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

Peter Joseph Jongen,1,2 Ludovicus G Sinnige,3 Björn M van Geel,4 Freek Verheul,5 Wim l Verhagen,6 Ruud A van der Kruijk,7 Reinoud Haverkamp,8 Hans M Schrijver,9 J Coby Baart,10 Leo H Visser,11 Edo P Arnoldus,12 H Jacobus Gilhuis,13 Paul Pop,14 Monique Booy,15 Wim Lemmens,16 Rogier Donders,16 Anton Kool,17 Esther van Noort17 1Department of Community and Occupational Medicine, University Medical Center Groningen, University Groningen, Groningen, 2MS4 Research Institute, Nijmegen, 3Multiple Sclerosis Centre Leeuwarden, Medical Centre Leeuwarden, Leeuwarden, 4Department of Neurology, Medical Centre Alkmaar, Alkmaar, 5Department of Neurology, Groene Hart Hospital, Gouda, 6Department of Neurology, Canisius Wilhelmina Hospital, Nijmegen, 7Department of Neurology, Slingeland Hospital, Doetinchem, 8Department of Neurology, Zuwe Hofpoort Hospital, Woerden, 9Multiple Sclerosis Centre, Westfries Gasthuis, Hoorn, 10Department of Neurology, Ziekenhuisgroep Twente, Almelo-Hengelo, 11Multiple Sclerosis Centre Midden Brabant, St Elisabeth Hospital, 12Multiple Sclerosis Centre Midden Brabant, Tweesteden Hospital, Tilburg, 13Department of Neurology, Reinier de Graaf Gasthuis, Delft, 14Department of Neurology, Viecuri Medical Centre, Venlo-Venray, 15Multiple Sclerosis Centre, Amphia Hospital, Breda, 16Department for Health Evidence, Radboud University Nijmegen Medical Centre, Nijmegen, 17Curavista bv, Geertruidenberg, the Netherlands Background: There is a growing need to offer persons with multiple sclerosis (PwMS) possibilities for self-management and to integrate multidisciplinary health data. In 2009–2014 we developed a patient-reported outcome based, interactive, web-based program (MSmonitor) for (self-)monitoring, self-management and integrated, multidisciplinary care in MS.Methods: The notions underlying the MSmonitor concept and the program’s elements are described. We analyze MSmonitor’s role in the self-management of fatigue by retrospective comparison of fatigue and health-related quality of life (HRQoL) before and after usage of specific elements of MSmonitor, and by a correlative analysis between frequency of usage and fatigue change.Results: After a step-wise development the program comprises six validated questionnaires: Multiple Sclerosis Impact Profile, Modified Fatigue Impact Scale-5 items (MFIS-5), Hospital Anxiety and Depression Scale, Multiple Sclerosis Quality of Life-54 items, and the 8-item Leeds Multiple Sclerosis Quality of Life (LMSQoL) questionnaires; two inventories: Medication and Adherence Inventory, Miction Inventory; two diaries: Activities Diary, Miction Diary; and two functionalities: e-consult and personal e-logbook. The program is now used in 17 hospitals by 581 PwMS and their neurologists, MS nurses, physical therapists, rehabilitative doctors, continence nurses, and family doctors. Those PwMS (N=105) who used the LMSQoL and MFIS-5 questionnaires at least twice in a period of up to 6 months, showed improved HRQoL (P

Introduction The syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) is a diagnosis made by exclusion. In the literature, different etiological explanations are proposed for HaNDL, including an immune-mediated reaction after a viral infection. Case description We present a case of a 23-year-old woman with several episodes of transient headache, neurological deficits and cerebrospinal fluid lymphocytosis. All diagnostic criteria for the HaNDL syndrome were fulfilled; however, additional cerebrospinal fluid analysis showed a positive polymerase chain reaction (PCR) for human herpes virus type 7 (HHV-7). Discussion The possible role of a (prodromal) viral infection in the etiology of HaNDL is discussed. Also the role of electroencephalography (EEG) recordings is discussed. Serial EEG recordings showed generalized slowing, frontal intermittent rhythmic delta activity (FIRDA) and symmetric triphasic frontal waves with a dilation lag.

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.

In a prospective multi-center observational study, we evaluated the frequency, severity, and impact on activities of daily living (ADL) of adverse effects (AEs) of high-dose intravenous methylprednisolone (IVMP) in relapsing remitting multiple sclerosis (MS) patients with a relapse. Online self-report questionnaires stating IVMP's most common AEs were completed at baseline, the 2nd day of treatment, and 1 day and 1 week after treatment. Eighty-five patients were included, 66 completed the baseline questionnaire, and 59 completed at least one post-baseline questionnaire. Patients reported on average 4 (median) AEs; two (3.4 %) reported no AE. Most frequent was change in taste (61 %), facial flushing (61 %), sick/stomach pain (53 %), sleep disturbance (44 %), appetite change (37 %), agitation (36 %), and behavioral changes (36 %). Of all AEs, 34.3 % were severe and 37.9 % impacted on ADL. A 3-day course resulted in 4 (median) AEs and a 5-day course in 7. All patients with high disease impact had two or more AEs, compared with 79 % of those with low impact (p < 0.01). Of patients with high disability, 45 % had severe AEs, compared with 16 % of those with low disability. Severe central nervous system (CNS)-related AEs occurred two times more frequently in patients with high disease impact, and two-and-a-half times more frequently in patients with high disability. Therefore, in virtually all patients, high-dose IVMP leads to AEs, with about one of three AEs being severe with impact on ADL. Patients with high disease impact or high disability may experience more (severe) AEs, due to a higher occurrence of severe CNS-related AEs.

The primary aim of our study was to determine the extent of vestibular dysfunction in patients with Parkinson's disease (PD). Our secondary aim was to determine if vestibular dysfunction in PD is a risk factor for falling. The tertiary aim was to determine both the extent of vestibular dysfunction and if this dysfunction is a risk factor for falling in patients with atypical parkinsonism (AP). Twenty-five healthy subjects, 30 PD patients and 14 AP patients were matched for age and gender in a case-control study design. All subjects underwent clinical neurological and neurotological assessments, cervical and ocular vestibular evoked myogenic potentials (VEMPs), brainstem auditory evoked potentials (BAEPs), subjective visual vertical measurements, and videonystagmography with caloric and rotatory chair stimulation. Ninety per cent of PD patients (27 of 30) and all 14 AP patients had signs of vestibular dysfunction on laboratory examinations. The evoked potential (VEMPs and BAEPs) test results of PD patients showed significant prolongation of the p13, n1 and interpeak III-V latencies on the symptomatic brainstem side (0.003 ≤  P ≤ 0.019) compared with healthy subjects. Also, vestibular testing abnormalities were correlated with an increased risk for falling when fallers among PD and AP patients were compared with the non-fallers (P ≤ 0.001). To conclude, vestibular dysfunction on vestibular laboratory testing is highly prevalent in both PD and AP patients compared with healthy subjects, and is associated with an increased risk for falling., (© 2016 Federation of European Neuroscience Societies and John Wiley & Sons Ltd.)

The static subjective visual vertical (SVV) was assessed in 24 healthy volunteers with different preset angles (i.e., 10, 20, and 30 degrees), and in 20 other volunteers, the static SVV was tested and retested 1 week later. The static SVV results are influenced by the side of the preset angle (Wilcoxon test, p ≤ 0.001), but not by the preset angle deviation. The test-retest static SVV outcomes are stable at a group level; however, they show statistically relevant variability at an individual level (-0.240 ≤ intraclass correlation coefficient [ICC] ≤ 0.508). A robust static SVV protocol is described in this paper.

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.

Patients with bilateral vestibular loss have balance problems in darkness, but maintain spatial orientation rather effectively in the light. It has been suggested that these patients compensate for vestibular cues by relying on extravestibular signals, including visual and somatosensory cues, and integrating them with internal beliefs. How this integration comes about is unknown, but recent literature suggests the healthy brain remaps the various signals into a task-dependent reference frame, thereby weighting them according to their reliability. In this paper, we examined this account in six patients with bilateral vestibular a-reflexia, and compared them to six age-matched healthy controls. Subjects had to report the orientation of their body relative to a reference orientation or the orientation of a flashed luminous line relative to the gravitational vertical, by means of a two-alternative-forced-choice response. We tested both groups psychometrically in upright position (0°) and 90° sideways roll tilt. Perception of body tilt was unbiased in both patients and controls. Response variability, which was larger for 90° tilt, did not differ between groups, indicating that body somatosensory cues have tilt-dependent uncertainty. Perception of the visual vertical was unbiased when upright, but showed systematic undercompensation at 90° tilt. Variability, which was larger for 90° tilt than upright, did not differ between patients and controls. Our results suggest that extravestibular signals substitute for vestibular input in patients' perception of spatial orientation. This is in line with the current status of rehabilitation programs in acute vestibular patients, targeting at recognizing body somatosensory signals as a reliable replacement for vestibular loss., (© 2015 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of the American Physiological Society and The Physiological Society.)

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.

Introduction: We tested the hypothesis that a bifid median nerve predisposes to development of carpal tunnel syndrome (CTS) and investigated differences in electrophysiological findings and outcome., Methods: A total of 259 consecutive patients with clinically defined CTS were included and investigated clinically, electrophysiologically, and ultrasonographically. Fifty-four healthy asymptomatic volunteers were investigated ultrasonographically., Results: The prevalence of bifid median nerves is equal in patients with CTS and controls. Electrophysiological and ultrasonographic abnormalities are more pronounced in patients with non-bifid median nerves. Some outcome data are better in patients with non-bifid median nerves, but others do not show significant differences., Conclusions: A bifid median nerve is not an independent risk factor for development of CTS. Some of our data suggest outcome after surgical decompression to be different, but others do not. The surgical technique in these patients may therefore have to be reevaluated., (© 2014 Wiley Periodicals, Inc.)

Background: Patients with complaints of carpal tunnel syndrome (CTS) with signs and symptoms not exclusively confined to the median nerve territory, but otherwise fulfilling the clinical criteria may erroneously be withheld from therapy., Methods: One hundred and twenty one patients who fulfilled the clinical criteria for the diagnosis of CTS with signs and symptoms restricted to the median nerve territory (group A) and 91 patients without this restriction (group B) were included in a prospective cohort study. All patients fulfilled electrodiagnostic criteria of CTS. Outcome was determined after 7 to 9 months by means of Symptom Severity Score (SSS) and Functional Status Score (FSS) according to Levine and a patient satisfaction questionnaire., Results: Response rates were 81.8% (group A) and 82.4% (group B). All patients in group B had sensory symptoms involving digit 5. There were no significant differences in improvement of SSS, FSS and patient satisfaction scores between groups after treatment., Conclusion: CTS patients with characteristic sensory signs and symptoms not exclusively restricted to the median nerve innervated area should be treated in the same manner as patients with CTS symptoms restricted to the median nerve innervated area and should therefore not be withheld from surgical treatment., (Copyright © 2014 Elsevier B.V. All rights reserved.)

In schwannomatosis, germline SMARCB1 mutations predispose to the development of multiple schwannomas, but not vestibular schwannomas. Many of these are missense or splice-site mutations or in-frame deletions, which are presumed to result in the synthesis of altered SMARCB1 proteins. However, also nonsense and frameshift mutations, which are characteristic for rhabdoid tumors and are predicted to result in the absence of SMARCB1 protein via nonsense-mediated mRNA decay, have been reported in schwannomatosis patients. We investigated the consequences of four of the latter mutations, i.e. c.30delC, c.34C>T, c.38delA, and c.46A>T, all in SMARCB1-exon 1. We could demonstrate for the c.30delC and c.34C>T mutations that the respective mRNAs were still present in the schwannomas of the patients. We hypothesized that these were prevented from degradation by translation reinitiation at the AUG codon encoding methionine at position 27 of the SMARCB1 protein. To test this, we expressed the mutations in MON cells, rhabdoid cells without endogenous SMARCB1 protein, and found that all four resulted in synthesis of the N-terminally truncated protein. Mutation of the reinitiation methionine codon into a valine codon prevented synthesis of the truncated protein, thereby confirming its identity. Immunohistochemistry with a SMARCB1 antibody revealed a mosaic staining pattern in schwannomas of the patients with the c.30delC and c.34C>T mutations. Our findings support the concept that, in contrast to the complete absence of SMARCB1 expression in rhabdoid tumors, altered SMARCB1 proteins with modified activity and reduced (mosaic) expression are formed in the schwannomas of schwannomatosis patients with a germline SMARCB1 mutation.

Purpose: The aim of this prospectively conducted study was to compare the diagnostic accuracy of onset versus peak latency measurements of sensory nerve action potentials in electrodiagnostic studies in diagnosing carpal tunnel syndrome., Methods: In 156 consecutive patients with clinically defined carpal tunnel syndrome, standardized nerve conduction studies (DIG1, DIG4, PALM3) were performed. Both onset and peak latency were measured. Sensitivity was calculated using the clinical diagnosis as golden standard. Bland-Altman plots were constructed to assess the agreement for quantitative measurements. Overall agreement, positive and negative percent agreement, and Kappa coefficient were computed., Results: The Bland-Altman plots, positive and negative percent agreement show good overall agreement. The kappa coefficient was 0.850, 0.847, and 0.815 for DIG1, DIG4, and PALM3, respectively., Conclusions: Onset and peak latencies used in electrodiagnostic tests show a good overall agreement in confirming the clinical diagnosis of carpal tunnel syndrome. Because onset latency measurement represents nerve conduction velocity of the fastest conducting fibers, the use of onset latencies is recommend. In case of uncontrollable stimulus artifacts, peak latencies may be used instead.

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.)

Objective: The aims of this study were to compare electrodiagnostic (EDX) confirmation of clinical diagnosis of carpal tunnel syndrome (CTS) with ultrasonography (US), using a new set of normal values taking wrist circumference of subjects into account, and to determine whether EDX examination can be replaced by US to confirm CTS., Design: A prospective cohort of 156 patients with idiopathic CTS underwent US and EDX studies. Upper levels of normal cross-sectional area of the median nerve were established by taking wrist circumference into account and using linear regression equations., Results: Of the selected patients, 83.3% met the EDX criteria for CTS. The findings from the US were normal in 67 (42.9%) of 156 patients, and within this group, the findings from the EDX were abnormal in 44 patients (65.7%). Of 89 patients with abnormal findings from the US, only 3 patients had normal findings from the EDX., Conclusions: US cannot replace EDX for confirmation of clinical diagnosis of CTS. However, an abnormal US test result has a high positive predictive value for abnormal EDX result in clinically defined CTS. US might reveal relevant anatomic information preoperatively that rarely has a direct influence on treatment management of patients with CTS. US testing, taking morphometric data into account, does not have the same diagnostic value as EDX does in confirming CTS.

Objective: To test our hypothesis that comparing the sensory nerve conduction velocity of the median nerve across the wrist with that of the forearm is more sensitive than comparing it with that of the palm in the electrodiagnostic confirmation of carpal tunnel syndrome (CTS)., Methods: One hundred and fifty seven consecutive patients with clinically defined CTS were prospectively included and electrophysiologically examined. Antidromic nerve conduction velocities were measured in 3 segments of the median nerve: forearm, wrist, and palm. Differences and ratios in nerve conduction velocities were computed between the forearm and wrist and between the palm and wrist segments., Results: Comparing the median nerve conduction velocities of the forearm with the wrist segment provides a greater sensitivity (79.6% and 82.8% for the second and third digit, respectively) than comparing the palm with the wrist segment (65.6% and 65.0%). Applying the ratio leads to slightly higher sensitivities for both comparisons., Conclusions: The modified segmental palmar test is a sensitive, robust and easily applicable method in diagnosing CTS., Significance: We recommend to use the median nerve sensory conduction velocity in the forearm as a reference in the segmental palmar test instead of that in the palm., (Copyright © 2012 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.)

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.

Introduction: In up to 30% of patients with carpal tunnel syndrome (CTS), the cross-sectional area (CSA) of the median nerve may not be enlarged. We hypothesize that this could be the result of secondary atrophy of the nerve in severe CTS. The aim of this study was to measure the ultrasonographic CSA of the median nerve at the wrist in patients with severe CTS., Methods: In 14 consecutive patients with clinically and electrophysiologically defined severe CTS, the CSA of the median nerve was measured and compared with that of control subjects., Results: CSA of the median nerve exceeded the upper limit of normal in the majority of patients with severe CTS., Conclusions: Atrophy of the median nerve in severe CTS does not explain negative ultrasonographic test results. Instead, the CSA of the median nerve is enlarged in most patients with severe CTS., (Copyright © 2011 Wiley Periodicals, Inc.)

Introduction: Ultrasonography of the ulnar nerve has been recommended as a useful additional test in ulnar neuropathy at the elbow (UNE)., Methods: We searched the literature and systemically reviewed all clinical trials in UNE. We also looked for articles about the normal sonoanatomy and specific causes of UNE., Results: Seven of 14 clinical trials in UNE were suitable for further analysis. Ultrasonographic ulnar nerve size measurement appears to be a test with good diagnostic accuracy. The most frequently reported abnormality was an increased cross-sectional area of the ulnar nerve at the elbow. However, several studies had methodological flaws. In addition, the ultrasonographic techniques and study designs differed among the studies. There were a few other uncontrolled studies about the underlying causes of UNE., Discussion: The role of ultrasonography in UNE seems promising but could not be firmly established. More prospective studies are needed, and we make several recommendations for further research., (Copyright © 2011 Wiley Periodicals, Inc.)

A patient is described who previously had daily complaints of angina pectoris, yet no longer experienced chest pain after an ischemic stroke of the right hemisphere, despite several recorded episodes of electrocardiographic changes and an elevation of cardiac enzymes compatible with myocardial ischemia. The cingulate gyrus is involved in spatial attention and neglect, and is, according to positron emission tomography studies, less activated in silent myocardial ischemia. We suggest that this patient was not aware of the angina because of putamen ischemia and secondary disturbed projection to the cingulate gyrus and no longer felt or experienced this referred pain., (Copyright © 2011 S. Karger AG, Basel.)

Optokinetic nystagmus (OKN) responses (stimuli 40°/s and 60°/s) were evaluated in 121 patients with vestibular areflexia (VA) and were compared with a control group of 99 control subjects matched by age. The mean response levels were significantly higher in the VA group than in the control group: 1.7°/s at 40°/s stimulation, and 4.4°/s at 60°/s. The VA group showed a significantly wider scattering and greater variances and, as a group, they exhibited higher OKN gains than the control subjects. We suggest that the higher gain of OKN responses in VA patients can be attributed to an increased efficiency in signal processing by the cortical optokinetic system. This enhancement may be similar to the enhancement which, in healthy subjects, is produced by "optokinetic training".

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.

Objective: The purpose of this study was to determine the independent contribution of visuospatial hemineglect to impaired postural control in the acute phase (<2 weeks) of stroke compared with other possible clinical and biological determinants., Methods: This study was conducted in 4 hospitals in the mid-east region of the Netherlands. A total of 78 consecutive patients with a first-ever acute supratentorial stroke was included. Functional balance was measured with the Trunk Impairment Scale, the Trunk Control Test, the Berg Balance Scale, and the Functional Ambulation Categories. Visuospatial hemineglect was assessed by means of an asymmetry index obtained from the Behavioral Inattention Test. The Motricity Index, vibration threshold, sustained attention, and the presence of hemianopia were registered as other possible clinical determinants. Stepwise backward multiple linear regression analysis was performed introducing all selected clinical determinants as well as age and poststroke time as possible biological determinants., Results: Hemineglect was present in 17 patients (21.8%). The groups with and without hemineglect were different for gender and the proportion of right hemisphere strokes, but not for age, type of stroke, or poststroke time. Neglect patients had on average lower scores on all functional balance tests as well as on the clinical assessments. Multivariate linear regression showed that, besides hemineglect, only muscle strength and age independently contributed to impaired balance explaining 65% to 72% of variance of the selected outcomes., Conclusion: This study showed that hemineglect independently contributes to impaired postural control in the acute phase of stroke.

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.

Objectives: Recently, ultrasonography of the ulnar nerve has been introduced to confirm the diagnosis of ulnar neuropathy at the elbow. The reported diameters appear to be small when compared to those known from surgery (=actual diameter). The actual diameter is compared with the diameter on ultrasonography., Patients and Methods: Nine patients that were scheduled for decompression of the ulnar nerve at the elbow underwent an ultrasonographic examination preoperatively and intraoperatively. Longitudinal and transversal sonographical scans were made. The maximal diameters of the ulnar nerve at ultrasound were compared with the maximal diameter of the ulnar nerve intraoperatively., Results: Pre- and intraoperatively on the longitudinal scans, the maximal diameter is smaller than the actual maximal diameter of the ulnar nerve. The difference was 3.7+/-1.0 mm (p<0.001) 3.0+/-1.1 mm (p<0.001), respectively. The same was true on the transverse scans: 1.5+/-1.3 mm (p=0.008) versus 1.1+/-0.6 mm (p=0.001)., Conclusion: The diameter of the ulnar nerve as determined sonographically suggesting ulnar neuropathy does not correspond with the anatomically determined actual diameter. The maximal diameter can be best estimated with transverse scans.

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.

Prostate cancer can be complicated by spontaneous intracranial haematoma. In the acute setting, an intracranial haematoma can appear isodense on CT scanning, especially if a coagulopathy is present. Diffuse intravascular coagulation (DIC) can be the underlying pathophysiological mechanism that leads to spontaneous bleeding. It is important to recognize the possibility of DIC in cancer patients, since it has important therapeutical implications that may be life saving.

Seven missense mutations and one in-frame deletion mutation have been reported in the coagulation factor C homology (COCH) gene, causing the adult-onset, progressive sensorineural hearing loss and vestibular disorder at the DFNA9 locus. Prevalence of COCH mutations worldwide is unknown, as there is no systematic screening effort for late-onset hearing disorders; however, to date, COCH mutations have been found on four continents and the possibility of COCH playing an important role in presbycusis and disorders of imbalance has been considered. Cochlin (encoded by COCH) has also been shown as a major target antigen for autoimmune sensorineural hearing loss. In this report, we present histopathology, immunohistochemistry and proteomic analyses of inner ear tissues from post-mortem DFNA9 temporal bone samples of an individual from a large Dutch kindred segregating the P51S mutation and adult human unaffected controls, and wild-type (+/+) and Coch null (-/-) knock-out mice. DFNA9 is an inner ear disorder with a unique histopathology showing loss of cellularity and aggregation of abundant homogeneous acellular eosinophilic deposits in the cochlear and vestibular labyrinths, similar to protein aggregation in well-known neurodegenerative disorders. By immunohistochemistry on the DFNA9 temporal bone sections, we have shown cochlin staining of the characteristic cochlear and vestibular deposits, indicating aggregation of cochlin in the same structures in which it is normally expressed. Proteomic analysis identified cochlin as the most abundant protein in mouse and human cochleae. The high-level expression and stability of cochlin in the inner ear, even in the absence and severe atrophy of the fibrocytes that normally express COCH, are shown through these studies and further elucidate the pathobiologic events occurring in DFNA9 leading to hearing loss and vestibular dysfunction.

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.

Objectives: To analyze cochleovestibular impairment features in P51S COCH mutation carriers (n = 22) in a new, large Dutch family and to compare the results to those obtained in previously identified similar mutation carriers (n = 52). To evaluate age-related features between progressive hearing and vestibular impairment of all mutation carriers (n = 74)., Study Design: Family study., Methods: Regression analysis was performed in relation to age to outline the development of hearing thresholds, speech recognition scores, and vestibulo-ocular reflex time constant as the key vestibular response parameter., Results: Pure tone thresholds, phoneme recognition scores, and vestibular responses of the mutation carriers in the new family were essentially similar to those previously established in all other mutation carriers. Hearing started to deteriorate in all mutation carriers from 43 years of age onwards, whereas deterioration of vestibular function started from age 34., Conclusion: Vestibular impairment starts earlier, progresses more rapidly, and, eventually, is more complete than hearing impairment in P51S COCH mutation carriers.

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.

Objective: To characterize and distinguish the types of sensorineural hearing impairment (SNHI) that occur in hereditary motor and sensory neuropathy Type 1a (HMSN-1a) and hereditary neuropathy with liability to pressure palsies (HNPP), which are caused by deletion or frameshift mutation., Study Design: Prospective study., Setting: Ambulatory patients in a university hospital., Patients: Twelve patients with HMSN-1a due to a duplication of the PMP22 gene on chromosome 17p11.2, 16 patients with HNPP due to the common PMP22 deletion (HNPP del), and 11 HNPP patients with a frame shift mutation (heterozygous PMP22 G-insertion) (HNPP mut), all confirmed by molecular genetic analysis., Interventions: Pure-tone audiograms and speech audiograms were obtained., Main Outcome Measures: Results of cross-sectional analysis comprising linear regression of hearing threshold on age., Results: Pure-tone audiograms showed mild to moderate SNHI, predominant at the low and the high frequencies. SNHI showed significant progression by approximately 0.4 dB per year at 0.25 to 4 kHz and up to 1 to 2 dB per year at 4 to 8 kHz. Patients with HMSN-1a had substantial, presumably congenital, SNHI but did not show significant progression beyond presbyacusis. Patients with HNPP showed postnatal onset at age 11 years with progression of SNHI in excess of presbyacusis by 0.4 dB per year. All three types of neuropathy showed normal speech recognition., Conclusions: All three types of neuropathy showed SNHI with normal speech recognition. HMSN-1a showed stable SNHI without progression beyond presbyacusis. HNPP showed progression beyond presbyacusis with postnatal onset. The differences in SNHI may be explained by the differences in PMP22 expression. The progressive SNHI in HNPP might be explained by the liability for exogenous factors associated with this disorder.

Objective: The main objective of this study was to compare the clinical outcome of participants treated by simple decompression (SD) of the ulnar nerve versus anterior subcutaneous transposition (AST)., Methods: A prospective randomized controlled study was performed. Three hundred forty participants were referred to our institution between March 1999 and July 2002. One hundred fifty-two patients met the inclusion criteria and were randomized into two surgical groups: 75 were assigned to SD, and 77 were assigned to AST. Participants were followed for 1 year after surgery. The main outcome measure was clinical outcome 1 year after surgery., Results: An excellent or good result was obtained in 49 of 75 participants who underwent SD and in 54 of 77 participants undergoing AST. The difference was not statistically significant. However, the complication rate was statistically lower in the SD group (9.6%) compared with the AST group (31.1%) (risk ratio, 0.32; 95% confidence interval, 0.14-0.69). Duration of symptoms, (sub)luxation of the ulnar nerve, and severity of the complaints did not influence outcome., Conclusion: Surgery for ulnar neuropathy at the elbow is effective. The outcomes of SD and AST are equivalent, except for the complication rate. Because the intervention is simpler and associated with fewer complications, SD is advised, even in the presence of (sub)luxation.

Objective: A randomized controlled trial was reported recently, in which simple decompression and anterior subcutaneous transposition were compared for treatment of ulnaropathy at the elbow. Clinically, both surgical options seem to be equally effective. The objective of this study is to compare the costs, from a societal standpoint, of simple decompression versus anterior subcutaneous transposition in Euros., Methods: Published and unpublished data from a randomized controlled trial that compared simple decompression and anterior subcutaneous transposition were used. The investigators tabulated the actual costs incurred for professional fees, use of the operating room, duration of sick leave, reintervention, and treatment of complications directly related to the surgical treatment., Results: The total costs per group and per patient were statistically significantly less for those treated with simple decompression. The total median costs per patient were 1124 Euros for simple decompression and 2730 Euros for anterior subcutaneous transposition. The main difference was in the costs related to sick leave, which is significantly shorter for simple decompression. There also was a statistically significant difference in operation time in favor of simple decompression. A remarkable finding was that paid workers in both treatment groups returned to work much later than unpaid workers., Conclusion: Although clinically equally effective, simple decompression was associated with lower cost than anterior subcutaneous transposition for the treatment of ulnar neuropathy at the elbow. Compared with anterior subcutaneous transposition, we recommend simple decompression for the treatment of idiopathic ulnar neuropathy at the elbow.

Background: Symptomatic brain metastases from prostatic carcinoma are rare (0.05% to 0.5%)., Case Report: A 70-year-old man presented with a homonymous hemianopsia due to brain metastatic prostatic carcinoma shortly before becoming symptomatic of prostatic disease. CT and MRI of the brain showed a tumour deep in the right hemisphere near the thalamus and involving the optic radiation., Results: Routine haematological and biochemical tests were normal. The prostate specific antigen level was low on two separate occasions. The prostatic and brain tumours showed identical appearances, namely of a poorly differentiated adenocarcinoma with neuroendocrine differentiation (small cell carcinoma)., Conclusion: A literature review suggests that small cell carcinoma of the prostate is more likely to spread to the brain compared to adenocarcinoma and that brain metastases indicate a poor prognosis. The prostate gland should be remembered as a possible cause of brain metastases and that a normal serum prostate specific antigen does not exclude this diagnosis.

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.

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.