Influence of PBL with open-book tests on knowledge retention measured with progress tests.

The influence of problem-based learning (PBL) and open-book tests on long-term knowledge retention is unclear and subject of discussion. Hypotheses were that PBL as well as open-book tests positively affect long-term knowledge retention. Four progress test results of fifth and sixth-year medical students (n = 1,648) of three medical schools were analyzed. Two schools had PBL driven curricula, and the third one had a traditional curriculum (TC). One of the PBL schools (PBLob) used a combination of open-book (assessing backup knowledge) and closed-book tests (assessing core knowledge); the other two schools (TC and PBLcb) only used closed-book tests. The items of the progress tests were divided into core and backup knowledge. T tests (with Bonferroni correction) were used to analyze differences between curricula. PBL students performed significantly better than TC students on core knowledge (average effect size (av ES) = 0.37-0.74) and PBL students tested with open-book tests scored somewhat higher than PBL students tested without such tests (av ES = 0.23-0.30). Concerning backup knowledge, no differences were found between the scores of the three curricula. Students of the two PBL curricula showed a substantially better long-term knowledge retention than TC students. PBLob students performed somewhat better on core knowledge than PBLcb students. These outcomes suggest that a problem-based instructional approach in particular can stimulate long-term knowledge retention. Distinguishing knowledge into core and backup knowledge and using open-book tests alongside closed-book tests could enhance long-term core knowledge retention.

Validation of the ICF core set for neuromuscular diseases.

BACKGROUND: Understanding of the consequences of a neuromuscular disease (NMD) can improve when a valid sample of disease-specific categories based on the International Classification of Functioning, Disabilities, and Health (ICF) is available. OBJECTIVE: To examine the content validity of the initial ICF Core Set for neuromuscular diseases (NMDs). The initial ICF Core Set was developed for three chronic neurological diseases. DESIGN: A qualitative method. METHODS: To examine the content validity of the initial ICF Core Set for NMD, concepts in established disease-specific health-related Quality of Life Questionnaires (HRQOL) were compared with ICF categories. Next, the selected ICF categories were linked to the ICF categories in the initial ICF Core Set. RESULTS: All concepts in the HRQOL questionnaires, except one body function concept, were covered by the initial ICF Core Set. However, the NMD Core Set reflects a broader scope concerning health problems than the concepts in the HRQOL questionnaires do, especially concerning the "Participation" and "Environmental Factors" components. CONCLUSION: The NMD Core Set, as well as a measurement based on this Core Set, can contribute to a better understanding of the consequences of NMDs and can also serve as a basis for clinical practice, research, social security systems, and educational programs. CLINICAL REHABILITATION IMPACT: The newly developed NMD Core Set can be a basis for enhancing the development of rehabilitation interventions and improving overall health care for patients with a NMD.

Directing students to profound open-book test preparation: the relationship between deep learning and open-book test time.

BACKGROUND: Considering the growing amount of medical knowledge and the focus of medical education on acquiring competences, using open-book tests seems inevitable. A possible disadvantage of these tests is that students underestimate test preparation. AIMS: We examined whether students who used a deep learning approach needed less open-book test time, and how students performed on open-book questions asked in a closed-book setting. METHOD: Second- (N = 491) and third-year students (N = 325) prepared half of the subject matter to be tested closed-book and half to be tested open-book. In agreement with the Board of Examiners, some questions in the closed-book test concerned open-book subject matter, and vice versa. Data were gathered about test time, deep learning and preparation time. Repeated measurement analysis, t-tests and partial correlations were used to analyse the data. RESULTS: We found a negative relationship between deep learning and open-book test time for second-year students. Students scored the lowest on closed-book questions about open-book subject matter. CONCLUSIONS: Reduction of the available test time might force students to prepare longer and deeper for open-book tests. Further research is needed to identify variables that influence open-book test time and to determine how restrictive this time should be.

Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy.

OBJECTIVE: Identification and examination of all patients with multifocal motor neuropathy (MMN) in the Netherlands to document the clinical spectrum and response to IV immunoglobulin (IVIg) and to determine correlates of outcome. METHODS: A national cross-sectional descriptive study was performed. Ninety-seven patients were identified; 88 participated. Logistic regression analysis was used to study determinants of outcome. RESULTS: Age at onset was younger in men than in women (38 vs 45 years, p = 0.05). Onset of weakness was in distal arm (61%) or distal leg (34%), and occasionally in the upper arm (5%). Initial diagnosis was motor neuron disease in one-third of patients. Brisk, but not pathologic, reflexes in weakened muscles were found in 8%. Conduction blocks were most frequently detected in the ulnar (80%) and median (77%) nerves, but occasionally only between Erb and axilla (6%), or in the musculocutaneous nerve (1%). Ninety-four percent responded to IVIg therapy: nonresponders had longer disease duration before the first treatment (p = 0.03). Seventy-six percent received IVIg maintenance treatment at the time of this study (median duration 6 years; range 0-17): the median dose increased over the years from 12 to 17 g per week (p < 0.01). Independent determinants of more severe weakness and disability were axon loss (p < 0.001; p < 0.0001) and longer disease duration without IVIg (p = 0.03; p = 0.07). CONCLUSION: The results of this study may help aid recognition the clinical picture of MMN. Early IVIg treatment may help to postpone axonal degeneration and permanent deficits. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that IVIg improves muscle strength of patients with MMN and disability (defined as an increase of >or=1 Medical Research Council grade in at least 2 muscle groups without decrease in other muscle groups) in 94% (95% confidence interval, 86.8%-97.4%) of patients.

'Last-minute' donations influence actuarial prediction in an anatomical body donation program.

BACKGROUND: At some American and European universities the dissection program is threatened by a shortage of anatomical specimens. In contrast, the annual numbers of registrations at the University Medical Center Groningen (UMCG) in the Netherlands increased substantially in recent years. Uncontrolled body registrations and an increasing number of incoming bodies urge institutes to halt registration. This is usually carried out on an ad hoc basis because to date no analyses were available to predict the consequences of such a stop, resulting in uncertainty about the number of incoming bodies or a shortage. METHODS: The UMCG holds a database consisting of two different data sets: registered potential body donors and records of deceased body donors. This database currently consists of 2357 potential body donors and 1363 deceased body donors. These data were incorporated in an actuarial predictive model. FINDINGS: A substantial number (on average 29%) of the persons registered between 2003-2008 died within 1 year after registration and seemed to have made a 'last-minute' donation decision. Last-minute registrations are significantly more likely to be males than females (n=155 vs. n=85, p<0.01%). This new information markedly influenced final modeling. In coherence with standard models of mortality, it was possible to construct a prediction for the incoming bodies for the coming years. CONCLUSIONS: The present study provides the first method to reliably model the number of incoming deceased donors of a body donation program for 5 years based on actuarial predictions, and to orchestrate these numbers by partial donor registration stops.

Learning strategies during clerkships and their effects on clinical performance.

BACKGROUND: Previous research revealed relationships between learning strategies and knowledge acquisition. During clerkships, however, students' focus widens beyond mere knowledge acquisition as they further develop overall competence. This shift in focus can influence learning strategy use. AIM: We explored which learning strategies were used during clerkships and their relationship to clinical performance. METHODS: Participants were 113 (78%) clerks at the university hospital or one of six affiliated hospitals. Learning strategies were assessed using the 'Approaches to Learning at Work Questionnaire' (deep, surface-rational and surface-disorganised learning). Clinical performance was calculated by taking the mean of clinical assessment marks. The relationship between learning strategies and clinical performance was explored using regression analysis. RESULTS: Most students (89%) did not clearly prefer a single learning strategy. No relationship was found between learning strategies and clinical performance. DISCUSSION: Since overall competence comprises integration of knowledge, skills and professional behaviour, we assume that students without a clear preference use more than one learning strategy. Finding no relationship between learning strategies and clinical performance reflects the complexity of clinical learning. Depending on circumstances it may be important to obtain relevant information quickly (surface-rational) or understand material thoroughly (deep). In future research we will examine when and why students use different learning strategies.

[Two patients with mitochondrial respiratory chain disease]. / Twee patiënten met een mitochondriale myopathie.

A 23-year-old woman and a 13-year-old boy were diagnosed with mitochondrial respiratory chain disease. The woman had muscle pain, fatigue and bilateral ophthalmoplegia--symptoms consistent with Kearns-Sayre syndrome. The boy had aspecific symptoms; eventually, reduced activity of complex 1 was found to be the cause of the mitochondrial respiratory chain disease in the boy and his mother, who had suffered from unexplained fatigue and muscle pain for 15 years. Mitochondrial diseases often involve several organ systems. Diagnosis can be difficult, because laboratory tests such as serum and urinary lactate and creatine kinase have low sensitivity and specificity. Biochemical assessment of muscle biopsy can reveal reduced oxidation ATP synthesis and sometimes specific abnormalities in individual protein complexes. DNA analysis may be helpful in demonstrating mitochondrial or nuclear mutations or deletions. The goal of treatment is to increase mitochondrial ATP production, improve clinical symptoms and enhance stamina. Replacement of the following substances (also referred to as cofactors) may be attempted: co-enzyme Q10, antioxidants (lipoic acid, vitamins C and E), riboflavin, thiamine, creatine and carnitine. Evidence regarding the optimal treatment approach is lacking; one usually has to rely on observing effects in the individual patient.

The Lambert-Eaton myasthenic syndrome 1988-2008: a clinical picture in 97 patients.

BACKGROUND: Neuromuscular symptoms in patients with Lambert-Eaton myasthenic syndrome (LEMS) and a small cell lung cancer (SCLC) develop more rapidly than in LEMS patients without a SCLC. We studied how this clinical information, which is readily available at the first consultation, can be used to predict the presence of SCLC. PATIENTS AND METHODS: In our study we included 52 LEMS patients with SCLC and 45 non-tumor patients (NT-LEMS). We interviewed patients using a structured checklist and reviewed their clinical records. We compared frequency and onset of symptoms during the course of LEMS. RESULTS: In the first six months, over half the SCLC-LEMS patients had developed seven separate symptoms, while NT-LEMS patients developed only two symptoms. Proximal leg weakness and dry mouth were early symptoms in both groups. Rapid involvement of proximal arm muscles (p=0.0001), distal arm muscles (p=0.0037), distal leg muscles (p=0.0002), dysartria (p=0.0091) and the presence of erectile dysfunction (p=0.007) were found significantly more often in SCLC-LEMS patients in both cohorts. Cerebellar symptoms, although present in 9% of LEMS patients, were almost exclusively related to SCLC-LEMS. CONCLUSION: A rapidly progressive course of disease from onset in LEMS patients should raise a high suspicion of SCLC. Special attention should be paid to involvement of upper extremities, involvement of distal arm and distal leg muscles, to erectile dysfunction and probably ataxia in order to discriminate between SCLC-LEMS and NT-LEMS.

Open-book tests to complement assessment-programmes: analysis of open and closed-book tests.

Today's health sciences educational programmes have to deal with a growing and changing amount of knowledge. It is becoming increasingly important for students to be able to use and manage knowledge. We suggest incorporating open-book tests in assessment programmes to meet these changes. This view on the use of open-book tests is discussed and the influence on test quality is examined. To cope with the growing amount of medical knowledge, we have divided the body of knowledge into core knowledge, which students must know without need for references, and backup knowledge, which students need to understand and use properly with the help of references if so desired. As a result, all tests consist of a subtest for reproduction and understanding of core knowledge (a closed-book test) and a subtest for the ability to understand and manage backup knowledge (an open-book test). Statistical data from 14 such double-subtest exams for first and second-year students were analyzed for two cohorts (N = 435 and N = 449) with multilevel analysis, in accordance with generalizability theory. The reliability of the open and closed-book sections of the separate tests varied between 0.712 and 0.850. The open-book items reduce reliability somewhat. The estimated disattenuated correlation was 0.960 and 0.937 for cohorts 1 and 2 respectively. It is concluded that the use of open-book items with closed-book items slightly decreases test reliability but the overall index is acceptable. In addition, open and closed-book sections are strongly positively related. Therefore, open-book tests could be helpful in complementing today's assessment programmes.

Strong association of MuSK antibody-positive myasthenia gravis and HLA-DR14-DQ5.

The authors studied the HLA profile of 23 white Dutch patients with muscle-specific kinase antibody-positive myasthenia gravis (MuSK Ab+ MG) and found an association with HLA-DR14-DQ5 (odds ratio 8.5; 95% CI 3.9 to 18.7; p = 4.9 x 10(-5)). Fifty-two percent of the patients carried the DR14 allele compared with 5% of the controls (p = 1.0 x 10(-8)). This association between MuSK Ab+ MG and a relatively rare HLA haplotype differs from the previously described association of early-onset acetylcholine receptor Ab+ MG with HLA-B8-DR3.

Association of the Fc gamma receptor IIA-R/R131 genotype with myasthenia gravis in Dutch patients.

Myasthenia gravis (MG) susceptibility is partially determined by allelic heterogeneity of immune-modulatory genes. IgG receptors (FcgammaR) link the humoral and cellular branches of the immune system, and regulate immune responses and inflammation. Three FcgammaR subclasses (FcgammaRIIa, FcgammaRIIIa, and FcgammaRIIIb) exhibit functional polymorphisms, which affect efficiency of FcgammaR-mediated functions. FcgammaRIIa genotypes, but not FcgammaRIIIa and FcgammaRIIIb genotypes, were differentially distributed among 107 MG patients as compared to 239 healthy controls (Pz.Lt;0.01), with a relative increase of the FcgammaRIIa-R/R131 genotype (Odds ratio 2.4, 95% confidence interval 1.4-3.9). These data suggest that the FcgammaRIIa-R/R131 genotype is a marker for susceptibility to MG.

Difference in distribution of muscle weakness between myasthenia gravis and the Lambert-Eaton myasthenic syndrome.

BACKGROUND: Myasthenia gravis and the Lambert-Eaton myasthenic syndrome (LEMS) may have a similar distribution of muscle weakness. Deciding on a diagnosis of myasthenia gravis or LEMS on clinical grounds may therefore be difficult. OBJECTIVE: To compare the localisation of initial muscle weakness and the distribution of weakness at the time of maximum severity in patients with myasthenia gravis and LEMS. SUBJECTS: 101 patients with myasthenia gravis and 38 patients with LEMS. RESULTS: In myasthenia gravis, initial weakness involved extraocular muscles in 59%, bulbar muscles in 29%, and limb muscles in 12% of the patients. In LEMS no patient had ocular weakness, 5% had bulbar weakness, and 95% had weakness of the limbs as the first symptom (p < 0.001). At the point of maximum severity, weakness in myasthenia gravis was purely ocular in 25%, oculobulbar in 5%, restricted to the limbs in 2%, and present in both oculobulbar muscles and limbs in 68%. At this point, none of the LEMS patients had weakness restricted to extraocular or bulbar muscles (p = 0.002). The legs were affected in all LEMS patients, whereas in 12 patients with generalised myasthenia gravis limb weakness was restricted to the arms (p = 0.024). CONCLUSIONS: In a patient suspected to have a myasthenic syndrome whose first symptom is ocular weakness, LEMS is virtually excluded. Limb weakness confined to the arms is only found in generalised myasthenia gravis and not in LEMS. Muscle weakness in myasthenia gravis tends to develop in a craniocaudal direction, and in the opposite direction in LEMS.

Masticatory performance in patients with myasthenia gravis.

Masticatory muscle electromyograms (EMGs) were recorded while patients with bulbar myasthenia gravis chewed artificial food and compared with those of patients with ocular myasthenia gravis, patients in clinical remission who had previously suffered from bulbar myasthenia gravis and healthy individuals. Masticatory performance and EMGs were significantly smaller in the bulbar group. There were no indications of subclinical masticatory muscle weakness in patients with bulbar myasthenia gravis in remission and in patients with ocular myasthenia gravis. Patients with bulbar myasthenia gravis barely compensated for muscular weakness by chewing at a higher percentage of their maximal EMG. These quantitative findings, when combined with subjective reports of masticatory muscle weakness, show that a need to support the jaw is characteristic of patients with bulbar myasthenia gravis who produce low EMG activity.

Grey scales uncover similar attentional effects in homonymous hemianopia and visual hemi-neglect.

Multi-component models of visual hemi-neglect have postulated that visual hemi-neglect is characterised by various attentional deficits. A grey scales task has been developed to quantify the early, automatic, (perhaps obligatory) ipsilesional orienting of visual attention, frequently assumed as the first of these attentional deficits. Explanations for this attentional imbalance are up until now mainly formulated in terms of right hemisphere activation. This lateral attentional bias has also been demonstrated in controls, in whom it is expressed as a leftward perceptual asymmetry. We reproduced previous literature findings on a grey scales task, considering controls and neglect patients. Three patients with neglect showed an extreme ipsilesional lateral bias. This bias did not change during or after cognitive rehabilitation. Additionally, we presented this grey scale task to 32 patients with left- and right-sided homonymous hemianopia (HP). HP is the loss of sight in one visual hemi-field. The HH patients had no clinical signs of impaired lateralised attention. Results revealed that HH patients showed a similar ipsilesional bias, albeit to a lesser degree than in neglect. Left-sided HH patients presented a quantitatively similar, but qualitatively opposite bias than the right-sided HH patients. We suggest that sensory effects can be an alternative source of attentional imbalance, which can interact with the previously proposed (right) hemispheric effects. This suggests that the perceptual asymmetry in the grey scales task is not necessarily an indicator of impaired right hemisphere attention. It rather suggests a pattern of functional cerebral asymmetry, which can also be caused by asymmetric sensory input.

HLA class I and II in Lambert-Eaton myasthenic syndrome without associated tumor.

Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder, in which antibodies against voltage-gated calcium channels located at nerve terminals cause muscle weakness and autonomic dysfunction. In approximately half of the patients the autoimmune process is initiated by a tumor. In the other half of patients no tumor is found and the etiology is unknown. The aims of this study were to investigate the strength of HLA-associations with nontumor LEMS (NT-LEMS) and to study the relation of HLA-haplotypes with age at onset of LEMS and other clinical features. Therefore, typing of HLA class I and II was performed in 19 patients with NT-LEMS, who were clinically evaluated. NT-LEMS was significantly associated with alleles of both HLA-class I (i.e. HLA-B8) as well as -class II (i.e. HLA-DR3 and -DQ2). HLA-B8+ patients had significantly younger age at onset of LEMS and tended to be female. This study shows that HLA-class I haplotype is associated with a distinct phenotype in NT-LEMS.

Controlled study of EMG activity of the jaw closers and openers during mastication in patients with myasthenia gravis.

Mastication was evaluated in patients with bulbar myasthenia gravis and compared with that of patients with ocular myasthenia gravis, patients in remission who previously suffered from bulbar symptoms, and healthy controls. Bulbar myasthenia gravis may impair mastication due to weakness of the masticatory muscles. The aim of the study was to objectively evaluate the influence of myasthenia gravis on mastication. The subjects chewed a piece of breakfast cake and chewed 1 min on a piece of chewing gum. Surface EMG of the masseter muscle, temporalis muscle and jaw opener muscles was recorded. Statistical analysis revealed that bulbar patients produced significantly less EMG activity in the closing phase of a chewing cycle in both experiments. The EMG of the masseter muscle expressed as percentage of the maximum EMG during maximal clenching showed significantly higher values in the bulbar group than in the other groups. This was not found for the temporalis muscle. It was suggested that bulbar patients use a strategy of limited effort to produce a bolus that can be swallowed. The ocular patients and the patients in remission showed no subclinical impairments in muscle function during chewing.