention of disease activity may preserve self-efficacy.

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

Summary Background Despite available treatment with intravenous immunoglobulin (IVIg), morbidity and mortality are considerable in patients with Guillain-Barre syndrome (GBS). Our aim was to assess whether methylprednisolone, when taken with IVIg, improves outcome when compared with IVIg alone. Methods We did a double-blind, placebo-controlled, multicentre, randomised study, to which we enrolled patients who were unable to walk independently and who had been treated within 14 days after onset of weakness with IVIg (0·4 g/kg bodyweight per day) for 5 days. We assigned 233 individuals to receive either intravenous methylprednisolone (500 mg per day; n=116) or placebo (n=117) for 5 days within 48 h of administration of first dose of IVIg. Because age is an important prognostic factor, we split treatment groups into two age-groups—ie, younger than age 50 years, or 50 years and older. Our primary outcome was an improvement from baseline in GBS disability score of one or more grades 4 weeks after randomisation. Analysis was by intention to treat. Findings We analysed 225 patients. GBS disability scores increased by one grade or more in 68% (76 of 112) of patients in the methylprednisolone group and in 56% (63 of 113) of controls (odds ratio [OR] 1·68, 95% CI 0·97–2·88; p=0·06). After adjustment for age and degree of disability at entry, treatment OR was 1·89 (95% CI 1·07–3·35; p=0·03). Side-effects did not differ greatly between groups. Interpretation We noted no significant difference between treatment with methylprednisolone and IVIg and IVIg alone. Because of the relevance of prognostic factors and the limited side-effects of methylprednisolone, the potential importance of combination treatment with the drug and IVIg, however, warrants further investigation.