The 2022 European postgraduate (residency) programme in neurology in a historical and international perspective.

BACKGROUND AND PURPOSE: Neurology residency programmes, which were first established at the beginning of the 20th century, have become mandatory all over Europe in the last 40-50 years. The first European Training Requirements in Neurology (ETRN) were published in 2005 and first updated in 2016. This paper reports the most recent revisions of the ETRN. METHODS: Members of the EAN board performed an in depth revision of the ETNR 2016-version, which was reviewed by members of the European Board and Section of Neurology of the UEMS, the Education and Scientific Panels, the Resident and Research Fellow Section and the Board of the EAN, as well as the presidents of the 47 European National Societies. RESULTS: The new (2022) ETRN suggest a 5-year training subdivided in three phases: a first phase (2 years) of general neurology training, a second phase (2 years) of training in neurophysiology/neurological subspecialties and a third phase (1 year) to expand clinical training (e.g., in other neurodisciplines) or for research (path for clinical neuroscientist). The necessary theoretical and clinical competences as well as learning objectives in diagnostic tests have been updated, are newly organized in four levels and include 19 neurological subspecialties. Finally, the new ETRN require, in addition to a programme director, a team of clinician-educators who regularly review the resident's progress. The 2022 update of the ETRN reflects emerging requirements for the practice of neurology and contributes to the international standardization of training necessary for the increasing needs of residents and specialists across Europe.

Slow Channel Syndrome Revisited: 40 Years Clinical Follow-Up and Genetic Characterization of Two Cases.

BACKGROUND: The slow channel syndrome is a rare hereditary disorder caused by a dominant gain-of-function variant in one of the subunits of the acetylcholine receptor at the neuromuscular junction. Patients typically experience axial, limb and particularly extensor finger muscle weakness. OBJECTIVE: Age at diagnosis is variable and although the long-term prognosis is important for newly diagnosed patients, extensive follow-up studies are rare. We aim to provide answers and perspective for this patient group by presenting an elaborate description of the lifetime follow-up of two slow channel syndrome patients. METHODS: We describe 40 years follow-up in two, genetically confirmed cases (CHRNA1; c.866G > T p.(Ser289Ile)(legacy Ser269Ile) and CHRNE; c.721C > T p.(Leu241Phe)(legacy Leu221Phe) variants). RESULTS: We find that the disease course has a fluctuating pattern and is only mildly progressive. However, hormonal imbalances, (psychological) stress or excessive hot or cold environments are often aggravating factors. Quinidine and fluoxetine are helpful, but ephedrine and salbutamol may also improve symptoms. CONCLUSION: Slow channel syndrome is mildly progressive with a fluctuating pattern. The observations reported here provide a lifespan perspective and answers to the most pressing questions about prognosis and treatment options for newly diagnosed patients.

Second intravenous immunoglobulin dose in patients with Guillain-Barré syndrome with poor prognosis (SID-GBS): a double-blind, randomised, placebo-controlled trial.

BACKGROUND: Treatment with one standard dose (2 g/kg) of intravenous immunoglobulin is insufficient in a proportion of patients with severe Guillain-Barré syndrome. Worldwide, around 25% of patients severely affected with the syndrome are given a second intravenous immunoglobulin dose (SID), although it has not been proven effective. We aimed to investigate whether a SID is effective in patients with Guillain-Barré syndrome with a predicted poor outcome. METHODS: In this randomised, double-blind, placebo-controlled trial (SID-GBS), we included patients (≥12 years) with Guillain-Barré syndrome admitted to one of 59 participating hospitals in the Netherlands. Patients were included on the first day of standard intravenous immunoglobulin treatment (2 g/kg over 5 days). Only patients with a poor prognosis (score of ≥6) according to the modified Erasmus Guillain-Barré syndrome Outcome Score were randomly assigned, via block randomisation stratified by centre, to SID (2 g/kg over 5 days) or to placebo, 7-9 days after inclusion. Patients, outcome adjudicators, monitors, and the steering committee were masked to treatment allocation. The primary outcome measure was the Guillain-Barré syndrome disability score 4 weeks after inclusion. All patients in whom allocated trial medication was started were included in the modified intention-to-treat analysis. This study is registered with the Netherlands Trial Register, NTR 2224/NL2107. FINDINGS: Between Feb 16, 2010, and June 5, 2018, 327 of 339 patients assessed for eligibility were included. 112 had a poor prognosis. Of those, 93 patients with a poor prognosis were included in the modified intention-to-treat analysis: 49 (53%) received SID and 44 (47%) received placebo. The adjusted common odds ratio for improvement on the Guillain-Barré syndrome disability score at 4 weeks was 1·4 (95% CI 0·6-3·3; p=0·45). Patients given SID had more serious adverse events (35% vs 16% in the first 30 days), including thromboembolic events, than those in the placebo group. Four patients died in the intervention group (13-24 weeks after randomisation). INTERPRETATION: Our study does not provide evidence that patients with Guillain-Barré syndrome with a poor prognosis benefit from a second intravenous immunoglobulin course; moreover, it entails a risk of serious adverse events. Therefore, a second intravenous immunoglobulin course should not be considered for treatment of Guillain-Barre syndrome because of a poor prognosis. The results indicate the need for treatment trials with other immune modulators in patients severely affected by Guillain-Barré syndrome. FUNDING: Prinses Beatrix Spierfonds and Sanquin Plasma Products.

Neurofilament light chain, a biomarker for polyneuropathy in systemic amyloidosis.

OBJECTIVE: To study serum neurofilament light chain (sNfL) in amyloid light chain (AL) amyloidosis patients with and without polyneuropathy (PNP) and to corroborate previous observations that sNfL is increased in hereditary transthyretin-related (ATTRv) amyloidosis patients with PNP. METHODS: sNfL levels were assessed retrospectively in patients with AL amyloidosis with and without PNP (AL/PNP+ and AL/PNP-, respectively), patients with ATTRv amyloidosis and PNP (ATTRv/PNP+), asymptomatic transthyretin (TTR) gene mutation carriers (TTRv carriers) and healthy controls. Healthy controls (HC) were age- and sex-matched to both AL/PNP- (HC/AL) and TTRv carriers (HC/TTRv). The single-molecule array (Simoa) assay was used to assess sNfL levels. RESULTS: sNfL levels were increased both in 10 AL/PNP+ patients (p < .001) and in 10 AL/PNP- patients (p < .005) compared to 10 HC/AL individuals. sNfL levels were higher in AL/PNP+ patients than in AL/PNP- patients (p < .005). sNfL levels were also increased in 15 ATTRv/PNP+ patients, compared to both 15 HC/TTRv (p < .0001) and 15 TTRv carriers (p < .0001). ATTRv/PNP+ patients with progressive PNP (PND-score > I) had the highest sNfL levels compared to patients with early PNP (PND-score I) (p = .05). sNfL levels did not differ between TTRv carriers and HC/TTRv individuals. In the group comprising all healthy controls and in the group of TTRv carriers, sNfL levels correlated with age. CONCLUSION: sNfL levels are increased in patients with PNP in both AL and ATTRv amyloidosis and are related to severity of PNP in ATTRv amyloidosis. sNfL is a promising biomarker to detect PNP, not only in ATTRv but also in AL amyloidosis.

Vibration threshold in non-diabetic subjects.

Measuring vibration perception threshold (VPT) accurately classifies and quantifies the severity of loss of vibration perception. A biothesiometer (Bio-thesiometer®; Bio Medical Instrument Co, Ohio, USA) appears to be the most suitable tool to determine VPT due to its low inter-rater variability and low occurence of adaption to the sensation. Different VPT values for a biothesiometer have been described, however, specification on age, height and different measurement locations is currently lacking. The objective of our study was to identify determinants of vibration perception in non-diabetic subjects, in order to provide individualized normal values of VPTs for clinical practice. Measurements of the vibration perception were performed on the big toes, insteps, lateral malleoli, and wrists. A total of 205 healthy subjects were included (108 (52.7%) males) with a median [interquartile range] age of 59 [51;64] (range 21-80) years. Mean height was 174.45 ± 9.20 cm and mean weight was 82.94 ± 14.84 kg, resulting in a mean BMI of 27.19 ± 4.00 kg/m2. In stepwise forward linear regression analyses, age (st. ß = 0.51, p < 0.001) and height (st. ß = 0.43, p < 0.001) were found to be the independent unmodifiable determinants of the VPT at the big toe. Regression coefficients for quantiles of the determinants age and height were incorporated in the corresponding regression equations. This study provides equations to calculate age- and height-specific normal values for VPT that can be used in clinical practice and in large research studies.

How students and specialists appreciate the mini-clinical evaluation exercise (mini-CEX) in Indonesian clerkships.

BACKGROUND: Cultural differences might challenge the acceptance of the implementation of assessment formats that are developed in other countries. Acceptance of assessment formats is essential for its effectiveness; therefore, we explored the views of students and specialists on the practicality and impact on learning of these formats. This study was conducted to explore Indonesian students' and specialists' appreciation of the implementation of the Mini-Clinical Evaluation Exercise (Mini-CEX) in Indonesian clerkships. METHODS: This study was conducted at the Universitas Gadjah Mada, Indonesia. Participants were 52 students and 21 specialists in neurology and 78 students and 50 specialists in internal medicine. They were asked to complete a 19-item questionnaire that covered the characteristics of the mini-CEX such as its practicality, and the impact on learning and professional development. We used a Mann-Whitney U test to analyse the data. RESULTS: In total, 124 students (46 from neurology and 78 from internal medicine) and 38 specialists (13 from neurology and 25 from internal medicine) participated in this study. Students and specialists were positive about the practicality of the mini-CEX and the impact of this assessment format on learning and on professional development. The Mann-Whitney U test showed that there were no significant differences between students' and specialists' opinions on the mini-CEX, except for 2 items: specialists' appreciation of direct observation (mean rank = 93.16) was statistically significantly higher than students' appreciation of it (mean rank = 77.93; z = 2.065; p < 0.05), but students' appreciation of the item that students' past mini-CEX results affected their recent mini-CEX outcomes (mean rank = 85.29) was significantly higher than specialists' appreciation of it (mean rank = 69.12; z = 2140; p < 0.05). CONCLUSION: Students and specialists were positive about the mini-CEX in Indonesian clerkships, although it was developed and validated in another culture. We found only small differences between their appreciations, which could be explained by the patterns of specialist-student interaction in Indonesian culture as large power distance and low individualism country.

Long-term follow-up, quality of life, and survival of patients with Lambert-Eaton myasthenic syndrome.

OBJECTIVE: To study survival and to characterize long-term functional impairments and health-related quality of life (HRQOL) of patients with Lambert-Eaton myasthenic syndrome (LEMS). METHODS: In this observational study, survival of patients with LEMS, separately for nontumor (NT) and small cell lung cancer (SCLC), was compared to that of the Dutch general population and patients with SCLC. Disease course in patients with LEMS was recorded retrospectively. Several scales for functional impairments and health-related quality of life were assessed. RESULTS: We included 150 patients with LEMS. Survival was similar to that of the general population in 65 patients with NT-LEMS. Tumor survival was significantly longer in 81 patients with SCLC-LEMS compared to patients with non-LEMS SCLC (overall median survival 17 vs 7.0 months, p < 0.0001). At diagnosis, 39 (62%) of 63 patients with complete follow-up data were independent for activities of daily living, improving to 85% at the 1-year follow-up. The physical HRQOL composite score (55.9) was significantly lower than in the general population (76.3, p < 0.0001) and comparable to that of patients with myasthenia gravis (60.5). The mental HRQOL composite score was 71.8 in patients with LEMS, comparable to that of the general population (77.9, p = 0.19) and patients with myasthenia gravis (70.3). CONCLUSIONS: This study shows that patients with NT-LEMS have normal survival. Patients with SCLC-LEMS have an improved tumor survival, even after correction for tumor stage. A majority of patients with LEMS report a stable disease course and remain or become independent for self-care after treatment.

Serum Acetylcholine Receptor Antibodies Before the Clinical Onset of Myasthenia Gravis.

A patient with autoimmune myasthenia gravis and a clinical and serological follow-up of 13 years is described. In this unique case, serum samples were available up to two years before the clinical onset of the myasthenia gravis and showed gradual increase of acetylcholine receptor antibodies, starting two years before onset of the clinical symptoms.

Clinical workplace learning: perceived learning value of individual and group feedback in a collectivistic culture.

BACKGROUND: Feedback is essential for workplace learning. Most papers in this field concern individual feedback. In collectivistic cultures, however, group feedback is common educational practice. This study was conducted to investigate the perceived learning value and characteristics of individual and group feedback in a collectivistic culture. METHODS: During two weeks, on a daily basis, clerkship students (n = 215) from 12 clinical departments at Faculty of Medicine, Universitas Gadjah Mada, Yogyakarta, Indonesia, recorded individual and group feedback moments by using a structured form: the providers, focus and perceived learning value of feedback. Data were analysed with logistic regression and multilevel techniques. RESULTS: Students reported 2687 group and 1535 individual feedback moments. Group feedback more often focused on history taking, clinical judgment, patient management, patient counselling, and professional behaviour (OR ranging from 1.232, p < .01, to 2.152, p < .001), but less often on physical examination (OR = .836, p < .01). Group feedback less often aimed at correcting performance deficiencies (OR = .523, p < .001) and more often at comparing performance to the standard (OR = 2.447, p < .001) and planning action to improve performance (OR = 1.759, p < .001). Group feedback was perceived as more valuable than individual feedback (M = 4.08 and 3.96, respectively, ß group = .065, SE = .026, p < .01). CONCLUSION: In collectivistic cultures, group feedback may add to the array of educational measures that optimize student learning. Congruence between culture and type of feedback may be important for the effectiveness of feedback.

The extremity function index (EFI), a disability severity measure for neuromuscular diseases: psychometric evaluation.

OBJECTIVE: To adapt and to combine the self-report Upper Extremity Functional Index and Lower Extremity Function Scale, for the assessment of disability severity in patients with a neuromuscular disease and to examine its psychometric properties in order to make it suitable for indicating disease severity in neuromuscular diseases. DESIGN: A cross-sectional postal survey study was performed among patients diagnosed with a neuromuscular disease. METHODS: Patients completed both adapted extremity function scales, questionnaires for psychometric evaluation, and disease-specific questions. Confirmatory factor analysis was performed, and reliability and validity were examined. RESULTS: Response rate was 70% (n = 702). The Extremity Function Index model with a two-factor structure - for upper and lower extremities - showed an acceptable fit. The Extremity Function Index scales showed good internal consistency (alphas: 0.97-0.98). The known-groups validity test confirmed that Extremity Function Index scales discriminate between categories of "Extent of limitations" and "Quality of Life." Convergent and divergent validity tests confirmed that Extremity Function Index scales measure the physical impact of neuromuscular diseases. Relative validity tests showed that the Extremity Function Index scales performed well in discriminating between subgroups of patients with increasing "Extent of limitations" compared to concurrent measurement instruments. CONCLUSION: The Extremity Function Index proved to be a sound and easy to apply self-report disability severity measurement instrument in neuromuscular diseases. Implications for rehabilitation The Extremity Function Index reflects the functioning of all muscles in the upper and lower extremities involved in activities of daily living. The Extremity Function Index is an easy to administer and patient-friendly disability severity measurement instrument that has the ability to evaluate differences in disability severity between relevant neuromuscular disease subgroups. The Extremity Function Index is a valid and reliable disability severity measurement instrument for neuromuscular diseases.

Stability and relative validity of the Neuromuscular Disease Impact Profile (NMDIP).

BACKGROUND: The aim of this study was to examine the stability and relative validity (RV) of the Neuromuscular Disease Impact Profile (NMDIP) using criterion-related groups. In a previous study the NMDIP-scales showed good internal consistency, convergent and discriminant validity. Known-groups analysis showed that the NMDIP discriminates between categories of extent of limitations. METHODS: A cross-sectional postal survey study was performed on patients diagnosed with a NMD and registered at the Department of Neurology, University Medical Center Groningen, the Netherlands. Participants were asked to complete the preliminary NMDIP, the Medical Outcome study Short Form Questionnaire (SF-36), the World Health Organization Quality Of Life-abbreviation version (WHOQOL-bref), and two generic domain specific measures: the Groningen Activity Restriction Scale (GARS) and the Impact on Participation and Autonomy Questionnaire (IPAQ). The variables 'Extent of Limitations' and 'Quality of Life' were used to create criterion-related groups. Stability over time was tested using the Wilcoxon Signed Rank Test for paired samples and the intraclass correlation coefficients for repeated measures. RV was examined by comparing the ability of NMDIP with generic multidimensional health impact measures, and domain specific measures in discriminating between criterion-related subgroups using the Kruskal-Wallis H-test. RESULTS: Response rate was 70% (n = 702). The NMDIP-scales showed sufficient stability over time, and satisfactory or strong RV. In general, the NMDIP scales performed as well as or better than the concurrent measurement instruments. CONCLUSIONS: The NMDIP proved to be a valid and reliable disease-targeted measure with a broad scope on physical, psychological and social functioning.

Influence of feedback characteristics on perceived learning value of feedback in clerkships: does culture matter?

BACKGROUND: Various feedback characteristics have been suggested to positively influence student learning. It is not clear how these feedback characteristics contribute to students' perceived learning value of feedback in cultures classified low on the cultural dimension of individualism and high on power distance. This study was conducted to validate the influence of five feedback characteristics on students' perceived learning value of feedback in an Indonesian clerkship context. METHODS: We asked clerks in Neurology (n = 169) and Internal Medicine (n = 132) to assess on a 5-point Likert scale the learning value of the feedback they received. We asked them to record whether the feedback provider (1) informed the student what went well, (2) mentioned which aspects of performance needed improvement, (3) compared the student's performance to a standard, (4) further explained or demonstrated the correct performance, and (5) prepared an action plan with the student to improve performance. Data were analyzed using multilevel regression. RESULTS: A total of 250 students participated in this study, 131 from Internal Medicine (response rate 99%) and 119 from Neurology (response rate 70%). Of these participants, 225 respondents (44% males, 56% females) completed the form and reported 889 feedback moments. Students perceived feedback as more valuable when the feedback provider mentioned their weaknesses (ß = 0.153, p < 0.01), compared their performance to a standard (ß = 0.159, p < 0.01), explained or demonstrated the correct performance (ß = 0.324, p < 0.001) and prepared an action plan with the student (ß =0.496, p < 0.001). Appraisal of good performance did not influence the perceived learning value of feedback. No gender differences were found for perceived learning value. CONCLUSIONS: In Indonesia, we could validate four out of the five characteristics for effective feedback. We argue that our findings relate to culture, in particular to the levels of individualism and power distance. The recognized characteristics of what constitutes effective feedback should be validated across cultures.

The epidemiology of neuromuscular disorders: Age at onset and gender in the Netherlands.

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.

Development and testing psychometric properties of an ICF-based health measure: The Neuromuscular Disease Impact Profile.

OBJECTIVES: To develop a measure that is based on the International Classification of Functioning, Disability and Health (ICF) and reflects the prevalence and severity of disabilities related to neuromuscular disorders, and to evaluate the psychometric properties of this measure. METHODS: A preliminary questionnaire was developed, based on the categories of the ICF Core Set for Neuromuscular Diseases. Next a cross-sectional postal survey was carried out among 702 patients (70% response rate) diagnosed with a neuromuscular disease. Finally, psychometric properties were examined. RESULTS: The preliminary Neuromuscular Disease Impact Profile (NMDIP) consisted of 45 items. Factor analysis showed that the NMDIP comprised domains representing 3 ICF-components: 5 factors in the Body Functions component, 2 factors in the Activities component, and 1 factor in the Participation component. Scales showed moderate to good internal consistency (α = 0.63-0.92) and mean inter-item correlation coefficients (0.38-0.77). Convergent and discriminant validity analysis indicated that the NMDIP measures the impact of neuromuscular disease on physical, mental, and social functioning. The NMDIP discriminates between groups who differ in extent of limitations. CONCLUSION: The NMDIP is an ICF-based measure that reflects neuromuscular disease-related disabilities. It consists of 36 items divided over 8 scales with satisfactory psychometric properties and 4 single items.

Student distress in clinical workplace learning: differences in social comparison behaviours.

In medical education, student distress is known to hamper learning and professional development. To address this problem, recent studies aimed at helping students cope with stressful situations. Undergraduate students in clinical practice frequently use experiences of surrounding peers to estimate their abilities to master such challenging situations. This use of the experiences of others, known as social comparison, may affect student distress both positively and negatively. To find characteristics of a beneficial use of social comparison, we examined differences in comparison behaviours between students expressing low and high levels of distress. The participants in our study, response rate 93% (N = 301/321), were all medical students in their first year in clinical practice. They completed the General Health Questionnaire (GHQ-12) to measure distress, and three separate questionnaires to measure: (1) orientation to comparison, (2) motive for comparison, and (3) interpretation of comparison. Differences were analysed using multivariate analysis of variance. Although all students were oriented towards social comparison, the analyses showed that this orientation was less apparent among low-distress students. Besides, the low-distress students were less inclined to use motives indicative for comparisons with peers perceived as performing worse and were less negative in the interpretations of their comparisons. As social comparison is frequently used among all students, we recommend to make them aware of their comparison behaviours and inform them about the pros and cons of the distinguished aspects of the comparison process.

Meeting international standards: a cultural approach in implementing the mini-CEX effectively in Indonesian clerkships.

UNLABELLED: Abstract Background: Medical schools all over the world try to adapt their programs to meet international standards. However, local culture might hamper innovation attempts. AIMS: To describe challenges in implementing the mini-CEX in Indonesia and investigate its effect on students' clinical competence. METHODS: The study was conducted in the Internal Medicine and Neurology departments of the Universitas Gadjah Mada, Indonesia. Implementing the mini-CEX into the existing curriculum, while taking the Indonesian culture into account, implied a shift from group to individual feedback. We compared students' final clinical competence before (Internal Medicine n = 122, Neurology n = 183) and after (n = 183 and 186, respectively) the implementation of the mini-CEX, using a modified Objective Structured Long Examination Record (OSLER). The Mann-Whitney test was used to analyze the data. RESULTS: We took power distance and individualism into account to facilitate the implementation process. After implementing the mini-CEX, the OSLER results were significant higher in Internal Medicine (p < 0.05). However, no differences were found in Neurology. CONCLUSION: By managing the innovation process carefully and taking culture and local context into account, the mini-CEX can be implemented without changing the underlying concept. The shift from group to individual feedback seems to have a positive effect on student learning.

Exploring cultural differences in feedback processes and perceived instructiveness during clerkships: replicating a Dutch study in Indonesia.

CONTEXT: Cultural differences between countries may entail differences in feedback processes. AIMS: By replicating a Dutch study in Indonesia, we analysed whether differences in processes influenced the perceived instructiveness of feedback. METHODS: Over a two-week period, Indonesian students (n = 215) recorded feedback moments during clerkships, noting who provided the feedback, whether the feedback was based on observations, who initiated the feedback, and its perceived instructiveness. Data were compared with the earlier Dutch study and analysed with χ(2) tests, t-tests and multilevel techniques. Cultural differences were explored using Hofstede's Model, with Indonesia and the Netherlands differing on "power distance" and "individualism." RESULTS: Perceived instructiveness of feedback did not differ significantly between both countries. However, significant differences were found in feedback provider, observation and initiative. Indonesian students perceived feedback as more instructive if provided by specialists and initiated jointly by the supervisor and student (ßresidents = -0.201, p < 0.001 and ßjoint = 0.193, p = 0.001). Dutch students appreciated feedback more when it was based on observation. CONCLUSIONS: We obtained empirical evidence that one model of feedback does not necessarily translate to another culture. Further research is necessary to unravel other possible influences of culture in implementing feedback procedures in different countries.

Open-book tests: search behaviour, time used and test scores.

BACKGROUND: Because of the increasing medical knowledge and the focus of medical education on acquiring competencies, the use of open-book tests seems inevitable. Dealing with a large body of information, indicating which kind of information is needed to solve a problem, and finding and understanding that knowledge at the right moment are behaviours that cannot be assessed during closed-book tests. AIMS: To examine whether there is a relationship between students' search behaviour - using references or not when answering a question - during open-book tests and their test scores. METHOD: Second- (N = 491) and third-year medical students (N = 325) participated in this study. Search behaviour was operationalized as the number of questions for which students consulted their references. Furthermore, we collected data on the time students spent on answering all open-book questions and their test scores. To determine the relations, we calculated Spearman's and Pearson's correlations. RESULTS: Second- and third-year students consulted their references for 87% and 73% of the questions and spent 5.0 and 4.3 min on answering an open-book question, respectively. We did not find significant correlations between search behaviour and test scores. CONCLUSION: Both 'well' and 'poorer performing' students often consulted their references. Spending almost 5 min per open-book question in multiple choice format seems to be too much. More research is needed to establish optimal open-book test time and to explore how 'well performing' students use their references during open-book tests.

Peer influence on students' estimates of performance: social comparison in clinical rotations.

CONTEXT: During clinical rotations, students move from one clinical situation to another. Questions exist about students' strategies for coping with these transitions. These strategies may include a process of social comparison because in this context it offers the student an opportunity to estimate his or her abilities to master a novel rotation. These estimates are relevant for learning and performance because they are related to self-efficacy. We investigated whether student estimates of their own future performance are influenced by the performance level and gender of the peer with whom the student compares him- or herself. METHODS: We designed an experimental study in which participating students (n = 321) were divided into groups assigned to 12 different conditions. Each condition entailed a written comparison situation in which a peer student had completed the rotation the participant was required to undertake next. Differences between conditions were determined by the performance level (worse, similar or better) and gender of the comparison peer. The overall grade achieved by the comparison peer remained the same in all conditions. We asked participants to estimate their own future performance in that novel rotation. Differences between their estimates were analysed using analysis of variance (ANOVA). RESULTS: Students' estimates of their future performance were highest when the comparison peer was presented as performing less well and lowest when the comparison peer was presented as performing better (p < 0.001). Estimates of male and female students in same-gender comparison conditions did not differ. In two of three opposite-gender conditions, male students' estimates were higher than those of females (p < 0.001 and p < 0.05, respectively). CONCLUSIONS: Social comparison influences students' estimates of their future performance in a novel rotation. The effect depends on the performance level and gender of the comparison peer. This indicates that comparisons against particular peers may strengthen or diminish a student's self-efficacy, which, in turn, may ease or hamper the student's learning during clinical rotations. The study is limited by its experimental design. Future research should focus on students' comparison behaviour in real transitions.

Clinical features and predictors for disease natural progression in adults with Pompe disease: a nationwide prospective observational study.

BACKGROUND: Due partly to physicians' unawareness, many adults with Pompe disease are diagnosed with great delay. Besides, it is not well known which factors influence the rate of disease progression, and thus disease outcome. We delineated the specific clinical features of Pompe disease in adults, and mapped out the distribution and severity of muscle weakness, and the sequence of involvement of the individual muscle groups. Furthermore, we defined the natural disease course and identified prognostic factors for disease progression. METHODS: We conducted a single-center, prospective, observational study. Muscle strength (manual muscle testing, and hand-held dynamometry), muscle function (quick motor function test), and pulmonary function (forced vital capacity in sitting and supine positions) were assessed every 3-6 months and analyzed using repeated-measures ANOVA. RESULTS: Between October 2004 and August 2009, 94 patients aged between 25 and 75 years were included in the study. Although skeletal muscle weakness was typically distributed in a limb-girdle pattern, many patients had unfamiliar features such as ptosis (23%), bulbar weakness (28%), and scapular winging (33%). During follow-up (average 1.6 years, range 0.5-4.2 years), skeletal muscle strength deteriorated significantly (mean declines of -1.3% point/year for manual muscle testing and of -2.6% points/year for hand-held dynamometry; both p<0.001). Longer disease duration (>15 years) and pulmonary involvement (forced vital capacity in sitting position <80%) at study entry predicted faster decline. On average, forced vital capacity in supine position deteriorated by 1.3% points per year (p=0.02). Decline in pulmonary function was consistent across subgroups. Ten percent of patients declined unexpectedly fast. CONCLUSIONS: Recognizing patterns of common and less familiar characteristics in adults with Pompe disease facilitates timely diagnosis. Longer disease duration and reduced pulmonary function stand out as predictors of rapid disease progression, and aid in deciding whether to initiate enzyme replacement therapy, or when.