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Biomedical subjects

B Johnsen

Publications and source records attributed to B Johnsen.

At least 19 recordsLinked to original sources
PubMed2006

Amplitude, area and duration of the compound muscle action potential change in different ways over the length of the ulnar nerve.

OBJECTIVE: To study physiological changes of the compound muscle action potential (CMAP) obtained from stimulation at different sites over the full length of a motor nerve and to study possible effects of anthropometrical factors. METHODS: Multicentre study of ulnar motor nerve conduction in five segments to Erb's point performed bilaterally on 100 healthy subjects aged 17-83 years. RESULTS: CMAP amplitude decreased linearly with conduction distance (0.31%/cm) from wrist to Erb's point. CMAP area decreased with the square of conduction distance. Decrease in area was smaller than decrease in amplitude especially distally. CMAP duration increased linearly (0.17%/cm). Amplitude decay correlated with age, height and BMI and dispersion correlated with age and height. There were no correlations between area decay and anthropometrical factors. There was no significant inter-examiner variation. CONCLUSIONS: Area decay may be preferred to amplitude decay in the evaluation of conduction block over short segments due to smaller physiological changes and independence of anthropometrical factors. The absence of inter-examiner variation indicates that the results are robust and may be used by other laboratories. SIGNIFICANCE: This study provides knowledge of physiological changes of CMAP parameters that may be of importance in the evaluation of nerve pathology, in particular conduction block.

Action Potentials↗
PubMed2006

Diurnal discrepancies in spectral solar UV radiation measurements.

Unexpected diurnal discrepancies between high-quality spectroradiometers were observed during the 2000 Nordic Ozone Group Intercomparison campaign. The spectral ratios of the irradiances showed a diurnal variation of approximately 2-9%. This cannot be explained by the nonideal angular response of the instruments' input optics in one plane (cosine effect). Instead, by using a radiative transfer model, we show that differences in the angular response in four azimuth planes have the potential to bias the measured data by up to 4.4% (azimuth effect). Other relevant factors are also discussed and quantified and are shown to be significant when diurnal changes in radiation are explained by environmental factors, or when measured data are compared with model or satellite data. Again, intercomparison campaigns have the potential to reveal errors that would otherwise remain undetected.

Journal Article↗
PubMed2004

Hereditary spastic paraplegia with cerebellar ataxia: a complex phenotype associated with a new SPG4 gene mutation.

Complex forms of hereditary spastic paraplegia (HSP) are rare and usually transmitted in an autosomal recessive pattern. A family of four generations with autosomal dominant hereditary spastic paraplegia (AD-HSP) and a complex phenotype with variably expressed co-existing ataxia, dysarthria, unipolar depression, epilepsy, migraine, and cognitive impairment was investigated. Genetic linkage analysis and sequencing of the SPG4 gene was performed and electrophysiologic investigations were carried out in six individuals and positron emission tomography (PET) in one patient. The disease was linked to the SPG4 locus on chromosome 2p as previously reported for pure HSP. Sequence analysis of the SPG4 (spastin) gene identified a novel 1593 C > T (GLN490Stop) mutation leading to premature termination of exon 12 with ensuing truncation of the encoded protein. However, the mutation was only identified in those individuals who were clinically affected by a complex phenotype consisting of HSP and cerebellar ataxia. Other features noted in this kindred including epilepsy, cognitive impairment, depression, and migraine did not segregate with the HSP phenotype or mutation, and therefore the significance of these features to SPG4 is unclear. Electrophysiologic investigation showed increased central conduction time at somatosensory evoked potentials measured from the lower limbs as the only abnormal finding in two affected individuals with the SPG4 mutation. Moreover, PET of one patient showed significantly relatively decreased regional cerebral blood flow in most of the cerebellum. We conclude that this kindred demonstrates a considerable overlap between cerebellar ataxia and spastic paraplegia, emphasizing the marked clinical heterogeneity of HSP associated with spastin mutations.

Adenosine Triphosphatases↗
PubMed2003

Variation in the classification of polyneuropathies among European physicians.

OBJECTIVE: Considerable debate still exists regarding the classification of polyneuropathies (PNPs) into predominantly demyelinating, predominantly axonal loss, mixed or unclassified. This study was designed to determine the variation among physicians in the classification of PNPs by using the European Standardized Telematic tool to Evaluate Electromyography knowledge-based systems and Methods (ESTEEM) multicenter database. METHODS: Seven physicians from 6 laboratories in Europe sent a total of 156 prospectively collected cases of PNP with electromyography (EMG) data including diagnosis (examination diagnosis) to the database. Each physician interpreted the electrophysiological data from all cases (interpretation diagnosis) and a final diagnosis was given at the consensus meetings of the group (consensus diagnosis). RESULTS: Comparison of each physician's examination diagnosis with his/her interpretation diagnosis, i.e. intra-physician variation, showed a change towards less classified PNPs (P < 0.05). Interpretation diagnoses showed large inter-physician variation in the classification of PNPs. The consensus group was more cautious than individual physicians in classifying PNPs as mixed and axonal. The probability of the consensus diagnosis increased with increasing number of abnormal motor and sensory segments tested. CONCLUSIONS: Recognition of variation in classification of PNP as shown in this study and suggesting standards of good clinical practice developed by a consensus group may increase the quality of EMG practice.

Consensus↗
PubMed2001

Medical technology assessment. Electrodiagnosis in motor neuron diseases and amyotrophic lateral sclerosis.

In motor neuron diseases/amyotrophic lateral sclerosis (MND-ALS), electrodiagnostic techniques are essential in supporting the diagnosis and excluding other conditions that clinically resemble MND-ALS. Electrodiagnostic techniques can also monitor disease progression and provide prognostic information. Electromyography has an important role in the diagnosis of MND-ALS, but some drawbacks should be borne in mind. Although internationally accepted electrophysiological criteria have been defined to support MND-ALS diagnosis, differences in different laboratories can raise unexpected difficulties in application of diagnosis criteria. Much work needs to be done to increase standardisation of the electrodiagnosis of MND-ALS in order to improve quality. Differential diagnosis with motor axonal neuropathies may be particularly difficult. EMG is an essential tool for the early diagnosis of MND-ALS, which increases potential benefit of therapeutic interventions. A wide discussion among neurophysiologists from different schools could create a sound consensus on early diagnosis of MND-ALS.

Amyotrophic Lateral Sclerosis↗
PubMed2000

Electrodiagnosis of polyneuropathy.

Electrodiagnostic studies comprising electromyography (EMG) and nerve conduction studies (NCS) are well-established objective methods for the diagnosis, quantification and classification of polyneuropathies (PNP). This paper reviews examination techniques, their pathophysiological interpretation, examination strategies and diagnostic criteria for the diagnosis and classification of a PNP. The routine electrodiagnostic evaluation includes sensory NCSs performed with surface or needle electrodes, motor NCSs, F-wave studies and EMG by qualitative or quantitative techniques. Sensory NCSs and F-wave studies have a high sensitivity in PNPs and the different techniques complement each other. The distinction between a PNP with predominantly axonal loss and a PNP with predominantly demyelination is one of the major aims of the electrophysiological examination. There are, however, large variation in suggested criteria for predominantly demyelination. The degree of slowing in conduction taken to indicate demyelination varies between a decrease of 50 to 30% from mean of controls, distal latency prolongation criteria vary from 35% to 70% of mean of controls, F-wave latency prolongation criteria vary from 120% to 150% of upper limit of controls, and criteria for partial motor conduction block vary from 11 to 50% reduction of CMAP amplitude and/or area between proximal and distal stimulation. Needle EMG studies may be valuable in order to detect and quantify denervation activity, to assess chronicity by an evaluation of the extent of reinnervation, and to evaluate the topographical distribution of changes. It is concluded that electrodiagnostic studies are valuable in patients with suspected PNP and the results may have consequences for prognosis and therapy of individual patients. Large variation in examination techniques, strategies, interpretations and diagnostic criteria have been found among electromyographers and it is suggested that the value of electrodiagnostic studies may be further improved by international standardisation.

Autonomic Nervous System↗
PubMed1999

Variation in diagnostic strategy of the EMG examination--a multicentre study.

OBJECTIVES: In order to improve the universal quality of the EMG examination, knowledge about the variation among physicians is needed. METHODS: The variation among physicians in diagnostic strategy or criteria for diagnosing was analysed from a multicentre database with 940 EMG examinations sampled by seven physicians from six laboratories in Europe. RESULTS: For the whole group of patients as well as for the subgroup of patients with polyneuropathy, variation among physicians in examination techniques, number of examined structures per patient and number of abnormal structures per patient required for a diagnosis was found. Some of the variation may be explained by use of different techniques, which showed differences in sensitivity, while some of the variation may be due to differences in diagnostic strategy and criteria for diagnosing. CONCLUSIONS: The study indicates a need for development and revision of international guidelines for EMG practice although implementation of standards requires caution.

Decision Making↗
PubMed1998

[Effects of sunscreening agents and reactions with ultraviolet radiation].

The use of sunscreens is extensive. During the last few years there have been indications that UV radiation causes breakdown of the sunlight absorbing filters in the sunscreens, i.e. the sunscreens are not photostable. We describe briefly UV propagation in skin, the chemical and physical properties of sunscreens, and how these may react during UV irradiation. We have studied the stability of several sunscreens in vitro. The stability tests were performed by applying a thin film of the sunscreen preparation to the wall of a quartz window, irradiating it with a sun simulator, and measuring the absorbance with spectrophotometry before and during irradiation. The sunscreen agent studied most thoroughly was the UVB filter octyl methoxy cinnamate, but other UVA and UVB filters and some commercial products were also tested. Considerable breakdown of most filters was observed after doses of irradiation equivalent to moderate sun exposure. It can be questioned whether the breakdown products of sunscreens also possess other physical or biological properties. General practitioners should be able to advise their patients on sun protection and the proper use of sunscreens, considering the extensive use of sunscreens and the fact that sunbathing may be a health hazard.

Cinnamates↗
PubMed1998

Can medical audit change electromyographic practice?

Considerable variation in EMG practice has been described previously. Since 1992, 7 clinical neurophysiologists from 6 European countries prospectively collected electromyographic (EMG) examinations. The aim of this study was to examine whether several years of mutual influence among physicians using medical audit resulted in a more uniform EMG practice. We studied whether there was a change in the number of examinations per patient and the techniques used from the first 12 to the last 12 EMG examinations on patients with polyneuropathy. For F-wave studies and motor nerve studies, there was a clear change towards a more uniform practice. For sensory nerve conduction studies and muscle studies, there were only minor changes. With regard to examination techniques, there was a reduction in the use of more time-consuming examination techniques (near-nerve sensory studies and quantitative muscle studies). It seems possible to change the EMG practice of individual physicians by international collaboration and medical audit. However, until now most changes have been minor and large differences in European EMG practices persist. The diversity of practices suggests a need for studies on the optimal application of existing examination techniques.

Electromyography↗
PubMed1997

Opposite base-dependent reactions of a human base excision repair enzyme on DNA containing 7,8-dihydro-8-oxoguanine and abasic sites.

The guanine modification 7,8-dihydro-8-oxoguanine (8-oxoG) is a potent premutagenic lesion formed spontaneously at high frequencies in the genomes of aerobic organisms. We have characterized a human DNA repair glycosylase for 8-oxoG removal, hOGH1 (human yeast OGG1 homologue), by molecular cloning and functional analysis. Expression of the human cDNA in a repair deficient mutator strain of Escherichia coli (fpg mutY) suppressed the spontaneous mutation frequency to almost normal levels. The hOGH1 enzyme was localized to the nucleus in cells transfected by constructs of hOGH1 fused to green fluorescent protein. Enzyme purification yielded a protein of 38 kDa removing both formamidopyrimidines and 8-oxoG from DNA. The enzymatic activities of hOGH1 was analysed on DNA containing single residues of 8-oxoG or abasic sites opposite each of the four normal bases in DNA. Excision of 8-oxoG opposite C was the most efficient and was followed by strand cleavage via beta-elimination. However, significant removal of 8-oxoG from mispairs (8-oxoG: T >G >A) was also demonstrated, but essentially without an associated strand cleavage reaction. Assays with abasic site DNA showed that strand cleavage was indeed dependent on the presence of C in the opposite strand, irrespective of the prior removal of an 8-oxoG residue. It thus appears that strand incisions are made only if repair completion results in correct base insertion, whereas excision from mispairs preserves strand continuity and hence allows for error-free correction by a postreplicational repair mechanism.

Amino Acid Sequence↗
PubMed1995

Comparison of the pattern reversal visual evoked potential mediated by separate cone systems.

With the purpose of recording responses mediated by the 3 cone systems visual evoked potentials (VEPs) were elicited by the reversal of monochromatic checkerboards superimposed upon strong monochromatic backgrounds (yellow, purple and blue-green). The sensitivity to light of various wave lengths were measured as the reciprocal of the intensity necessary to elicit a VEP amplitude of 3 microV. The spectral sensitivity curves based on this VEP amplitude criterion in the presence of blue-green, purple and yellow adaptation showed peak sensitivities in the red, the green and the blue part of the spectrum, respectively. This indicates that the responses reflect separate modulation of the 3 different cone mechanisms. The potentials obtained with yellow adaptation differed from those obtained with purple and blue-green adaptation. The amplitude versus log intensity function was flatter and the latency of the major positive peak was increased by 20-25 msec. Repeated examinations of 4 subjects suggest that the method yields reliable latency measurements of responses mediated by separate cone mechanisms.

Adult↗
PubMed1995

Variation in performance of the EMG examination at six European laboratories.

The quality of the EMG examination might be improved by standardization. However, knowledge about interlaboratory differences in the performance of the EMG examination is a prerequisite for standardization. The aim of this study was to describe differences in EMG techniques used and number of muscles and nerves examined per patient at 6 European EMG laboratories. The EMG results of 595 patients were prospectively sampled. The average number of muscles examined per patient in different disorders varied from laboratory to laboratory, for example from 3.0 to 10.8 muscles in anterior horn cell disorders and from 2.0 to 5.5 muscles in myopathies. The average number of muscles examined by quantitative EMG varied from 0 to 4.3 in anterior horn cell disorders and from 0.0 to 4.5 in myopathies. Also the average number of nerve segments examined per patient varied from laboratory for example from 2.7 to 17.7 for motor segments and from 3.1 to 9.0 for sensory segments in polyneuropathies. The laboratories that used needle electrodes for nerve conduction studies and quantitative analysis of individual motor unit potentials examined a smaller number of muscles and nerves than laboratories using surface electrodes for nerve conduction studies and qualitative EMG studies. The results of this study may have impact on guidelines and examination protocols as well as on quality assurance.

Electromyography↗
PubMed1995

Inter- and intraobserver variation in the interpretation of electromyographic tests.

This study was undertaken with the aim of evaluating inter- and intraobserver variation on the pathophysiological interpretation of individual electromyographic (EMG) tests on muscles and nerve segments. Seven physicians from 6 European EMG laboratories independently interpreted 81 EMG studies comprising 735 muscle tests and 726 tests on nerve segments. Pathophysiological conclusions were inferred from findings of these tests without considering clinical information. For most combinations of findings, both the inter- and intraobserver variations on the interpretation were low, suggesting that common criteria for pathophysiological interpretations were used and that these were used consistently. For some combinations of findings, however, there was disagreement on whether these indicated specific or unspecific pathophysiological changes. In particular disagreement on whether findings indicated demyelination may be of clinical significance. A large part of the intraobserver variation may be explained by a change towards more cautious interpretations during the study for most of the physicians. It is concluded that there is a need to seek for consensus on the pathophysiological interpretation of individual findings and for incentives to ensure consistency in interpretations. The fact that experienced physicians changed their ways to interpret findings during the study suggests that agreement may be improved globally.

Data Interpretation, Statistical↗
PubMed1995

Quality assurance in clinical neurophysiology: the ESTEEM project example.

This paper describes the current status of the utilisation of clinical practice guidelines (protocols) in the ESTEEM project as one form of the clinical quality assurance procedures considered in the project. The performance of electrodiagnostic studies, in terms of which types of conclusions need to be inferred during the examination plan, is briefly described. The main focus of the paper is a description of an European multicentre clinical audit in the field of Clinical Neurophysiology, using a common data-entry protocol for prospective EMG case collection and assessment with the ultimate objective of building up a multicentre reference database of EMG cases.

Computer Communication Networks↗
PubMed1995

ESTEEM: a European telematic project for quality assurance within clinical neurophysiology.

From the prospective multi-center evaluation study of the decision support system KANDID, significant variations were revealed in local epidemiology, local examination techniques, local preferred examination protocols, and local diagnostic criteria among seven European EMG laboratories. A clinical network of eight Electromyographic (EMG) centers has, due to this study, harmonized terminology and the interpretation of EMG examinations. The ESTEEM project has, based on these specifications, developed the EMG-Platform for the local data acquisition, storage, interpretation, and telecommunication between the clinical ESTEEM centers. On this basis, a medical audit and consensus process across Europe is being carried out on a daily basis. This process has shown an impact on diagnostic criteria within the clinical group. This study has illustrates informatics as a necessary prerequisite for the quality assurance of clinical EMG practice.

Computer Systems↗
PubMed1994

Differences in the handling of the EMG examination at seven European laboratories.

This study was undertaken to analyse epidemiological and methodological differences in referral pattern, examination techniques and distribution of diagnoses among different European EMG laboratories. Seven European EMG laboratories filled in questionnaires and sampled 700 cases retrospectively. The use of needle or surface electrodes for nerve studies and the selection of quantitative techniques for muscle studies exhibited considerable variation. The pattern of referral varied with respect to the type of referral source and the neurological expertise of the referring physician. The proportion of patients without any neurophysiological abnormality ranged from 16 to 33%. The 3 most common diagnostic groups were mononeuropathies, polyneuropathies and radiculopathies although a great inter-laboratory variation was found. The proportion of patients with multiple diagnoses varied from 0 to 23% and most of these diagnoses were from a few known combinations. The presence of an inter-laboratory variation suggests that the quality of the EMG examination may be improved by focussing on the use of techniques, strategies and diagnostic criteria.

Electromyography↗
PubMed1994

ESTEEM (European Standardised Telematic Tool to Evaluate EMG Knowledge-Based Systems and Methods): AIM Project A2010.

ESTEEM is an AIM project which is primarily concerned with how to develop, integrate and clinically test knowledge-based systems for electromyography (EMG) in order to get them generally acceptable, useful and applicable into disseminated clinical routines. A medical workstation entitled the 'EMG-Platform' on which different kinds of application modules including KBSs can be interfaced to a kernel is being developed. Accordingly, an EMG communication protocol is being developed. The ESTEEM consortium is composed of a technical specialist group and a group of clinical experts in EMG from seven European countries. The last group has, besides extensive data collection for building up a multicentre EMG database, agreed on a common EMG terminology and a subsequent general EMG data set specification which covers the informatic needs for describing an EMG examination of different 'EMG schools'.

Electromyography↗