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Most current research on land-use intensification addresses its potential to either threaten biodiversity or to boost agricultural production. However, little is known about the simultaneous effects of intensification on biodiversity and yield. To determine the responses of species richness and yield to conventional intensification, we conducted a global meta-analysis synthesizing 115 studies which collected data for both variables at the same locations. We extracted 449 cases that cover a variety of areas used for agricultural (crops, fodder) and silvicultural (wood) production. We found that, across all production systems and species groups, conventional intensification is successful in increasing yield (grand mean + 20.3%), but it also results in a loss of species richness (-8.9%). However, analysis of sub-groups revealed inconsistent results. For example, small intensification steps within low intensity systems did not affect yield or species richness. Within high-intensity systems species losses were non-significant but yield gains were substantial (+15.2%). Conventional intensification within medium intensity systems revealed the highest yield increase (+84.9%) and showed the largest loss in species richness (-22.9%). Production systems differed in their magnitude of richness response, with insignificant changes in silvicultural systems and substantial losses in crop systems (-21.2%). In addition, this meta-analysis identifies a lack of studies that collect robust biodiversity (i.e. beyond species richness) and yield data at the same sites and that provide quantitative information on land-use intensity. Our findings suggest that, in many cases, conventional land-use intensification drives a trade-off between species richness and production. However, species richness losses were often not significantly different from zero, suggesting even conventional intensification can result in yield increases without coming at the expense of biodiversity loss. These results should guide future research to close existing research gaps and to understand the circumstances required to achieve such win-win or win-no-harm situations in conventional agriculture., (© 2019 John Wiley & Sons Ltd.)

Background: Multiple sclerosis (MS) is a chronic disorder of the central nervous system with an unpredictable disease course. Life partners often become caregivers, which can be both rewarding and challenging, as the caregiver's physical and mental health is often negatively affected. Previous studies on caregiver strain focused on caregivers of persons with MS with relatively high disability levels, while caregiver strain may already be experienced by life partners living with mildly disabled persons with MS., Objective: The current study examines factors associated with caregiver strain in life partners of persons with mild disability due to relapsing-remitting MS., Methods: We included 173 persons with relapsing-remitting MS (79% female; mean age 42.8 years; 90% employed; median EDSS 2.0) and their life partners. The life partners completed questionnaires on caregiver strain and neuropsychiatric and cognitive functioning of the person with MS. The persons with MS completed questionnaires about demographics, fatigue, personality, physical, cognitive and neuropsychiatric functioning, and underwent neuropsychological and neurological examinations. A linear regression analysis was conducted to examine predictors of caregiver strain., Results: 24% of the life partners experienced above average levels of caregiver strain. A multivariate linear regression analysis revealed that a higher age of the person with MS (β = 0.16, p = 0.04), more physical disability (β = 0.17 p = 0.04), more cognitive and neuropsychiatric problems of the person with MS as reported by the life partner (β = 0.33, p = 0.001) and higher severity of neuropsychiatric symptoms as reported by the life partner (β = 0.32, p = 0.001) were associated with higher caregiver strain (R 2  = 0.49)., Conclusion: Higher caregiver strain in life partners of persons with mild disability due to relapsing-remitting MS was primarily associated with cognitive and neuropsychiatric problems of the person with MS., (Copyright © 2019. Published by Elsevier B.V.)

Background: The aim of this study was to examine whether work capabilities differ between workers with Multiple Sclerosis (MS) and workers from the general population. The second aim was to investigate whether the capability set was related to work and health outcomes., Methods: A total of 163 workers with MS from the MS@Work study and 163 workers from the general population were matched for gender, age, educational level and working hours. All participants completed online questionnaires on demographics, health and work functioning. The Capability Set for Work Questionnaire was used to explore whether a set of seven work values is considered valuable (A), is enabled in the work context (B), and can be achieved by the individual (C). When all three criteria are met a work value can be considered part of the individual's 'capability set'., Results: Group differences and relationships with work and health outcomes were examined. Despite lower physical work functioning (U = 4250, p = 0.001), lower work ability (U = 10591, p = 0.006) and worse self-reported health (U = 9091, p ≤ 0.001) workers with MS had a larger capability set (U = 9649, p ≤ 0.001) than the general population. In workers with MS, a larger capability set was associated with better flexible work functioning (r = 0.30), work ability (r = 0.25), self-rated health (r = 0.25); and with less absenteeism (r = - 0.26), presenteeism (r = - 0.31), cognitive/neuropsychiatric impairment (r = - 0.35), depression (r = - 0.43), anxiety (r = - 0.31) and fatigue (r = - 0.34)., Conclusions: Workers with MS have a larger capability set than workers from the general population. In workers with MS a larger capability set was associated with better work and health outcomes., Trial Registration: This observational study is registered under NL43098.008.12: 'Voorspellers van arbeidsparticipatie bij mensen met relapsing-remitting Multiple Sclerose'. The study is registered at the Dutch CCMO register ( https://www.toetsingonline.nl ). This study is approved by the METC Brabant, 12 February 2014. First participants are enrolled 1 st of March 2014.

Policies and research increasingly focus on the protection of ecosystem services (ESs) through priority-area conservation. Priority areas for ESs should be identified based on ES capacity and ES demand and account for the connections between areas of ES capacity and demand (flow) resulting in areas of unique demand-supply connections (flow zones). We tested ways to account for ES demand and flow zones to identify priority areas in the European Union. We mapped the capacity and demand of a global (carbon sequestration), a regional (flood regulation), and 3 local ESs (air quality, pollination, and urban leisure). We used Zonation software to identify priority areas for ESs based on 6 tests: with and without accounting for ES demand and 4 tests that accounted for the effect of ES flow zone. There was only 37.1% overlap between the 25% of priority areas that encompassed the most ESs with and without accounting for ES demand. The level of ESs maintained in the priority areas increased from 23.2% to 57.9% after accounting for ES demand, especially for ESs with a small flow zone. Accounting for flow zone had a small effect on the location of priority areas and level of ESs maintained but resulted in fewer flow zones without ES maintained relative to ignoring flow zones. Accounting for demand and flow zones enhanced representation and distribution of ESs with local to regional flow zones without large trade-offs relative to the global ES. We found that ignoring ES demand led to the identification of priority areas in remote regions where benefits from ES capacity to society were small. Incorporating ESs in conservation planning should therefore always account for ES demand to identify an effective priority network for ESs., (© 2016 The Authors. Conservation Biology published by Wiley Periodicals, Inc. on behalf of Society for Conservation Biology.)

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.

Almost 20 % of cerebral ischaemic strokes occur in the posterior circulation. Estimates are that 20 % of these patients present with isolated vertigo. In approximately one-sixth to one-third of these patients, this symptom is wrongly diagnosed to be peripheral vestibular in origin. As a result, these missed stroke patients are withheld from therapeutic and secondary prophylactic treatment, which may result in unnecessary morbidity and mortality. We therefore propose a diagnostic algorithm concerning the clinical differentiation of acute vestibular syndrome (AVS) patients based on a critical review of the available literature.

Biodiversity conservation and agricultural production are often seen as mutually exclusive objectives. Strategies for reconciling them are intensely debated. We argue that harmonization between biodiversity conservation and crop production can be improved by increasing our understanding of the underlying relationships between them. We provide a general conceptual framework that links biodiversity and agricultural production through the separate relationships between land use and biodiversity and between land use and production. Hypothesized relationships are derived by synthesizing existing empirical and theoretical ecological knowledge. The framework suggests nonlinear relationships caused by the multifaceted impacts of land use (composition, configuration, and intensity). We propose solutions for overcoming the apparently dichotomous aims of maximizing either biodiversity conservation or agricultural production and suggest new hypotheses that emerge from our proposed framework.

Objective: The Neck Disability Index (NDI) is a patient self-assessed outcome measurement tool to assess disability, and that is frequently used to evaluate the effects of the treatment of neck-related problems. In individualized medicine it is mandatory that patients can interpret data in order to choose a treatment. A change of NDI or an absolute NDI is generally meaningless to a patient. Therefore, a correlation between the qualification of the clinical situation rated by the patient and the NDI score was evaluated., Methods: Patients who completed an NDI after anterior surgery because of symptomatic single level degenerative cervical disc disease were asked one month after completion of the NDI to qualify their clinical situation of a 5-item Likert scale varying from excellent to bad. Since a clear distinction between the categories was not possible based on the total NDI score, a ROC-curve was built, and the AUC computed in order to estimate best dichotomization in qualification of the clinical situation. The best corresponding cut-off point for the NDI total score was found by studying sensitivity and specificity for all possible cut-off points., Results: 102 patients were included. The highest AUC was obtained by dichotomizing the qualification into a group with good outcome and less-good outcome. The highest sensitivity and specificity for the dichotomized qualification as good outcome corresponded to a NDI ≤ 7. Sensitivity was 81.08% and specificity was 78.57%., Conclusion: This is the first study that correlated the qualification of the situation by the patients themselves and NDI. An NDI ≤ 7 corresponded to a good outcome according to the patients. This is valuable information to inform patients in their decision for any treatment., Competing Interests: The authors have declared that no competing interests exist.

Background: About 5-15% of people in the Western world will suffer from an extended period of tinnitus during their lifetime. This is often a non-treatable, disabling disorder. Tinnitus can be classified as pulsatile or non-pulsatile. Pulsatile tinnitus can be caused by a treatable neurovascular compression., Case Description: Here we describe two patients, a 68-year old woman and 40-year old man suffering from pulsatile tinnitus and hemifacial spams due to neurovascular compression of the facial and acoustic nerve in the anterior cranial fossa. After microvascular decompression using the Jannetta procedure, in which a sponge was placed between the blood vessel and the nerve, the tinnitus and hemifacial spasm disappeared., Conclusion: Recognition of hemifacial spasm in patients with tinnitus is important because the symptoms are treatable when it is due to neurovascular compression. Because hemifacial spasm can manifest in subtle forms - for instance unilateral blinking - this important symptom can easily be overlooked.

Objective: The aims of the study are to determine the intra-, interobserver, and the test re-test reliability of the cervical and ocular vestibular evoked myogenic potentials (VEMPs)., Methods: Twenty healthy subjects underwent acoustically and forehead tap elicited cervical and ocular VEMPs. The measurements were repeated one week later., Results: The intra- and interobserver reliability of both ocular and cervical VEMPs is excellent. The test re-test reliability of the raw p13n23 peak-to-peak amplitudes of the cervical VEMPs is excellent (ICC: 0.76, 0.87) and the p13 latencies show a good reliability (ICC: 0.56, 0.73). The raw n1p1 peak-to-peak amplitudes of the ocular VEMPs show a fair-to-good test re-test reliability (ICC: 0.51, 0.64) and the n1 and p1 latencies show a poor reliability (ICC: -0.17 ≤ x ≤ 0.44)., Conclusions: The intra- and interobserver reliability of the cervical and ocular VEMPs is excellent. The raw ocular and cervical VEMP peak-to-peak amplitudes are the most reliable parameters, followed by the cervical VEMP latencies. The ocular VEMP latencies shows a poor test re-test reliability. The individual VEMP results, however, remained within normal limits despite the test re-test variability.

Objective: Warming cold limbs by hot water immersion prior to nerve conduction studies may be cumbersome in certain patients. The aim of the present study is to test whether application of hot packs would be as efficient as hot water immersion., Methods: Cold limbs of 10 healthy volunteers were warmed: in half of subjects by hot packs and, after cooling down, by hot water immersion; vice versa in the other half. Motor and sensory nerve conduction studies of upper and lower extremities were performed before and after warming with two different methods., Results: There are no relevant differences in temperatures or nerve conduction velocities after warming with either hot packs or water., Conclusion: Hot packs are as effective as hot water immersion for warming cold limbs prior to electrodiagnostic testing. Since hot packs are safer, cleaner, and easier to use, we prefer warming by hot packs instead of hot water immersion before performing nerve conduction studies., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Background: Currently available disease-modifying treatments acting by modifying the immune response are ineffective in progressive multiple sclerosis (MS), which is caused by a widespread axonal degeneration. Mechanisms suspected to be involved in this widespread axonal degeneration are reduced axonal energy metabolism, axonal glutamate toxicity, and reduced cerebral blood flow. Fluoxetine might theoretically reduce axonal degeneration in MS because it stimulates energy metabolism through enhancing glycogenolysis, stimulates the production of brain-derived neurotrophic factor, and dilates cerebral arterioles. The current document presents the protocol of a clinical trial to test the hypothesis that fluoxetine slows down the progressive phase of MS., Methods/design: The FLUOX-PMS trial is a multi-center, randomized, controlled and double-blind clinical study. A total of 120 patients with the diagnosis of either secondary or primary progressive MS will be treated either by fluoxetine (40 mg daily) or placebo for a total period of 108 weeks. The primary endpoint is the time to confirmed disease progression defined as either at least a 20% increase in the timed 25-Foot Walk or at least a 20% increase in the 9-Hole Peg Test. Secondary endpoints include the Hauser ambulation index, cognitive changes, fatigue, magnetic resonance imaging of the brain, and in a small subgroup optical coherence tomography., Discussion: The FLUOX-PMS trial will gives us information as to whether fluoxetine has neuroprotective effects in patients with progressive MS., Trial Registration: Eudra-CT: 2011-003775-11.

The aim of this study was to investigate the results of both clinical testing and standardised nerve conduction studies performed on patients with Carpal tunnel syndrome (CTS) complaints, who had been referred to the neurologist by their general practitioners. Analysis of the data of neurological examination and electrodiagnostic tests (EDX) were performed on patients that had been referred by general practitioners. A total of 232 patients with clinically defined CTS, who had been referred by general practitioners, were seen by a neurologist and subsequently underwent electrodiagnostic testing. The diagnosis of CTS made by general practitioners was clinically confirmed by the neurologist in 187 of 232 (81%) patients. In these 187 patients, EDX confirmed CTS clinical diagnosis in 180. In 40 (17%), the neurologists disagreed with the clinical diagnosis of CTS because signs and symptoms were not those of clinical CTS. We showed that general practitioners are very well capable of making a clinical diagnosis of CTS. Therefore, direct referral of patients by general practitioners for nerve conduction studies to have their diagnosis of CTS confirmed is a desirable and time-saving procedure.

Background: Higher rates of acute rejection (AR) and reduced graft survival have been reported in patients with cytomegalovirus (CMV) infection, but an association between these factors remains controversial., Methods: In this study, serial protocol biopsies (PBs) and clinically indicated biopsies (IBs) from a large cohort of renal allograft recipients (n &frac14; 594) were analyzed to examine the relation between CMV and AR., Results: Patients with CMV were more likely to receive IB (85 of the 153 patients; 56%) compared to patients without CMV (138 of 441 patients; 32%; P = 0.003). However, this did not translate into a greater number of patients with episodes of acute cellular rejection on histopathology in IBs. Analysis of PBs revealed a significantly higher number of episodes of rejection per patient with CMV infection (P = 0.04), but only in a subgroup of patients with triple immunosuppression. Long-term graft function post-transplantation was analyzed in four different subgroups according to CMV infection and/or AR. Differences in renal function were apparent within the first 6 weeks after transplantation and persisted during follow-up, with the best renal function in patients without AR or CMV, whereas patients with both AR and CMV had the worst (P < 0.012 at 1 year; P < 0.001 at 2 years). On average, the latter group had significantly older donors and more often delayed graft function., Conclusions: Our data suggests that the link between CMV and AR is far less significant than previously thought. Outcome in patients with CMV may be more determined by coexisting conditions like high donor age and delayed graft function.

High resolution sonography is a relatively new diagnostic technique in diagnosing carpal tunnel syndrome (CTS). Normal values in different studies, however, vary and this makes their practical use difficult. The aim of this study was to establish normal values for the median nerve cross-sectional area (CSA) and to investigate the value of measuring additional parameters. Ninety-eight wrists of 29 women and 25 men without signs or symptoms of CTS were included. Width and circumference of the wrist were measured. The CSA of the median nerve at the level of the pisiform bone was measured using ultrasonography. We found a significant correlation between the CSA of the median nerve at the wrist and wrist circumference. Measuring wrist circumference will establish the upper level of normal more accurately compared to predictions solely based upon gender. This has important implications in diagnosing CTS with ultrasonography.

Although infections with the novel pandemic 2009 influenza A (H1N1) virus (A/H1N1/2009) appeared to be relatively mild during the first summer of circulation ('off season'), there has been significant morbidity and hospitalization and several fatal cases. Thus, rapid detection of A/H1N1/2009 is crucial for efficient treatment and infection control measures. In contrast to seasonal influenza, where point-of-care (POC) rapid antigen tests and direct fluorescent antibody (DFA) staining ensure rapid detection, diagnosis of A/H1N1/2009 has so far been based on RT-PCR. This study retrospectively compared the performance of the Quidel QuickVue POC test, DFA staining and virus isolation with that of RT-PCR for A/H1N1/2009 detection in 526 respiratory specimens collected during the first wave of the outbreak from May to September 2009. A/H1N1/2009 was detected in 9.1% (48/526) of samples. One hundred and thirty-seven of the A/H1N1/2009 PCR-negative samples were additionally tested using a RealAccurate Respiratory RT-PCR panel, revealing other respiratory viruses (mainly entero/rhino- and adenoviruses) in 42.3% (58/137). All methods analysed detected A/H1N1/2009 with excellent specificity but different sensitivities (POC test: 18.2%; DFA staining: 38.7%; virus isolation: 45.7%). Therefore, the POC test was not suitable for diagnosis, detecting A/H1N1/2009 only if present in high concentrations (corresponding median Ct value=19.0; range=16.5-21.4). DFA staining was also able to detect A/H1N1/2009 in specimens with a lower virus concentration (median Ct value=24.0; range=16.5-29.8). Virus isolation, which was positive after a median time of 7.5 days, was too time-consuming. In summary, DFA staining is superior to POC testing and may be appropriate for patients expected to have a rather high level of virus replication. Nevertheless, in DFA-negative specimens, A/H1N1/2009 should be excluded by RT-PCR.

Angiogenesis plays an important role in the growth and viability of hematologic malignancies. Emerging data suggest a crucial involvement of the endothelial-specific Tie2 receptor and its antagonistic ligand Angiopoietin-2 (Ang-2) in this process. The purpose of this study was to elucidate whether the soluble domain of the Tie2 receptor (sTie2)predicts outcome in patients with acute myeloid leukemia(AML) undergoing allogeneic hematopoietic stem cell transplantation(HSCT). Serum levels of sTie2 and Ang-2 were measured by ELISA in 181 AML patients before conditioning for HSCT. The median follow-up time was 22 months after HSCT. Pre-HSCT sTie2 levels were significantly higher inpatients (median 2.2 (range 1.8-3.0) ng/mL) compared to healthy controls (1.3 (0.9-1.6); p<0.0001). Elevated sTie2 levels were independently associated with active AML but did not relate to cytogenetics/mutational status before transplantation. Logistic regression analysis identified elevated sTie2 (odds ratio (OR) 3.07 (95% confidence interval(CI; 1.56-6.04), p=0.001) as a strong predictor for disease relapse and poor overall survival after HSCT. In a multimarker approach the highest risk for relapse was observed inpatients with both elevated sTie2 and elevated Ang-2 (OR 4.07, (95% CI 1.79-9.25) p<0.0001), as well as patients with both elevated Ang-2 and elevated bone marrow blast count (OR 4.16, (95% CI 1.88-7.36) p<0.0001). Elevated serum sTie2 levels were related to active leukemia,correlated with the percentage of leukemic blasts in the bone marrow, and independently predicted relapse in AML patients after allogeneic HSCT. Furthermore, our data indicate that Tie2 shedding and Ang-2 release seem to reflect overlapping, but nevertheless distinctive features in leukemia-associated neoangiogenesis.

A 87-year-old woman was admitted with a rapidly progressive confusion, disorientation and myoclonus, all suggestive of sporadic Creutzfeldt-Jakob disease (sCJD). This diagnosis was initially strongly supported by the EEG, which showed slow background activity and triphasic waves, combined with the finding of an increased level of 14-3-3 protein in the cerebrospinal fluid. Remarkably, this patient had also developed hypothermia, which, after warming-up, resulted in alleviation of the mental disturbances and disappearance of myoclonus. Over time, the EEG abnormalities disappeared. She recovered clinically for which reason the diagnosis of sCJD had to be rejected; however, she kept the inability to maintain body temperature (poikilothermia). Therefore, in patients with the aforementioned symptoms body temperature should be measured and adequately managed. Our hypothesis is that she suffered from a misleading acquired encephalopathy with reversible EEG and laboratory features, mimicking sCJD. When laboratory findings suggest sCJD it remains very important to see whether or not these findings are compatible with the clinical observations.

Biliary atresia (BA) is the most frequent indication for paediatric liver transplantation. We tested the hypothesis of a viral aetiology of this disease by screening liver samples of a large number of BA patients for the common human hepatotropic viruses. Moreover, we correlated our findings to the expression of Mx protein, which has been shown to be significantly up-regulated during viral infections. Seventy-four liver biopsies (taken during Kasai portoenterostomy) were tested by polymerase chain reaction (PCR) for DNA viruses (herpes simplex virus [HSV], Epstein-Barr virus [EBV], varicella zoster virus [VZV], cytomegalovirus [CMV], adenovirus, parvovirus B19 and polyoma BK) and RNA viruses (enteroviruses, rotavirus and reovirus 3). Mx protein expression was assessed by immunohistochemistry. Virus DNA/RNA was found in less than half of the biopsies (8/74 CMV, 1/74 adenovirus; 21/64 reovirus, 1/64 enterovirus). A limited number presented with double infection. Patients that had detectable viral RNA/DNA in their liver biopsies were significantly older than virus-free patients (P = 0.037). The majority (54/59) of the liver biopsies showed expression of Mx proteins in hepatocytes, bile ducts and epithelium. Our data suggest that the known hepatotropic viruses do not play a major role in the aetiology and progression of BA. Their incidence appears to be, rather, a secondary phenomenon. Nonetheless, the inflammatory response in the livers of BA patients mimics that observed during viral infections.

Emerging data suggest a critical role for bone marrow angiogenesis in hematologic malignancies. The angiopoietin/Tie ligand-receptor system is an essential regulator of this process. We evaluated whether circulating angiopoietin-2 (Ang-2) is a predictor for the probability of disease-free survival (DFS) in allogeneic hematopoietic stem cell transplantation (allo-HSCT) for high-risk acute myeloid leukemia or myelodysplastic syndrome. Ang-2 was measured by enzyme-linked immunosorbent assay in serum from 20 healthy controls and 90 patients with acute myeloid leukemia or myelodysplastic syndrome before conditioning for HSCT. Circulating Ang-2 was elevated in patients (median, 2.21 ng/mL; range, 0.18-48.84 ng/mL) compared with controls (median, 0.87 ng/mL; range, 0.27-4.51 ng/mL; P < .001). Multivariate analyses confirmed the independent prognostic impact of Ang-2 (hazard ratio [HR] = 2.46; 95% confidence interval [CI], 1.27-4.76, P = .005), percentage of bone marrow infiltration (HR = 1.14; 95% CI, 1.01-1.29, P = .033), and chemotherapy cycles before HSCT (HR = 1.38; 95% CI, 1.01-1.08, P = .048). Regression tree analysis detected optimal cutoff values for Ang-2 and recursively identified bone marrow blasts and Ang-2 as the best predictors for DFS. Because few predictors for DFS exist in the setting of allo-HSCT, Ang-2 may be used as a readily available powerful biomarker to pre-estimate DFS and may open new perspectives for risk-adapted treatment of high-risk myeloid malignancies.

An 80-year-old man is described with a left-sided recurrent laryngeal nerve palsy after primary total hip arthroplasty. Intubation was uncomplicated. Aetiology is discussed. It might be caused by intubation injury, stretch-induced injury due to cervical hyperextension or inappropriate positioning during surgery in the lateral approach or a combination.

The current practice in and the opinion about the treatment of carpal tunnel syndrome by surgeons in the Netherlands was evaluated in respect of the extent to which electrodiagnostic studies are used or needed to confirm the diagnosis. Questionnaires were sent to all Dutch surgeons who operate on patients with carpal tunnel syndrome. The response rate was 47% (324 out of 686). The majority of neurosurgeons and orthopaedic surgeons seldom operate without electrodiagnostic confirmation in line with the Dutch consensus guideline on this subject. In contrast, plastic surgeons operate more often on patients with clinically defined carpal tunnel syndrome even with normal electrodiagnostic studies. Knowledge of these strikingly different diagnostic and therapeutic strategies and opinions may influence diagnostic and referral behaviour of clinical neurologists and others.

DFNA9 is an autosomal dominant genetic inner-ear hearing impairment that starts to show itself in the 3rd and 4th decades of life. This hearing impairment may be of a different degree of severity in each ear. Progression of hearing loss is about 3 dB/year. In about one in three patients severe vestibular symptoms similar to those in Ménière's disease are present as a result of a progressive impairment of the vestibular system. Several mutations were found in the COCH-gene on chromosome 14. There are indications that some of the mutations disrupt the folding of the cochlin protein, an important component of the extracellular matrix in the inner ear. DNA-diagnostics confirming the diagnosis ofDFNA9 are possible.

Several neurological complications are associated with severe falciparum malaria. Cerebral malaria is one of the most life-threatening complications. A few patients may experience a neurological syndrome after complete recovery from Plasmodium falciparum infection. In the literature especially the postmalaria neurological syndrome (PMNS), acute disseminated encephalomyelitis (ADEM) and delayed cerebellar ataxia have been reported. We describe a case of a 53-year-old woman who was readmitted after an adequately treated P. falciparum infection with word-finding difficulties, confusion and tremor. Peripheral blood smears were repeatedly negative for malarial parasites. The clinical features best fitted a PMNS. Because of the severity of the syndrome she was treated with high-dose prednisone. She recovered completely. The possibility of ADEM is also discussed. Aetiology of these syndromes is still unknown, but it could be mediated by an immunological mechanism. PMNS or ADEM must be considered when neurological signs and symptoms occur after recovery from a P. falciparum infection.

Cochleovestibular impairment was evaluated, in relation to age, in a longitudinal follow-up study on a Dutch family with a DFNA9 trait caused by a Pro51Ser mutation in the COCH gene on chromosome 14q12-q13. Fourteen cases were genotyped. The onset age of progressive impairment reported by the mutation carriers was between age 35 and 45 years. Pure-tone thresholds deteriorated by about 2-7 dB per year (mean 3.8 dB per year) in a variable, often asymmetrical, fashion. One mutation carrier developed recurrent episodes of vertigo accompanied by nausea and vomiting, resembling Ménière's disease. Two others developed special susceptibility for motion sickness and appeared to have a hyperactive vestibulo-ocular reflex. More advanced stages of vestibular impairment, i.e. vestibular hyporeflexia and complete vestibular areflexia, were eventually found in a number of cases. DFNA9/COCH should be considered as a possible cause in patients developing combined progressive cochlear and vestibular impairment, or suspected hereditary Ménière-like disease, from around middle age.

Background: Extrahepatic biliary atresia (EHBA), the etiology of which still remains unclear, occurs exclusively in newborns and has recently been simulated in an animal model. It is possible to trigger an EHBA corresponding to the human disease by means of intraperitoneal infection of newborn Balb/c mice with rhesus rotavirus (RRV). The aim of the present study was to determine the conditions and circumstances for inducing biliary atresia in this model focusing on first-line immunological aspects., Methods: Newborn as well as pregnant Balb/c mice were intraperitoneally infected with RRV., Results: The highest incidence of cholestasis (86%) was achieved by infection with 10(6) PFU/ml RRV within the first 12 h postpartum, resulting in EHBA with a lethality of 100%. However, the later the newborn mouse is infected, the less likelihood there is that EHBA is triggered. Additionally, the incidence of biliary atresia in this model depends on the quantity of the virus that is given intraperitoneally. However, the development of biliary atresia is not correlated to the virus in the liver. The antepartum infection of pregnant mice does not induce EHBA in the offspring. Female mice that are immunized against RRV protect their newborns from developing RRV-induced cholestasis and EHBA. This protection is transmitted transplacentally and not by breast milk., Conclusion: It is obvious that a temporary immunological gap is essential for virally induced EHBA. Further studies should focus on specific parameters of the immune system of newborn mice in this biliary atresia model., (Copyright 2001 Academic Press.)

Objective: To analyze the relationship between pure-tone hearing threshold and speech recognition performance in DFNA2/KCNQ4 and DFNA9/COCH, 2 types of high-frequency nonsyndromic hearing impairment., Design: Case series with cross-sectional analysis of phoneme recognition scores related to age and hearing level., Setting: University hospital., Patients: Forty-five members of 4 separate families, all carrying 1 of 3 different mutations in the KCNQ4 gene at the DFNA2 locus (1p34); 42 members of 7 separate families, all carrying the same Pro51Ser mutation in the COCH gene at the DFNA9 locus (14q12-q13)., Results: The deterioration of speech recognition dropped to a 90% score at a higher level of hearing impairment (pure-tone-average at 1, 2, and 4 kHz) in DFNA2-affected patients (65 dB) than in DFNA9-affected patients (46 dB)., Conclusion: At similar levels of hearing impairment, DFNA2/KCNQ4-affected patients showed better speech recognition performance than DFNA9/COCH-affected patients.

Objective: To describe the decline of vestibulocochlear function in a man with vestibulocochlear dysfunction caused by a Pro51Ser mutation within the COCH gene on chromosome 14q12-13 (DFNA9)., Methods: A follow-up of more than 15 years was performed in a single case. Clinical investigations were supplemented by oculomotor, vestibular, and auditory tests., Results: A 50-year-old man had had progressive sensorineural hearing loss and dysequilibrium for 15 years; he had been asymptomatic at the age of 35 years. He suffered from instability in the dark, head movement-dependent oscillopsia, paroxysmal positional vertigo, and vertigo with and without nausea. Hearing impairment started unilaterally, predominantly in the high frequencies. He also reported tinnitus. Disease progressed to severe bilateral high-frequency hearing impairment and vestibular areflexia. Fluctuation of vestibulocochlear function was documented and mentioned by the patient., Conclusions: Our patient proved to suffer from an autosomal dominant vestibulocochlear disorder caused by a COCH gene mutation. The remarkable medical history has some features in common with Meniere disease; however, there are also different clinical and neurophysiological features. In the family, phenotypic variability is present.

Complaints on cognitive functioning are often reported in patients suffering from whiplash syndrome, although objective neuropsychological test results do not always support these. In addition, radiological abnormalities and anatomical lesions are found only in a minority of these patients. This has led to a controversy about its existence in the literature. In this systematic review, the results of 22 neuropsychological studies on whiplash were quantitatively analyzed, focusing on working memory, attention, immediate and delayed recall, visuomotor tracking, and cognitive flexibility. Our findings suggest that a consistent overall pattern of cognitive dysfunction can be demonstrated after whiplash injury through neuropsychological testing, both compared to healthy and to asymptomatic controls. Six months after the accident, improvement is found in working memory, attention, immediate recall, and visuomotor tracking. The results are discussed in the light of recent findings on the effect of cerebral dysfunction, malingering, pain-related factors, and the role of coping strategies and posttraumatic stress on neuropsychological test performance.

Two patients with spinal arachnoiditis following subarachnoid haemorrhage are described. A complete spinal block was seen at the mid thoracic level with characteristics of spinal arachnoiditis. Only one patient had severe symptoms. Both patients were treated conservatively. Signs and symptoms diminished in time. A review of the literature is given and the aetiology is discussed.

Poliovirus type 1 was isolated from an immunodeficient patient 4 days after onset of paresis (IS1) and after 5.5 years of prolonged enteral virus replication (IS2). Antigenic characterization revealed that IS1 was Sabin 1-like, whereas IS2 reacted like poliovirus 1 Mahoney. Complete genomic sequencing demonstrated the phylogenetic relationship (94.9% identity) of IS1 and IS2, which differed from the most closely related Sabin 1 by 5.4 and 8.3%, respectively. Both isolates had revertant-like mutations at nucleotides 480 and 6203. Deduced amino acid sequences indicated significant changes between IS1 and IS2 at the neutralizing antigenic site 1. Prolonged enteral replication, evolution, and shedding of poliovirus by immunodeficient patients should be considered in the poliovirus eradication strategy., (Copyright 1999 Academic Press.)

A pregnant Somalian woman aged 28 years, in the Netherlands for the last five years, suffered from a progressive hemiparesis, epilepsia, behavioural problems and low fever. Brain MRI showed multiple lesions with contrast enhancement. Extensive serologic and parasitologic tests on serum and cerebrospinal fluid did not disclose any cause. A brain biopsy revealed only necrosis, but bacterial culture and polymerase chain reaction (PCR) supplied the diagnosis of 'tuberculosis'. PCR on the cerebrospinal fluid remained negative. In the meantime the chest X-ray showed miliary tuberculosis and a spine MRI thoracic spondylodiscitis and a large paravertebral abscess. Subsequently the patient was treated successfully with tuberculostatic agents. Her healthy child which was born by caesarean section was treated with the tuberculostatic agents as well.

Objectives: Analysis of phenotype-genotype correlation., Study Design: Family study., Methods: Auditory and vestibulo-ocular functions were examined in a Dutch family with autosomal dominantly inherited sensorineural hearing impairment caused by a 208C > T mutation in the COCH gene, located in chromosome 14q12-q13 (DFNA9). Linear regression analysis of individual longitudinal hearing threshold data (n = 11) on age was performed., Results: Fifteen of the 16 genetically affected persons could be evaluated. They all developed hearing and vestibular impairment symptoms--and in many cases also cardiovascular disease--in the fourth to fifth decade. At the low frequencies (0.25-2 kHz), hearing loss started at the age of about 40 years and showed an average annual progression of approximately 3 dB, finally resulting in profound hearing losses. In two exceptional cases, annual progression attained levels of up to 24 dB. At the high frequencies (4-8 kHz), the average threshold increased from about 50 dB at the age of 35 years to about 120 dB at the age of 75 years (which amounts to 1.8 dB annual threshold increase). All affected individuals tested showed normal ocular motor functions. The patients older than 46 years generally showed absence of the vestibulo-ocular reflex, but their cervico-ocular reflex was enhanced compared with normal subjects, whereas those aged 40 to 46 years showed either severe vestibular hyporeflexia or unilateral caloric areflexia., Conclusion: These findings suggest a gradual development of cochleovestibular impairment caused by the new mutation found.

Human corneas are explanted for grafting as late as 72 h after death, for example, from medical examiner cases. Currently, infection of the donor with human immunodeficiency virus (HIV), hepatitis B virus (HBV), and hepatitis C virus (HCV) is excluded in most cornea banks by serological testing of the cadaveric serum only. The reliability of this strategy was investigated by testing paired cadaveric and premortem sera of 33 potential donors. Results were discordant in 17 of 33 donors by at least one assay. Most frequently, HBsAg enzyme-linked immunosorbent assay (ELISA) yielded false-positive results with the cadaveric serum (16 of 33 serum pairs). Virus safety of the graft was affected in a single case, which was HCV antibody negative in the cadaveric serum, but positive in the premortem serum (confirmed by HCV-RIBA strip immunoassay). Forensic DNA profiling by polymerase chain reaction (PCR) of both serum samples confirmed that these were derived from the same individual. In conclusion, the results indicate that serological testing of cadaveric sera is not a reliable method for screening of potential cornea donors, and may not be sufficient for the virus safety of cornea grafts. Therefore, other screening strategies such as detection of viral nucleic acids by PCR should be evaluated.

We analysed a Dutch family with autosomal dominant non-syndromic progressive sensorineural hearing loss and mapped the underlying gene defect by genetic linkage analysis to a 11.0 cM region overlapping the DFNA9 interval on chromosome 14q12-q13. Clinically, the Dutch family differs from the original DFNA9 family by a later age at onset and a more clearly established vestibular impairment. A gene that is highly and specifically expressed in the human fetal cochlea and vestibule, COCH (previously described as Coch5B2 ), was mapped to the DFNA9 critical region. Sequence analysis revealed a 208C-->T mutation in the COCH gene, resulting in a Pro51Ser substitution in the predicted protein in all affected individuals of the family but not in unaffected family members and 200 control individuals. The same mutation was also identified in three apparently unrelated families with a similar phenotype, suggesting the presence of a Dutch founder mutation. The function of COCH is unknown but several characteristics of the protein point to a structural role in the extracellular matrix. The mutant serine at position 51 is situated between cysteines and possibly interferes with proper COCH protein folding or its interaction with extracellular matrix proteins.

Object: Surgical treatment for cubital ulnar nerve compression includes medial epicondylectomy, simple decompression, or anterior transposition (subcutaneous, intramuscular, or submuscular). There is a dearth of prospective randomized studies on which to base guidelines for choosing one operative treatment over another. The authors review the literature on this subject and present their findings., Methods: The authors reviewed the literature from January 1970 to July 1997. Two authors decided independently whether an article should be included for review based on previously formulated inclusion and exclusion criteria. In addition to demographic information, data concerning preoperative status and outcome were extracted. For statistical analyses chi-square and Kruskal-Wallis tests were performed. Irrespective of their preoperative status, patients with simple decompression had the best outcome, whereas those with anterior subcutaneous and submuscular transposition had the worst. If outcome was related to the patient's preoperative status, a significant difference was not found among the various groups for those patients with a preoperative McGowan Grade 2. However, for those with McGowan Grade 3 (severe) symptoms, patients with anterior intramuscular transposition had the best outcome followed by those with simple decompression and anterior submuscular transposition. Statistical analysis was not possible for patients with McGowan Grade 1 because of the small numbers of patients in several treatment modality groups., Conclusions: Formulating a uniform guideline for operative treatment is not possible based on the results of this study. However, the authors believe that support is given to their policy, which is primarily to perform a simple decompression. Its surgical simplicity with preservation of the anatomy, especially the vascularization, and the possibility of rapid postoperative rehabilitation are also taken into consideration. If subluxation is found intraoperatively, anterior transposition is proposed.

Whiplash patients often have physical, psychosomatic and cognitive complaints, although clear neurological and neuropsychological signs of damage are absent. However, in some studies a functional loss of attention was found. In order to compare attentional dysfunctions in whiplash patients with age-matched controls, attention was measured neuropsychologically with the aid of the PASAT, and psychophysiologically with the aid of the prepulse inhibition paradigm. In addition, the reactivity for intense acoustic stimulation was investigated. The POMS and the SCL-90 were used to evaluate psychological and somatic signs. The results showed that whiplash patients (n=24) had lower scores on the PASAT and higher scores on the questionnaires compared to healthy controls (n=21). However, no group differences could be determined on the psychophysiological variables. Furthermore, the PASAT and prepulse inhibition data did not correlate. The lower PASAT scores indicate that whiplash patients seem to have deteriorated divided attention, but an attention deficit as measured with the prepulse inhibition paradigm is not disclosed. Finally, there were no signs of a heightened reactivity of the auditory system, which casts doubts on a presumed heightened sensitivity for sound in whiplash patients.

Camurati-Engelmann disease (CED, MIM # 131300) was diagnosed in a 27-year-old woman, who had pain in her lower legs. There was no history of hearing loss (or CED) in her family. She had been suffering from progressive hearing impairment since childhood. Serial audiograms demonstrated progressive mixed hearing loss from the age of 11 years onwards, for which she underwent successful stapes surgery bilaterally at the age of 13-18 years. Clinical ('juvenile') otosclerosis was found with stapes footplate fixation. Unfortunately, the sensorineural hearing loss component proved to be progressive bilaterally after surgery. At the age of 30 years, she experienced more rapid deterioration of her hearing loss in the right ear and episodes of vertigo. Vestibular examinations demonstrated progressive vestibular impairment 'otospongiotic' lesions with encroachment on the cochlear and vestibular labyrinth were found on high resolution CT scans at the age of 40 years; the internal auditory canal was normal bilaterally. CED may have formed a chance combination with juvenile otosclerosis in our patient, or otosclerosis may be part of the CED phenotype.

Interstitial pneumonia caused by Herpes simplex virus type 1 (HSV-1) is a severe complication of orthotopic liver transplantation (LTX). The records of patients were reviewed who had an LTX at the age of 16 years or older between 1991 and 1994 with a mean follow-up of 21 months (range, 10 to 44 months). Six patients were included who had fever of > 38 degrees C, deterioration of arterial blood gases, radiological evidence of interstitial pneumonia and proof of HSV-1 in bronchoalveolar lavage fluid. All patients were anti-HSV-IgG positive before LTX. All patients were successfully treated with intravenous acyclovir, mechanical ventilation and reduced immunosuppression. Three patients who received cyclosporin A had a rejection which was successfully treated by switching to FK 506. Four patients were discharged in good health. One patient died 36 months after LTX of an unrelated cause. One patient died of urosepsis on postoperative day 139. Acyclovir together with mechanical ventilation and reduced immunosuppression proved to be an effective treatment for HSV-1 pneumonia following LTX.

Vestibular, oculomotor and respiratory tests were performed on 32 patients after whiplash injury caused by a rear-end car collision. Oculomotor functions were generally normal. The cervico-ocular reflex was usually absent or displayed the low gain typical of normal subjects. There was no nystagmic response to static neck torsion. The vestibulo-ocular reflex showed vestibular hyperreactivity (VH) in a significantly large number of cases (n = 17; 53%). The respiratory test results were also typical of the hyperventilation syndrome (HVS) in a significantly large number of cases (n = 12; 38%). The findings of VH and the HVS were not significantly correlated within the patient group. However, the combination of VH and the HVS occurred significantly more often (n = 7; 22%) than could be accounted for by combined false positivity. Most of the significant findings were due to high relative frequencies in the women: 11 out of the 17 women (65%) showed VH, 8 (47%) had the HVS and 5 (29%) showed a combination of VH and the HVS. The findings were not correlated with the patient's age or the time interval between the accident and the examination. VH might have been the result of plastic adaptation to limited head mobility secondary to neck pain. Behavioural and emotional distress might offer alternative explanations for both VH and the HVS.

The interhemispheric subdural hematoma is a relatively uncommon type of subdural hematoma, especially seen in patients with blood clotting disturbances. When its mass becomes sufficiently large, specific neurological abnormalities such as hemiparesis and signs of the falx syndrome are seen. Treatment can consist of conservative observation or craniotomy and is dictated by the clinical course. Conservative management is the treatment of choice for patients without disturbances of consciousness and for patients with stable clinical conditions. Surgical treatment is necessary in patients with progressive deterioration. Three case reports are presented, as well as a review of 64 cases described in the literature. The salient aspects of this clinical entity are discussed.

The role of transcranial Doppler ultrasound (TCD) was studied retrospectively in relation to the management of 73 patients with a subarachnoid hemorrhage due to a ruptured intracranial aneurysms. TCD velocity measurements provided very valuable data to assist scheduling of angiography and delayed surgery. Angiography and surgery were planned significantly sooner in the TCD examined group of patients with TCD velocities indicative for the absence of vasospasm than in the group of patients who did not have TCD examinations. The cerebral circulatory resistance index (R), calculated as: (maximum systolic velocity--end diastolic velocity)/maximum systolic velocity, and changes in R did not predict a change in clinical grade or outcome.

Auditory function tests were performed on 13 patients with myotonic dystrophy (MD). Seven patients had a sensorineural high-frequency hearing loss (HFL) of 30-85 dB at 8 kHz in their pure-tone audiogram, which was in excess of that expected for their age and could be attributed to MD. Their hearing loss resembled 'precocious presbyacusis', i.e. if the patients had been considerably older (or 'functionally' older) than they really were, their HFL could (to some extent) have been attributed to presbyacusis alone. The HFL showed the phenomenon of (genetic) anticipation. Tympanograms and acoustic reflexes were normal. Brainstem auditory evoked potentials (BAEPs) showed a significant increase in the I-V interpeak interval (by 0.35-0.7 ms) and in the III-V interpeak interval (by 0.21-0.67 ms). There was no correlation between the BAEP and the audiometric findings. It should be noted that precocious presbyacusis may be linked to specific gene defects.