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Biomedical subjects

N E Gilhus

Publications and source records attributed to N E Gilhus.

At least 19 recordsLinked to original sources

Polio survivors--well educated and hard working.

243 patients were diagnosed with acute poliomyelitis (polio) in Western Norway between 1950 and 1954; 186 were paralytic and 57 non-paralytic. This study examines how polio influenced their education, employment, profession, annual income, marital status and energy for leisure activities. 149 of the patients identified were alive and 98 of the matched controls responded to a questionnaire. Education length did not differ between acute paralytic polio patients, acute non-paralytic polio patients and controls. Fifty percent of the patients with residual weakness and 77 % of the patients with normal muscle power were employed, against 73 % of the controls (P=0.014). A higher proportion of patients without motor deficits had manual work than those with weakness or controls (P=0.002). There was no significant association between severity of weakness and education, employment and profession. Physical ability had been an important factor for the choice of education and profession for all the polio patients, but not for controls (P < 0.001). Annual income did not differ significantly between patients and controls. Residual weakness increased the chance of being single (P=0.023), although as many as 79% had married. 53 % of the patients with weakness claimed that fatigue prevented hobbies, compared wich 31% of the other patients and only 16% of the controls (P < 0.001). There was no significant association between severity of weakness and fatigue. In conclusion, the polio patients are generally well educated, provide their own income and marry. However, their polio has influenced choice of education and profession, and polio patients with persisting weakness differ from controls and polio patients without motor deficits regarding employment and marital status.

Aged↗

Autoimmunity against the ryanodine receptor in myasthenia gravis.

Some myasthenia gravis (MG) patients have antibodies against skeletal muscle antigens in addition to the acetylcholine receptor (AChR). A major antigen for these antibodies is the Ca2+ release channel of the sarcoplasmic reticulum the ryanodine receptor (RyR). These antibodies are found mainly in MG patients with a thymoma MG and correlate with severe MG symptoms. The antibodies recognize a region near the N-terminus on the RyR, which seems to be of importance for RyR regulation. The antibodies cause allosteric inhibition of RyR function in vitro, inhibiting Ca2+ release from sarcoplasmic reticulum.

Animals↗

[Patients with cluster headache in a neurological department].

BACKGROUND: Cluster headache is a devastating disorder which needs intensive drug treatment in the cluster periods. MATERIAL AND METHODS: All patients hospitalised for cluster headache (n = 37) at the Department of Neurology, Haukeland University Hospital 1988-1998 were studied. Data were retrieved from patient files and all patients were asked to fill in a postal questionnaire, which were returned by 27 (73%). RESULTS: Median age was 47 years, range 22-78 years. 28 patients were male. Ten of the patients had chronic cluster headache, all men. 20 out of 27 patients who answered the questionnaire used sumatriptan for acute attacks, 15 of them as subcutaneous injection. 17 patients had tried oxygen inhalation during an attack. Ten of the patients had tried prednisone as prophylactic treatment during a cluster, half of them with effect. Only two patients had tried verapamil and four had tried lithium. None of the patients had ever used valproate as drug prophylaxis. 18 of the patients smoked more than five cigarettes per day. INTERPRETATION: The treatment of cluster headache patients is at present suboptimal, both regarding symptomatic and prophylactic drugs.

Adult↗

Complement activation by titin and ryanodine receptor autoantibodies in myasthenia gravis. A study of IgG subclasses and clinical correlations.

To elucidate the mechanism of immune damage caused by titin and ryanodine receptor (RyR) autoantibodies in myasthenia gravis (MG), we studied the complement-activating capacity and the IgG subclass distribution of these autoantibodies in sera from 49 MG patients. Complement activation occurred in 38 out of 49 titin antibody positive sera, and in 14 out of 21 RyR antibody positive sera. The titin antibodies occurred only in the IgG 1 and IgG 4 subclasses, whereas the RyR antibodies occurred in all four IgG subclasses but with IgG 1 predominance. Complement-activating RyR antibodies occurred with higher frequency in sera of thymoma MG than of late-onset MG. RyR IgG 1 antibodies occurred more often in severe MG than in mild and moderate disease groups. Mean total IgG and IgG 1 titin and RyR antibody titers fell during long-time patient observation together with an improvement of the MG symptoms.

Adult↗

The severity of myasthenia gravis correlates with the serum concentration of titin and ryanodine receptor antibodies.

BACKGROUND: Myasthenia gravis (MG) is caused by autoantibodies to the acetylcholine receptor (AChR). Non-AChR muscle autoantibodies are present in many MG serum samples, mainly from patients with thymoma or late-onset MG. The exact relationship between MG severity and several non-AChR muscle antibodies is unknown. OBJECTIVE: To study the correlation between the severity of MG and the concentration of antibodies against striated muscle tissue sections, titin, citric acid antigen, ryanodine receptor, and AChR. SETTING: The severity of MG was graded in 146 consecutive patients with MG, and their serum samples were tested for the presence of autoantibodies. Ten patients who were titin antibody positive were observed in longitudinal follow-up. RESULTS: No significant difference was found in MG severity between late-onset and thymoma MG. Titin, citric acid antigen, and ryanodine receptor antibodies occurred significantly more often among patients with severe MG than among patients with less severe disease. Changes in MG severity correlated with changes in titin antibody titer in the individual patient. Titin antibodies showed a better longitudinal correlation with disease severity than the AChR antibodies. CONCLUSIONS: Non-AChR muscle autoantibodies occurred more frequently in severe MG regardless of MG subgroup. Thymoma per se does not generate a more severe MG. It may well be the presence of a humoral immune response to non-AChR muscle antigens such as titin, citric acid antigen, and ryanodine receptor that leads to a severe disease, not the presence of thymoma or a late age of onset. These antibodies can serve as important prognostic markers in MG regardless of the presence of thymoma.

Adult↗

Autoantibodies in thymoma-associated myasthenia gravis with myositis or neuromyotonia.

BACKGROUND: About 50% of patients with thymoma have paraneoplastic myasthenia gravis (MG). Myositis and myocarditis or neuromyotonia (NMT) will also develop in some. Patients with thymoma-associated MG produce autoantibodies to a variety of neuromuscular antigens, particularly acetylcholine receptor (AChR), titin, skeletal muscle calcium release channel (ryanodine receptor [RyR]), and voltage-gated potassium channels (VGKC). OBJECTIVE: To examine whether neuromuscular autoantibodies in patients with thymoma correlate with specific clinical syndromes. METHODS: Serum and plasma samples from 19 patients with thymoma-associated MG, of whom 5 had myositis and 6 had NMT, underwent testing for antibodies to AChR, titin, RyR, and VGKC. RESULTS: Antibodies to AChR and titin were found in 19 and 17 patients, respectively. Antibodies to RyR correlated with the presence of myositis (P = .03); they were found in all 5 patients with myositis and in only 1 patient with NMT, but also in 4 of 8 patients with neither disease. Antibodies to VGKC were found in 4 patients with NMT, 1 of 3 patients undergoing testing for myositis, and 2 of 7 patients undergoing testing with neither comorbidity. Presence of RyR antibodies correlated with high levels of titin antibodies. CONCLUSIONS: The results appear to distinguish partially between 3 groups of patients with thymoma-associated MG: the first with RyR antibodies and myositis or myocarditis, the second with NMT without RyR antibodies, and the third without RyR antibodies, myositis, or NMT. Differences in the thymoma may underlie these pathologic associations.

Adult↗

Muscle autoantibodies in subgroups of myasthenia gravis patients.

Myasthenia gravis (MG) is caused by autoantibodies to the acetylcholine receptor (AChR), but several other muscle autoantibodies have also been identified in patient sera. We studied muscle autoantibodies against AChR, striated muscle tissue sections (SH), titin, citric acid antigen (CA), and ryanodine receptor (RyR) in sera from 146 consecutive MG patients to evaluate whether a single test or several tests together can predict a thymoma. The MG patients were divided into five subgroups; ocular MG, early-onset MG (< 50 years), late-onset MG (> 50 years), MG with thymoma, and AChR antibody negative MG. AChR, SH, titin, CA, and RyR antibodies were detected in 85%, 34%, 34%, 25%, and 14% of the MG patients, respectively. For thymoma MG, AChR, SH, titin, CA, and RyR antibodies were detected in 100%, 75%, 95%, 70%, and 70% respectively. SH, titin, CA, RyR antibodies, and computed tomography of the anterior mediastinum have similar sensitivity for thymoma MG. The specificity of RyR, titin, CA, and SH antibodies for thymoma was 70%, 39%, 38%, and 31%, respectively, which is significantly higher for RyR antibodies than for the others. No single muscle antibody assay can predict a thymoma, and a combination of several antibody assays is preferred, although RyR antibody testing alone showed 70% sensitivity and specificity for thymoma MG. SH and CA antibodies provided only little additional information.

Adult↗

EFNS task force report: a questionnaire-based survey on the service provision and quality assurance for determination of diagnostic autoantibody tests in European neuroimmunology centres. European Federation of Neurological Societies.

Autoantibodies to a wide variety of neural components are frequently sought in the sera of patients with neurological diseases suspected to have an antibody-associated autoimmune basis. Variations in assay methodology and availability are likely to exist throughout European diagnostic immunology centres, and interlaboratory discrepancies in performance for some assays have been reported. The availability of quality assurance is largely unknown. In this questionnaire-based EFNS task force, all 18 national representatives of the Neuroimmunology Panel within the EFNS were invited to estimate the service provision within their country; 12 panel members responded. From these responses, it emerged that a range of assays are being performed throughout European centres, involving over 20 separate antigens, using a broad array of immunodetection techniques. With the exception of the estimation of anti-AChR antibodies for the diagnosis of myasthenia gravis, no systematic quality assurance schemes are available, this being conducted on an ad hoc basis, or not at all. Since quality is a central component of assay sensitivity and specificity, we conclude that there is an urgent need to introduce pan-European quality assurance schemes, based on provision of positive and negative test sera from a central source, and in which all neuroimmunology laboratories should participate.

Autoantibodies↗

[Specialized positions, time allocation and clinical research at the Haukeland hospital].

An aim for Norwegian health care is to improve clinical medical research. Special positions for junior doctors are designated for research, quality control projects or subspecialization. In addition, four hours per week for all doctors at Haukeland University Hospital have recently been allocated to theoretical study. A questionnaire was sent to the heads of all 27 departments of Haukeland University Hospital to survey the use of these opportunities for medical research. There were 27 junior doctor positions for clinical research in 12 different departments, constituting 4% of all positions for doctors at the hospital. 15 departments, including several large ones, did not have any junior doctor positions for clinical research. For 15 of the 27 positions, approximately 50% of the working time was used for research with the goal of publishing in international referee-based journals. Four positions were used for clinical subspecialization, and eight positions were used entirely for routine clinical work. 14 department heads would give high priority to a new position for clinical research. Only 11 department heads claimed that the hours scheduled for theoretical work had improved research to some degree. There was general agreement that other aspects of department organisation was more important for promotion of research. Clinical research positions for junior doctors should be used for research, but even in an ambitious university hospital they are not fully used for such work. Most of the department heads have a clear ambition to improve the conditions for research in their departments.

Education, Medical, Continuing↗

TNFA and TNFB polymorphisms in myasthenia gravis.

BACKGROUND: Tumor necrosis factor (TNF) alpha and TNF-beta are proinflammatory cytokines thought to be involved in the pathogenesis of myasthenia gravis (MG). OBJECTIVE: To examine whether TNF polymorphisms are associated with MG, MG subgroups, and the presence of titin and ryanodine-receptor antibodies. PATIENTS AND METHODS: We did genotyping on 30 patients with MG and 92 healthy blood donors for 2 biallelic TNFA polymorphisms (G to A at positions -238 and -308) and 1 TNFB polymorphism (NcoI digestive site) using methods based on the polymerase chain reaction. RESULTS: Patients with thymoma were typically homozygous for both the TNFA*T1 and the TNFB*2 alleles, but patients having an early onset of MG without thymoma were carriers of the TNFA*T2 and TNFB*1 alleles. Patients without thymoma who had the titin antibody had the same high frequency of TNFA*T1 and TNFB*2 as patients with thymoma, whereas patients without the titin antibody carried the same allele, TNFA*T2 and TNFB*1, regardless of age and thymic disease. No association was found with acetylcholine-receptor levels or disease severity for any of the TNFA or TNFB polymorphisms. CONCLUSION: Patients having MG, including those with thymoma, who have the titin antibody are most often homozygous for the TNFA*T1 and TNFB*2 alleles, whereas the presence of the TNFA*T2 and TNFB*1 alleles correlates with early-onset MG and the absence of titin antibodies.

Adult↗

A new way of building a database of EEG findings.

Whereas computer-based electroencephalography (EEG) is widely applied, the EEG interpretations are usually not stored in a way that favours exploitation of modern computer technology. This paper reports an EEG description system facilitating categorization of EEG data in a computerized database. The system interactively communicates with the digital EEG system and also with the general patient administrative system. The main new quality of this system is the methods for data input and automatic data retrieval from several systems, rather than the establishment of a database of EEG data itself. The EEGs are visually analysed and categorized. Manually marked EEG events are automatically transferred to the database and such events as well as defined electrode positions within these epochs are directly linked to their corresponding descriptions. The database is updated without demand for filling in the events in the database in a second operation. Thereby, the EEG interpreter builds the database while analysing the EEG. This system provides an improved accessibility of EEG data for clinical, normative, educational and scientific use.

Brain↗

[Wound botulism in heroin addiction].

Botulism is a rare disease which usually is caused by preformed botulinum toxin in food. However, this article describes a case of wound botulism in a 29-year-old male heroin addict who developed progressive diplopia, dysphagia and proximal weakness of skeletal limb muscles. He needed mechanical ventilation for two weeks. The clinical diagnosis of botulism was supported by neurophysiological tests. Assays for detection of botulinum toxin and Clostridium botulinum were negative. The patient had not eaten any contaminated food the last two weeks before symptoms appeared, but he had multiple contaminated skin wounds. After treatment with botulinum antitoxins and antibiotics he gradually recovered, and six weeks later he was discharged from hospital in good condition. To the best of our knowledge this is the first case of wound botulism reported in Norway.

Adult↗

[The annual neurological conferences 1990-97--extensive scientific presentation for Norwegian neurologists].

324 free oral presentations were put forward during the Norwegian annual neurology conferences in 1990-1997. The lead authors of 216 presentations (67%) were employed at university hospitals; 108 authors were in employment at other hospitals or institutions. 159 lead authors (49%) were specializing in neurology and these candidates became increasingly active as presenters during the period in question. International collaboration occurred in 27 of the presentations (8%), nine of the presenters working with colleagues in the USA and seven with colleagues in Sweden. The presentations were classified as either mainly clinical, with 275 presentations (85%), or mainly basal science, with 31 presentations. In the remaining 18 presentations administrative or historic-literary topics were discussed. The disorders most frequently dealt with were epilepsy (88 presentations), cerebrovascular disorders (52 presentations), and autoimmune disorders (47 presentations). There was an increasing trend towards the latter two topics during the conference period. In conclusion, this survey shows neurology to be an expansive specialty with a focus on active development and research.

Humans↗