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  • 251. Sosnoff, Jacob J.
    et al.
    Vaillancourt, David E.
    Larsson, Lars
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Newell, Karl M.
    Coherence of EMG activity and single motor unit discharge patterns in human rhythmical force production.2005In: Behavioural Brain Research, ISSN 0166-4328, E-ISSN 1872-7549, Vol. 158, no 2, p. 301-310Article in journal (Refereed)
    Abstract [en]

    The purpose of this study was to examine the modulation of the motor neuronal pool as a function of task dynamics. Specifically, we investigated the effects of task frequency on the single motor unit discharge pattern, electromyogram (EMG) activity and effector force output. Myoelectric activity and effector force were recorded while young adults isometrically abducted their first dorsal interosseus at five sinusoidal targets (0.5 Hz, 1 Hz, 2 Hz, 3Hz and 4 Hz) and at two force levels (5% and 25% maximum voluntary contraction (MVC)). Individual motor unit spike trains were isolated from the EMG. Auto-spectral and coherence analyses were performed on the force output, EMG and motor unit spike trains. The frequency of maximal coherence between the EMG and force output closely corresponded to the target frequency in all conditions. There was a broadband distribution of power with multiple peaks in the EMG and motor unit spectrums in the 0.5 Hz and 1Hz targets. However, the EMG and motor unit spectrums in the 2 Hz, 3Hz and 4Hz targets were characterized by an increasingly narrower band of activity with one dominant peak that closely corresponded to the target. There is high coherence between EMG output and target force frequency, but the relative contribution of the fast and slow neuromuscular bands are differentially influenced by the task frequency. The rhythmical organization of neuromuscular output in the 0.5 Hz task is relatively broadband and similar to that shown previously for constant level force output. The frequency structure of neuromuscular organization becomes increasingly more narrowband as the frequency of the target increases (2–4 Hz). The modulation of the motor neuronal pool is adaptive and depends on the relative contribution of feedback and feedforward control processes, which are driven by the task demands.

  • 252. Stening, Kent D
    et al.
    Berg, Göran
    Hammar, Mats
    Voster, Helene
    Eriksson, Olle
    Amandusson, Åsa
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Blomqvist, Anders
    Influence of Estrogen Levels on Thermal Perception, Pain Thresholds, and Pain Tolerance: Studies on Women Undergoing in Vitro Fertilization.2012In: Journal of Pain, ISSN 1526-5900, E-ISSN 1528-8447, Vol. 13, no 5, p. 459-466Article in journal (Refereed)
    Abstract [en]

    We examined the relationship between estrogen and pain in women undergoing in vitro fertilization (IVF). Quantitative sensory tests (QST) were performed twice during the IVF-regimen: once during hormonal down-regulation and once during hormonal up-regulation. A group of healthy men and a group of women using monophasic contraceptives were also examined, to control for session-to-session effects. Among the women undergoing IVF, serum 17β-estradiol levels differed strongly between treatments as expected, and increased from 65.7 (SD = 26) pmol/L during the down-regulation phase, to 5,188 (SD = 2,524) pmol/L during the up-regulation phase. Significant outcomes in the QST were only seen for temperature perception thresholds (1.7°C versus 2.2°C; P = .003) and cold pain threshold (11.5°C versus 14.5°C; P = .04). A similar change in cold pain threshold was also seen in the 2 control groups, however, and statistical analysis suggested that this change was due to a session-to-session effect rather than being the result of hormonal modulation. Heat pain thresholds, heat tolerance, pressure pain thresholds, and the cold pressor test showed no significant differences between sessions. These data demonstrate that pain perception and pain thresholds in healthy women show little, if any, changes even with major variations in serum estradiol levels. PERSPECTIVE: This study shows that pain perception and tolerance in women undergoing in vitro fertilization do not vary, despite the dramatic changes in 17β-estradiol levels induced by the treatment regimen. The result thus suggests that in humans, contrary to experimental animals, changes in estrogen levels have little influence on pain sensitivity.

  • 253. Stening, Kent D.
    et al.
    Eriksson, Olle
    Henriksson, Karl G.
    Brynhildsen, Jan
    Lindh-Åstrand, Lotta
    Berg, Göran
    Hammar, Mats
    Amandusson, Åsa
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Blomqvist, Anders
    Hormonal replacement therapy does not affect self-estimated pain or experimental pain responses in post-menopausal women suffering from fibromyalgia: a double-blind, randomized, placebo-controlled trial2011In: Rheumatology, ISSN 1462-0324, E-ISSN 1462-0332, Vol. 50, no 3, p. 544-551Article in journal (Refereed)
    Abstract [en]

    Objectives. FM is a condition that preferentially affects women. Sex hormones, and in particular oestrogens, have been shown to affect pain processing and pain sensitivity, and oestrogen deficit has been considered a potentially promoting factor for FM. However, the effects of oestrogen treatment in patients suffering from FM have not been studied. Here, we examined the effect of transdermal oestrogen substitution treatment on experimental as well as self-estimated pain in women suffering from FM. Methods. Twenty-nine post-menopausal women were randomized to either 8 weeks of treatment with transdermal 17β-oestradiol (50 µg/day) or placebo according to a double-blind protocol. A self-estimation of pain, a set of quantitative sensory tests measuring thresholds to temperature, thermal pain, cold pain and pressure pain, and a cold pressor test were performed on three occasions: before treatment, after 8 weeks of treatment and 20 weeks after cessation of treatment. Results. Hormonal replacement treatment significantly increased serum oestradiol levels as expected (P < 0.01). However, no differences in self-estimated pain were seen between treatment and placebo groups, nor were there any differences between the two groups regarding the results of the quantitative sensory tests or the cold pressor test at any of the examined time points. Conclusion. Eight weeks of transdermal oestradiol treatment does not influence perceived pain, pain thresholds or pain tolerance as compared with placebo treatment in post-menopausal women suffering from FM. Trial registration. ClinicalTrials.gov Registration; http://www.clinicaltrials.gov; NCT01087593.

  • 254. Stigsdotter-Broman, Lina
    et al.
    Olsson, Ingrid
    Flink, Roland
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Rydenhag, Bertil
    Malmgren, Kristina
    Long-term follow-up after callosotomy: A prospective, population based, observational study2014In: Epilepsia, ISSN 0013-9580, E-ISSN 1528-1167, Vol. 55, no 2, p. 316-321Article in journal (Refereed)
    Abstract [en]

    Objective

    Analyze the long-term outcome of callosotomies with regard to seizure types and frequencies and antiepileptic drug treatment.

    Methods

    This longitudinal observational study is based on data from the prospective Swedish National Epilepsy Surgery Register. Thirty-one patients had undergone callosotomy in Sweden 1995-2007 and had been followed for 2 and 5 or 10years after surgery. Data on their seizure types and frequencies, associated impairments, and use of antiepileptic drugs have been analyzed.

    Results

    The median total number of seizures per patient and month was reduced from 195 before surgery to 110 twoyears after surgery and 90 at the long-term follow-up (5 or 10years). The corresponding figures for drop attacks (tonic or atonic) were 190 before surgery, 100 2years after surgery, and 20 at the long-term follow-up. Ten (56%) of the 18 patients with drop attacks were free from drop attacks at long-term follow-up. Three of the remaining eight patients had a reduction of >75%. At long-term follow-up, four were off medication. Only one of the 31 patients had no neurologic impairment.

    Significance

    The present population-based, prospective observational study shows that the corpus callosotomy reduces seizure frequency effectively and sustainably over the years. Most improvement was seen in drop attacks. The improvement in seizure frequency over time shown in this study suggests that callosotomy should be considered at an early age in children with intractable epilepsy and traumatizing drop attacks.

  • 255.
    Stålberg, Erik
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Jitter analysis with concentric needle electrodes2013In: Myasthenia Gravis and Related Disorders / [ed] Wolfe, GI; Meriggioli, MN; Ciafaloni, E; Ruff, RL, 2013, Vol. 1274, no 1, p. 77-85Conference paper (Refereed)
    Abstract [en]

    Because of certain restrictions in medical praxis, reusable materials are only allowed in some countries. This also applies to electrodes for electromyography; the special single-fiber electromyography electrode must be replaced. This article gives some details of the possibilities of using an alternative-a small concentric needle electrode. Practical hints, reference values, and the application in diagnostic work for myasthenia gravis are summarized.

  • 256.
    Stålberg, Erik
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Macro electromyography: An update2011In: Muscle and Nerve, ISSN 0148-639X, E-ISSN 1097-4598, Vol. 44, no 2, p. 292-302Article in journal (Refereed)
    Abstract [en]

    The macro electromyography method was developed in the 1980s. Since then, technical modifications have been made, and a number of conditions have been explored. This study is a methodological introduction and an update of findings in some nerve-muscle disorders. The spike component of a motor unit potential (MUP) recorded by a concentric or monopolar needle electromyography (EMG) electrode is generated primarily by fibers within 1-2 mm of the needle recording area. Given that a MUP's typical anatomical reach is 5-15 mm in diameter, it follows that conventional EMG is unable to record activity from the entire motor unit. Such information could promote understanding of muscle in health and disease. Macro EMG, with its large recording area, appears to provide this information by recording the activity from most of the fibers in a given motor unit. The value of combining macro EMG with single-fiber EMG and conventional EMG recordings is discussed.

  • 257.
    Stålberg, Erik
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Kouyoumdjian, João
    Sanders, Donald
    Reference values in concentric needle electrode studies2013In: Clinical Neurophysiology, ISSN 1388-2457, E-ISSN 1872-8952, Vol. 124, no 6, p. 1255-1256Article in journal (Refereed)
  • 258.
    Stålberg, Erik
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Sanders, Donald B.
    Bibliographical item: Joze Trontelj; June 1, 1939 To December 9, 2013 Obituary, in Muscle & Nerve, vol. 49, issue 3, pages 313-3142014Other (Other academic)
  • 259.
    Stålberg, Erik
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Sanders, Donald B
    Ali, Sajjad
    Cooray, Gerald
    Leonardis, Lea
    Löseth, Sissel
    Machado, Flavia
    Maldonado, Antonio
    Martinez-Aparicio, Carmen
    Sandberg, Arne
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Smith, Benn
    Widenfalk, Johan
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Aris Kouyoumdjian, João
    Reference values for jitter recorded by concentric needle electrodes in healthy controls: A multicenter study.2016In: Muscle and Nerve, ISSN 0148-639X, E-ISSN 1097-4598, Vol. 53, no 3, p. 351-362Article in journal (Refereed)
    Abstract [en]

    INTRODUCTION: The aim of this study was to create reference values for jitter measured with concentric needle electrodes.

    METHODS: Operators worldwide contributed recordings from orbicularis oculi (OO), frontalis (FR), and extensor digitorum (ED) muscles in healthy controls. Criteria for acceptable signal quality were agreed upon in advance. Fifteen or 20 recordings of acceptable quality from each muscle were required for voluntary and electrical stimulation recordings, respectively.

    RESULTS: Recordings from 59 to 92 subjects were obtained for each muscle and activation type. Outlier limits for mean consecutive difference and individual jitter data for voluntary activation were: OO, 31 and 45 µs; FR, 28 and 38 µs; ED, 30 and 43 µs; and for electrical stimulation they were: OO, 27 and 36 µs; FR, 21 and 28 µs; ED, 24 and 35 µs.

    CONCLUSION: Reference jitter values from concentric needle electrode recordings were developed from signals of defined quality while seeking to avoid creating supernormal values.

  • 260.
    Stålberg, Erik
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Sanders, Donald B.
    Duke Univ, Med Ctr, Dept Neurol, Durham, NC 27710 USA.
    Kouyoumdjian, Joao Aris
    Fac Med Sao Jose do Rio Preto, Invest Neuromuscular Lab, BR-15090000 Sao Jose Do Rio Preto, SP, Brazil.
    Pitfalls and errors in measuring jitter2017In: Clinical Neurophysiology, ISSN 1388-2457, E-ISSN 1872-8952, Vol. 128, no 11, p. 2233-2241Article, review/survey (Refereed)
    Abstract [en]

    The safety factor of neuromuscular transmission can be assessed by measuring the neuromuscular jitter, which reflects the time variability of processes in the motor end-plate. Jitter is increased in any condition with disturbed end-plate function, such as myasthenic conditions and ongoing reinnervation. Jitter is increasingly being measured with concentric needle (CN) electrodes, which are more prone to artefacts than single fiber EMG recordings. The objective of this review is to identify and demonstrate pitfalls that can be seen with CN jitter measurements, made with both voluntary activation and electrical stimulation. With voluntary activation, errors are caused by poor signal quality; inappropriate time reference points on the signal; an irregular firing rate; and signals with dual latencies, i.e., "flip-flop." With electrical stimulation, additional errors result from insufficient stimulation intensity; from abrupt change in firing rate; and from axon reflexes. Many pitfalls cannot be avoided during recording and can only be detected during post-processing. It is critical to be aware of these artefacts when measuring jitter with CN electrodes. (C) 2017 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  • 261.
    Stålberg, Erik V.
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Sanders, Donald B
    Jitter recordings with concentric needle electrodes2009In: Muscle and Nerve, ISSN 0148-639X, E-ISSN 1097-4598, Vol. 40, no 3, p. 331-339Article, review/survey (Refereed)
    Abstract [en]

    Neuromuscular jitter is generally recorded with a single fiber (SF) electromyography (EMG) electrode. Due to concern about using reusable needle electrodes, an acceptable alternative for the SF electrode has been sought. This is a review of the issues involved in using disposable concentric needle (CN) electrodes to measure jitter. Signals recorded with CN electrodes frequently represent the summation of many single fiber action potentials, which will decrease the apparent jitter. The influence of these artifacts on the final result also depends on the analysis method. Reference values obtained with CN electrodes correlate with SF EMG values, but they are a few microseconds lower. Overall results show that the CN method is a good alternative to SFEMG and will facilitate the use of jitter analysis. The results must be interpreted with caution, particularly in borderline cases, but they may be acceptable for clinical use when SF electrodes cannot be used.

  • 262.
    Sundblom, Jimmy
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurology.
    Stålberg, Erik
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Österdahl, Maria
    Rücker, Franz
    Montelius, Maria
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Genetics and Pathology.
    Kalimo, Hannu
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Pathology.
    Nennesmo, Inger
    Islander, Gunilla
    Smits, Anja
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurology.
    Dahl, Niklas
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Genetics and Pathology.
    Melberg, Atle
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurology.
    Bedside diagnosis of rippling muscle disease in CAV3 p.A46T mutation carriers2010In: Muscle and Nerve, ISSN 0148-639X, E-ISSN 1097-4598, Vol. 41, no 6, p. 751-757Article in journal (Refereed)
    Abstract [en]

    Thirty-nine members, ages 1 to 67 years, of a Swedish family with rippling muscle disease (RMD) were investigated to assess genotype-phenotype correlations. Clinical, neurophysiological, and muscle morphological examinations were performed. Genetic analysis was performed in 38 individuals. Twenty-three patients had percussion-induced muscle mounding (PIMM) and percussion-induced rapid contractions (PIRC). Rippling and hyperCKemia were not found in all patients. Weakness was minor or absent. The electromyogram showed absence of electrical activity in ripples and PIMM, and muscle biopsy specimens confirmed caveolin-3 deficiency and absence of caveolae. Genetic analysis revealed a CAV3 c.G136A transition resulting in a p.A46T missense mutation in affected family members. The phenotype in these 23 cases of RMD with this mutation appears to be homogenous, benign, and nonprogressive. The presence of PIMM and PIRC seems to be diagnostic at all ages, whereas the absence of hyperCKemia and rippling does not exclude the diagnosis.

  • 263. Tannheimer, Markus
    et al.
    Tannheimer, Sibylle
    Thomas, Alfred
    Engelhardt, Michael
    Schmidt, Roland
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Auto-PEEP in the therapy of AMS in one person at 4,330 m.2009In: Sleep and Breathing, ISSN 1520-9512, E-ISSN 1522-1709, Vol. 13, no 2, p. 195-199Article in journal (Refereed)
    Abstract [en]

    Background

    The human organism is exposed to considerable hypoxic stress at high altitudes. Our intention was to investigate if a special breathing pattern with expiration against the resistance of pursed lips leads to an elevation in oxygen saturation (SaO2). For the first time ever, oxygen saturation was measured continuosly during the initial situation, during self-performed positive end-expiratory pressure breathing (auto-PEEP) itself, and during observation afterwards.

    Materials and methods

    The investigation was performed on a 33-year-old male suffering from high-altitude illness (Lake Louise Score, 9) after a 4-day rapid ascent from 350 m to 4,330 m during an expedition to Mount McKinley (6,198 m). SaO2 was measured continuously at 4-s intervals. After a rest of 1.5 h in a dorsal recumbent position with a slightly elevated (about 15°) upper body, the patient used a wristwatch to breathe according to a special time pattern (inspiration 2 s, expiration 8 s against the resistance of pursed lips). After 30 min, breathing was then allowed without any restrictions, and the inspiration/expiration ratio was approximately 1:1.

    Results

     

    There was a relatively sharp rise in SaO2 from an average of 62% to 85% within only 5 min after auto-PEEP began. This was followed by a comparable rise to values of 95% at the end of the auto-PEEP period. During normal breathing, SaO2 decreased slowly within half an hour to values of about 70% and remained at this level. The person reported relief in symptoms and no exhaustion. Vertigo—an indication of hypocapnia caused by hyperventilation—was not observed.

    Discussion

    The 30% rise in SaO2 and the improved saturation level after auto-PEEP are remarkable. Elevated intra-thoracal pressure may lead to a reopening of collapsed alveoli in addition to an improved gradient of alveolarcapillary pressure. In addition, a pressure-induced displacement of interstitial fluid resulting in a reduced diffusion distance may lead to improved alveolar-capillary diffusion. This would explain the slower rise in SaO2 after 10 min of auto-PEEP and the elevated level of SaO2 compared to the initial level before auto-PEEP.

    Conclusion

    As a result of the substantial and lasting improvement in SaO2 in combination with relief in AMS symptoms and its easy use, auto-PEEP (30 min every 2 h) can be a useful therapy option in the event of high-altitude-induced hypoxia and AMS.

     

     

     

  • 264.
    Torbergsen, Torberg
    et al.
    Univ Hosp North Norway, Dept Clin Neurophysiol, Tromso, Norway..
    Jurkat-Rott, Karin
    Univ Ulm, Div Neurophysiol, D-89069 Ulm, Germany.;Univ Ulm, Neuromuscular Ctr, D-89069 Ulm, Germany..
    Stalberg, Erik V.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Loseth, Sissel
    Univ Hosp North Norway, Dept Clin Neurophysiol, Tromso, Norway..
    Hodneo, Anne
    Vestfold Cty Hosp, Dept Neurol, Tonsberg, Norway..
    Lehmann-Horn, Frank
    Univ Ulm, Div Neurophysiol, D-89069 Ulm, Germany.;Univ Ulm, Neuromuscular Ctr, D-89069 Ulm, Germany..
    PAINFUL CRAMPS AND GIANT MYOTONIC DISCHARGES IN A FAMILY WITH THE NAV1.4-G1306A MUTATION2015In: Muscle and Nerve, ISSN 0148-639X, E-ISSN 1097-4598, Vol. 52, no 4, p. 680-683Article in journal (Refereed)
    Abstract [en]

    Introduction: Two previously reported Norwegian patients with painful muscle cramps and giant myotonic discharges were genotyped and compared with those of members of 21 families harboring the same mutation. Methods: Using primers specific for SCN4A and CLCN1, the DNA of the Norwegian family members was amplified and bidirectionally sequenced. Clinical and neurophysiological features of other families harboring the same mutation were studied. Results: A G1306A mutation in the Nav1.4 voltage-gated sodium channel of skeletal muscle was identified. This mutation is known to cause myotonia fluctuans. No giant myotonic discharges or painful muscle cramps were found in the other G1306A families. Conclusions: Ephaptic transmission between neighboring muscle fibers may not only cause the unusual size of the myotonic discharges in this family, but also a more severe type of potassium-aggravated myotonia than myotonia fluctuans.

  • 265. Uesugi, Haruo
    et al.
    Sonoo, Masahiro
    Higashihara, Mana
    Stålberg, Erik
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Saito, Hisatoshi
    [Non-invasive quantitative EMG: invention of the "Clustering Index (CI)" method].2012In: Rinshō shinkeigaku = Clinical neurology, ISSN 1882-0654, Vol. 52, no 11, p. 1249-1251Article in journal (Refereed)
    Abstract [en]

    There have been few previous studies trying to evaluate neuromuscular disorders using surface electro myography (SEMG). The greatest obstacle to such an approach must be the difficulty in identifying individual motor unit potentials (MUPs) on the SEMG signal under voluntary contraction because of their dense overlap. We tried to solve this problem by reducing the overlap of MUPs using appropriate electrode setting, as well as by developing a new method of interference pattern analysis. The tibialis anterior muscle was examined in our first study. A new method to analyse SEMG signal, the Clustering Index (CI) method, achieved 100% and 61% sensitivities for neurogenic and myopathic patients, and 97% specificity for control subjects. In the second study, the abductor digiti minimi muscle was examined. Subjects were 29 spinal and bulbar muscular atrophy patients and 27 controls. The CI method was compared with the motor unit number estimation, and the amplitude of compound muscle action potential. As results, the CI method achieved the best sensitivity and among these three techniques. The CI method is a novel, simple, and quantitative analysis method without using any special equipments, and is promising as a non-invasive complement to needle EMG.

  • 266. Uesugi, Haruo
    et al.
    Sonoo, Masahiro
    Stålberg, Erik
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Matsumoto, Kohji
    Higashihara, Mana
    Murashima, Hideharu
    Ugawa, Yoshikazu
    Nagashima, Yu
    Shimizu, Teruo
    Saito, Hisatoshi
    Kanazawa, Ichiro
    "Clustering Index method": A new technique for differentiation between neurogenic and myopathic changes using surface EMG2011In: Clinical Neurophysiology, ISSN 1388-2457, E-ISSN 1872-8952, Vol. 122, no 5, p. 1032-1041Article in journal (Refereed)
    Abstract [en]

    Objective: To establish a non-invasive and quantitative analysis method using single-channel surface EMG (SEMG) for diagnosing neurogenic and myopathic changes.

    Methods: The subjects consisted of 66 healthy controls, 12 patients with neurogenic diseases, and 18 patients with myopathic diseases. The tibialis anterior muscle was examined using a belly to the adjacent bone lead. From each subject, 20-40 signals of 1 s length were collected of various strengths. A new parameter, the "Clustering Index (CI)", was developed to quantify the uneven distribution of the SEMG signal, and was plotted against the SEMG area. The results were expressed as the Z-score of each subject calculated using linear regression from the normative data.

    Results: When +/- 2.5 was used as the cut-off value of the Z-score, the specificity was 95%, whereas the sensitivity was 92% (11/12) and 61% (11/18) for the neurogenic and myopathic patients, respectively. There was no overlap of the Z-score values between the neurogenic and myopathic groups.

    Conclusions: The CI method achieved a reasonably high diagnostic yield in detecting neurogenic or myopathic changes.

    Significance: This is a new simple and quantitative analysis method using SEMG with good reproducibility, and is promising as a non-invasive complement to needle EMG.

  • 267. Verdinelli, C.
    et al.
    Olsson, I
    Edelvik, A.
    Flink, Roland
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Hallbook, T.
    Rydenhag, B.
    Malmgren, K.
    Long-Term Follow-Up after Hemispherotomy from the Patients' Perspective2014In: Epilepsia, ISSN 0013-9580, E-ISSN 1528-1167, Vol. 55, p. 177-177Article in journal (Other academic)
  • 268.
    Wang, Shuhui
    et al.
    George Washington Univ, Dept Neurol, Washington, DC USA; Capital Med Univ, Beijing Friendship Hosp, Dept Neurol, Beijing, Peoples R China.
    Breskovska, Iva
    George Washington Univ, Dept Neurol, Washington, DC USA.
    Gandhy, Shreya
    George Washington Univ, Dept Neurol, Washington, DC USA.
    Rostedt Punga, Anna
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Guptill, Jeffery T.
    Duke Univ, Med Ctr, Div Neurol, Durham, NC USA.
    Kaminski, Henry J.
    George Washington Univ, Dept Neurol, Washington, DC USA.
    Advances in autoimmune myasthenia gravis management2018In: Expert Review of Neurotherapeutics, ISSN 1473-7175, E-ISSN 1744-8360, Vol. 18, no 7, p. 573-588Article, review/survey (Refereed)
    Abstract [en]

    Introduction: Myasthenia gravis (MG) is an autoimmune neuromuscular disorder with no cure and conventional treatments limited by significant adverse effects and variable benefit. In the last decade, therapeutic development has expanded based on improved understanding of autoimmunity and financial incentives for drug development in rare disease. Clinical subtypes exist based on age, gender, thymic pathology, autoantibody profile, and other poorly defined factors, such as genetics, complicate development of specific therapies.

    Areas covered: Clinical presentation and pathology vary considerably among patients with some having weakness limited to the ocular muscles and others having profound generalized weakness leading to respiratory insufficiency. MG is an antibody-mediated disorder dependent on autoreactive B cells which require T-cell support. Treatments focus on elimination of circulating autoantibodies or inhibition of effector mechanisms by a broad spectrum of approaches from plasmapheresis to B-cell elimination to complement inhibition.

    Expert commentary: Standard therapies and those under development are disease modifying and not curative. As a rare disease, clinical trials are challenged in patient recruitment. The great interest in development of treatments specific for MG is welcome, but decisions will need to be made to focus on those that offer significant benefits to patients.

  • 269.
    Westerberg, Elisabet
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Environmental Factors of Importance in Myasthenia Gravis: Emphasis on Physical Activity2018Doctoral thesis, comprehensive summary (Other academic)
    Abstract [en]

    Myasthenia Gravis (MG) is an autoimmune disease caused by antibodies directed against proteins at the neuromuscular junction (NMJ). The disturbed neuromuscular transmission induces fatigable weakness in skeletal muscles. The severity of MG ranges from purely ocular symptoms to extensive weakness with, at worst, respiratory failure and need of intensive care. Due to different pathogenic antibodies, as well as varying phenotypes there is a classification into MG subgroups.

    As lifestyle disorders are common conditions they occur also in MG patients. It is unknown whether there are any lifestyle factors associated with MG or MG subgroups. However, medication side effects and disability due to disease could contribute to higher risks for lifestyle related diseases. General treatment guidelines are not suitable to MG patients, since various medications are known to affect the disease negatively and as the tolerance and effects of physical activity in MG is unknown.

    In this thesis the occurrence of lifestyle related environmental factors in MG patients were investigated. MG epidemiology, subgroup differences and the presence of lifestyle related risks in MG patients were evaluated in Jönköping county in Southern Sweden. The findings were compared to a similar evaluation in a region of Estonia. We found that MG patients in the late-onset disease subgroup had a more disadvantageous pattern of life-style related risk factors, with a higher rate of obesity, smoking and physical inactivity and a lower rate of fish consumption in comparison to matched population controls. Furthermore, we found that despite comparable clinical muscle fatigue status, Estonian MG patients subjectively scored fatigue and disease severity higher than Swedish MG patients. More Estonian patients were physically inactive. The tolerance and effects of physical exercise was further evaluated in two intervention studies, where MG patients with stable disease performed a 12-week-training-program according to general exercise recommendations to healthy adults. We found that physical exercise was well tolerated by the MG patients and that they had a beneficial skeletal muscle response to physical exercise.

    List of papers
    1. Diversity in mental fatigue and social profile of patients with myasthenia gravis in two different Northern European countries
    Open this publication in new window or tab >>Diversity in mental fatigue and social profile of patients with myasthenia gravis in two different Northern European countries
    2017 (English)In: Brain and Behavior, ISSN 2162-3279, E-ISSN 2162-3279, Vol. 7, no 4, article id e00653Article in journal (Refereed) Published
    Abstract [en]

    Tntroduction: Self-estimated health can be used for comparison of different diseases between countries. It is important to elaborate on whether disparities in self-estimated health are due to disease-specific parameters or socioeconomic differences. In this study, we aimed at evaluating clinical and social similarities and differences in myasthenia gravis (MG) patients between comparable regions in two Baltic Sea countries, Estonia and Sweden. Methods: This cross-sectional study included southern counties in Sweden and Estonia of comparable size. All patients with a confirmed MG diagnosis were asked to answer two questionnaires including demographic and disease-specific data, lifestyle issues, and mental fatigue (Fatigue Severity Scale [FSS]). Clinical fatigue was assessed objectively through the Quantitative Myasthenia Gravis Score (QMG). Results: Thirty-six of 92 identified patients in Estonia and 40 of 70 identified MG patients in Sweden chose to participate in the study. The demographic characteristics and symptoms reported by the patients were similar. QMG score did not differ; however, the Estonian patients scored their current subjective disease severity significantly higher (5.6 +/- 2.8) compared to the Swedish patients (3.4 +/- 2.3, p=.0005). Estonian patients also had significantly higher FSS scores (5.0 +/- 1.7) than Swedish patients (3.5 +/- 1.6; p=.001). Swedish patients were more active and performed physical activity more regularly (29.1% in Estonia and 74.2% in Sweden, p=.004). Conclusions: Although, the patients had comparable clinical fatigue, Estonian patients evaluated their health state as being more severe and reported more mental fatigue than Swedish patients. These data indicate large regional differences in disease perception of MG, which is important to consider in international studies.

    Place, publisher, year, edition, pages
    WILEY, 2017
    Keywords
    Estonia, fatigue, myasthenia gravis, Sweden
    National Category
    Neurology
    Identifiers
    urn:nbn:se:uu:diva-322219 (URN)10.1002/brb3.653 (DOI)000399452500009 ()28413704 (PubMedID)
    Funder
    Swedish Research Council, VR-523-2014-2048
    Available from: 2017-05-17 Created: 2017-05-17 Last updated: 2018-03-08Bibliographically approved
    2. Lifestyle factors and gender-related differences in clinical subgroups of Myasthenia Gravis in southern Sweden
    Open this publication in new window or tab >>Lifestyle factors and gender-related differences in clinical subgroups of Myasthenia Gravis in southern Sweden
    (English)In: BMC Neurology, ISSN 1471-2377, E-ISSN 1471-2377Article in journal (Refereed) Submitted
    Abstract [en]

    Background: Based on the need for further regional and global epidemiological knowledge on Myasthenia Gravis (MG) and MG subgroups, we conducted a questionnaire-based epidemiological survey in a medium-sized health care region in southern Sweden. The primary aims were to investigate disease-specific items and lifestyle related factors between MG subgroups and matched population controls.

    Methods: All MG patients (n=70) in Jönköping County were invited to participate in a survey, using a standardized disease-specific questionnaire, previously developed by an expert group. This questionnaire contains three parts, including demographic and disease-specific data, lifestyle and health-related aspects as well as co-morbidity and mental fatigue. The patients were clinically evaluated. Four hundred age- and gender matched randomly selected population controls excluding MG were invited to answer the non-disease-specific part of the questionnaire. Statistical tests included student t-test (for parametric data) or Mann–Whitney U test (for nonparametric data) and Chi-square test and Fisher’s exact test for evaluations between MG subgroups. In the case-control analysis conditional logistic regression was used to estimate the adjusted Odds Ratios.

    Results: Forty of the 70 identified MG patients and 188 of the 400 age-and gender matched population controls participated in the study (response rate 57.1%). In the late onset MG (LOMG; N=18) subgroup, the male predominance was higher than previously reported. In the early onset MG (EOMG; N=17) group, time to diagnosis was longer and bulbar weakness was the dominant symptom (65%). EOMG patients had higher fatigue compared to LOMG. Compared to their matched population controls, LOMG patients were obese more often, ate ess fish, smoked more, had a lower educational level and were employed as manual laborers more often. Mental health problems and sickness benefits were more common among MG patients than in controls and MG patients were less regularly doing focused physical activity.

    Conclusions: These findings highlight lifestyle and gender related differences between the EOMG and LOMG subgroups and between MG patients and population controls. Importantly, these lifestyle issues such as cardiovascular risk factors and physical inactivity, as well as mental health problems should be addressed intensively in the clinical follow-up of MG patients.

    Keywords
    Myasthenia Gravis, late onset MG, early onset MG, lifestyle, fatigue
    National Category
    Neurology
    Research subject
    Neurology
    Identifiers
    urn:nbn:se:uu:diva-344665 (URN)
    Funder
    Swedish Research Council, VR-523-2014-2048Futurum - Academy for Health and Care, Jönköping County Council, Sweden, 488501
    Available from: 2018-03-08 Created: 2018-03-08 Last updated: 2018-03-08
    3. Physical exercise in Myasthenia Gravis is safe and improves neuromuscular parameters and physical performance-based measures: A pilot study
    Open this publication in new window or tab >>Physical exercise in Myasthenia Gravis is safe and improves neuromuscular parameters and physical performance-based measures: A pilot study
    Show others...
    2017 (English)In: Muscle and Nerve, ISSN 0148-639X, E-ISSN 1097-4598, Vol. 56, no 2, p. 207-214Article in journal (Refereed) Published
    Abstract [en]

    INTRODUCTION: Due to the shortage of exercise-related research in Myasthenia Gravis (MG), there are no consensus guidelines on physical exercise for MG patients.

    METHODS: In this prospective pilot study, 10 MG patients with mild disease performed supervised aerobic and resistance training twice weekly for 12 weeks. The Myasthenia Gravis Composite (MGC) score, compound motor action potential (CMAP), repetitive nerve stimulation, muscle force, physical performance-based measures, serum levels of interleukin-6, muscle enzymes as well as immuno-microRNAs miR-150-5p and miR-21-5p were assessed before and after the training period.

    RESULTS: Physical exercise was well tolerated, and the MGC score was unchanged. Muscle resistance weights and CMAP amplitudes increased for biceps brachii and rectus femoris muscles, and physical performance-based measures improved. Muscle enzymes remained normal, whereas disease-specific microRNAs miR-150-5p and miR-21-5p were reduced after the training period.

    CONCLUSIONS: We propose that general recommendations regarding physical exercise safely can be applied to well-regulated MG patients.

    National Category
    Neurology
    Identifiers
    urn:nbn:se:uu:diva-314678 (URN)10.1002/mus.25493 (DOI)000406875000009 ()27935072 (PubMedID)
    Funder
    Swedish Research Council, 2014-02048Futurum - Academy for Health and Care, Jönköping County Council, Sweden, 520281, 520411Swedish Society of Medicine, SLS-499271
    Available from: 2017-02-04 Created: 2017-02-04 Last updated: 2018-03-08Bibliographically approved
    4. The impact of physical exercise on neuromuscular function in Myasthenia gravis patients: A single-subject design study
    Open this publication in new window or tab >>The impact of physical exercise on neuromuscular function in Myasthenia gravis patients: A single-subject design study
    Show others...
    2018 (English)In: Medicine (Baltimore, Md.), ISSN 0025-7974, E-ISSN 1536-5964, Vol. 97, no 31, article id e11510Article in journal (Refereed) Published
    Abstract [en]

    There is a need for tailored exercise recommendations to patients with Myasthenia Gravis (MG). A few pilot studies have recently shown that physical exercise in accordance with general recommendations to healthy adults can be applied safely to patients with mild MG symptoms. How physical exercise affects muscle parameters and risk factors for life-style diseases in patients with MG is, however, only poorly known. We evaluated functional skeletal muscle parameters in 11 MG patients, before and after conducting a 12-week supervised physical therapy regimen of aerobic and high-resistance strength training. After the training program, parameters of the proximal leg muscle rectus femoris improved: compound motor action potential (from 4.5 ± 2.6 to 5.3 ± 2.8 mV, p=0.016), isometric muscle force (from 25.2 ± 4.4 to 30.2 ± 3.8 kg; p=0.014) and ultrasound muscle thickness (from 19.6 ± 5.6 to 23.0 ± 3.9 mm, p=0.0098) all increased. Further, physical performance-based measures improved, including the 30-Second Chair Stand Test (median change +2, p=0.0039) as well as the clinical MG composite score (from 3[2-5] to 2 [0-4], p=0.043). These findings indicate that MG patients can improve their functional muscle status as a result of aerobic and high-resistance strength training, especially in proximal leg muscles. This is important knowledge when physical therapy is considered for this patient group, for whom no guidelines on physical exercise currently exist.

    Place, publisher, year, edition, pages
    Lippincott Williams & Wilkins, 2018
    Keywords
    Myasthenia Gravis, physical exercise, neuromuscular, CMAP, resistance training
    National Category
    Neurology Physiotherapy Sport and Fitness Sciences
    Research subject
    Neurology
    Identifiers
    urn:nbn:se:uu:diva-344666 (URN)10.1097/MD.0000000000011510 (DOI)000442259200025 ()30075515 (PubMedID)
    Funder
    Swedish Research Council, VR-523-2014-2048
    Note

    Title in thesis list of papers: The impact of physical exercise on functional muscle measures in Myasthenia Gravis patients – a single subject design study

    Available from: 2018-03-08 Created: 2018-03-08 Last updated: 2018-10-30Bibliographically approved
  • 270.
    Westerberg, Elisabet
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Landtblom, Anne-Marie
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Landtblom: Neurology.
    Rostedt Punga, Anna
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Rostedt Punga: Clinical Neurophysiology.
    Lifestyle factors and disease-specific differences in subgroups of Swedish Myasthenia Gravis2018In: Acta Neurologica Scandinavica, ISSN 0001-6314, E-ISSN 1600-0404, Vol. 138, no 6, p. 557-565Article in journal (Refereed)
    Abstract [en]

    PURPOSE: To evaluate disease-specific differences between Myasthenia Gravis (MG) subgroups and compare patterns of lifestyle between MG patients and population controls.

    METHODS: All MG patients (n=70) in Jönköping County, Sweden, were invited to answer a disease-specific questionnaire, containing questions about disease-specific data, lifestyle, co-morbidity and mental fatigue. The patients were clinically evaluated. Four hundred age- and gender matched population controls were invited to answer the non-disease-specific part of the questionnaire. Disease-specific issues were compared between MG subgroups. Lifestyle related factors and concomitant conditions were compared to the population controls.

    RESULTS: Forty MG patients and 188 population controls participated in the study. In the late onset MG (LOMG; N=18) subgroup, the male predominance was higher than previously reported. In the early onset MG (EOMG; N=17) subgroup, time to diagnosis was longer, fatigue was higher and bulbar weakness was the dominant symptom (65%). Compared to their matched population controls, LOMG patients were more obese (OR 13.7, p=0.015), ate less fish (OR 4.1, p=0.012), tended to smoke more (OR 4.1, p=0.086) and tended to be employed as manual laborers more often (OR 2.82, p=0.083). Mental health problems and sickness benefits were more common among MG patients than in controls and MG patients were less regularly doing focused physical activity.

    CONCLUSIONS: It is important to consider disease-specific differences when tailoring the management of individual MG patients. There is a need for improved knowledge on how to apply primary and secondary prevention measures to lifestyle disorders in MG patients without risk of deterioration.

  • 271.
    Westerberg, Elisabet
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Landtblom, Anne-Marie
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurology.
    Rostedt Punga, Anna
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Lifestyle factors and gender-related differences in clinical subgroups of Myasthenia Gravis in southern SwedenIn: BMC Neurology, ISSN 1471-2377, E-ISSN 1471-2377Article in journal (Refereed)
    Abstract [en]

    Background: Based on the need for further regional and global epidemiological knowledge on Myasthenia Gravis (MG) and MG subgroups, we conducted a questionnaire-based epidemiological survey in a medium-sized health care region in southern Sweden. The primary aims were to investigate disease-specific items and lifestyle related factors between MG subgroups and matched population controls.

    Methods: All MG patients (n=70) in Jönköping County were invited to participate in a survey, using a standardized disease-specific questionnaire, previously developed by an expert group. This questionnaire contains three parts, including demographic and disease-specific data, lifestyle and health-related aspects as well as co-morbidity and mental fatigue. The patients were clinically evaluated. Four hundred age- and gender matched randomly selected population controls excluding MG were invited to answer the non-disease-specific part of the questionnaire. Statistical tests included student t-test (for parametric data) or Mann–Whitney U test (for nonparametric data) and Chi-square test and Fisher’s exact test for evaluations between MG subgroups. In the case-control analysis conditional logistic regression was used to estimate the adjusted Odds Ratios.

    Results: Forty of the 70 identified MG patients and 188 of the 400 age-and gender matched population controls participated in the study (response rate 57.1%). In the late onset MG (LOMG; N=18) subgroup, the male predominance was higher than previously reported. In the early onset MG (EOMG; N=17) group, time to diagnosis was longer and bulbar weakness was the dominant symptom (65%). EOMG patients had higher fatigue compared to LOMG. Compared to their matched population controls, LOMG patients were obese more often, ate ess fish, smoked more, had a lower educational level and were employed as manual laborers more often. Mental health problems and sickness benefits were more common among MG patients than in controls and MG patients were less regularly doing focused physical activity.

    Conclusions: These findings highlight lifestyle and gender related differences between the EOMG and LOMG subgroups and between MG patients and population controls. Importantly, these lifestyle issues such as cardiovascular risk factors and physical inactivity, as well as mental health problems should be addressed intensively in the clinical follow-up of MG patients.

  • 272.
    Westerberg, Elisabet
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology. Ryhov Cty Hosp, Dept Neurol, Jonkoping, Sweden.
    Molin, Carl Johan
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Lindblad, Ida
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Emtner, Margareta
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Physiotherapy. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Lung- allergy- and sleep research.
    Rostedt Punga, Anna
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Physical exercise in Myasthenia Gravis is safe and improves neuromuscular parameters and physical performance-based measures: A pilot study2017In: Muscle and Nerve, ISSN 0148-639X, E-ISSN 1097-4598, Vol. 56, no 2, p. 207-214Article in journal (Refereed)
    Abstract [en]

    INTRODUCTION: Due to the shortage of exercise-related research in Myasthenia Gravis (MG), there are no consensus guidelines on physical exercise for MG patients.

    METHODS: In this prospective pilot study, 10 MG patients with mild disease performed supervised aerobic and resistance training twice weekly for 12 weeks. The Myasthenia Gravis Composite (MGC) score, compound motor action potential (CMAP), repetitive nerve stimulation, muscle force, physical performance-based measures, serum levels of interleukin-6, muscle enzymes as well as immuno-microRNAs miR-150-5p and miR-21-5p were assessed before and after the training period.

    RESULTS: Physical exercise was well tolerated, and the MGC score was unchanged. Muscle resistance weights and CMAP amplitudes increased for biceps brachii and rectus femoris muscles, and physical performance-based measures improved. Muscle enzymes remained normal, whereas disease-specific microRNAs miR-150-5p and miR-21-5p were reduced after the training period.

    CONCLUSIONS: We propose that general recommendations regarding physical exercise safely can be applied to well-regulated MG patients.

  • 273.
    Westerberg, Elisabet
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Molin, Carl Johan
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Spörndly-Nees, Søren
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Physiotherapy.
    Widenfalk, Johan
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Rostedt Punga, Anna
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    The impact of physical exercise on neuromuscular function in Myasthenia gravis patients: A single-subject design study2018In: Medicine (Baltimore, Md.), ISSN 0025-7974, E-ISSN 1536-5964, Vol. 97, no 31, article id e11510Article in journal (Refereed)
    Abstract [en]

    There is a need for tailored exercise recommendations to patients with Myasthenia Gravis (MG). A few pilot studies have recently shown that physical exercise in accordance with general recommendations to healthy adults can be applied safely to patients with mild MG symptoms. How physical exercise affects muscle parameters and risk factors for life-style diseases in patients with MG is, however, only poorly known. We evaluated functional skeletal muscle parameters in 11 MG patients, before and after conducting a 12-week supervised physical therapy regimen of aerobic and high-resistance strength training. After the training program, parameters of the proximal leg muscle rectus femoris improved: compound motor action potential (from 4.5 ± 2.6 to 5.3 ± 2.8 mV, p=0.016), isometric muscle force (from 25.2 ± 4.4 to 30.2 ± 3.8 kg; p=0.014) and ultrasound muscle thickness (from 19.6 ± 5.6 to 23.0 ± 3.9 mm, p=0.0098) all increased. Further, physical performance-based measures improved, including the 30-Second Chair Stand Test (median change +2, p=0.0039) as well as the clinical MG composite score (from 3[2-5] to 2 [0-4], p=0.043). These findings indicate that MG patients can improve their functional muscle status as a result of aerobic and high-resistance strength training, especially in proximal leg muscles. This is important knowledge when physical therapy is considered for this patient group, for whom no guidelines on physical exercise currently exist.

  • 274. Winbanks, Catherine E.
    et al.
    Chen, Justin L.
    Qian, Hongwei
    Liu, Yingying
    Bernardo, Bianca C.
    Beyer, Claudia
    Watt, Kevin I.
    Thomson, Rachel E.
    Connor, Timothy
    Turner, Bradley J.
    McMullen, Julie R.
    Larsson, Lars
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    McGee, Sean L.
    Harrison, Craig A.
    Gregorevic, Paul
    The bone morphogenetic protein axis is a positive regulator of skeletal muscle mass2013In: Journal of Cell Biology, ISSN 0021-9525, E-ISSN 1540-8140, Vol. 203, no 2, p. 345-357Article in journal (Refereed)
    Abstract [en]

    Although the canonical transforming growth factor. signaling pathway represses skeletal muscle growth and promotes muscle wasting, a role in muscle for the parallel bone morphogenetic protein (BMP) signaling pathway has not been defined. We report, for the first time, that the BMP pathway is a positive regulator of muscle mass. Increasing the expression of BMP7 or the activity of BMP receptors in muscles induced hypertrophy that was dependent on Smad1/5-mediated activation of mTOR signaling. In agreement, we observed that BMP signaling is augmented in models of muscle growth. Importantly, stimulation of BMP signaling is essential for conservation of muscle mass after disruption of the neuromuscular junction. Inhibiting the phosphorylation of Smad1/5 exacerbated denervation-induced muscle atrophy via an HDAC4-myogenin-dependent process, whereas increased BMP-Smad1/5 activity protected muscles from denervation- induced wasting. Our studies highlight a novel role for the BMP signaling pathway in promoting muscle growth and inhibiting muscle wasting, which may have significant implications for the development of therapeutics for neuromuscular disorders.

  • 275. Winbanks, Catherine E.
    et al.
    Chen, Justin L.
    Turner, Bradley J.
    Larsson, Lars
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Harrison, Craig A.
    Gregorevic, Paul
    Bone Morphogenetic Protein (BMP) Signaling Is A Positive Regulator Of Skeletal Muscle Mass2013In: Journal of Gene Medicine, ISSN 1099-498X, E-ISSN 1521-2254, Vol. 15, no 8-9, p. 324-325Article in journal (Other academic)
  • 276. Yu, Fushun
    et al.
    Hedström, Margaretha
    Cristea, Alexander
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Dalén, Nils
    Larsson, Lars
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Effects of ageing and gender on contractile properties in human skeletal muscle and single fibres2007In: Acta Physiologica, ISSN 1748-1708, E-ISSN 1748-1716, Vol. 190, no 3, p. 229-241Article in journal (Refereed)
    Abstract [en]

    Aim: The objective of this study is to improve our understanding of the mechanisms underlying the ageing- and gender-related muscle weakness.

    Methods: Ageing- and gender-related differences in regulation of muscle contraction have been studied in knee-extensor muscles at the whole muscle and single muscle fibre levels in young and old sedentary men and women. In vivo knee-extensor muscle function was measured at slow (30° s−1) and faster (180 ° s−1) speeds of movement. Maximum velocity of unloaded shortening (V0) and maximum force normalized to cross-sectional area (CSA) [specific tension (ST)] were measured in single 'skinned' skeletal muscle fibre segments.

    Results: Significant ageing- and gender-related differences were observed in muscle torque. A 33–55% ageing-related decline (P < 0.001) in maximum torque was observed irrespective of gender. At the single muscle fibre level, the ageing-related decline in knee-extensor muscle function was accompanied by a 20–28% decline in ST in muscle fibres expressing the type I MyHC isoform in both men and women, and a 29% decline in type IIa muscle fibre CSA, but the decreased fast-twitch fibre size was restricted to the men. Furthermore, in both men and women, V0 decreased in muscle cells expressing the type I and IIa MyHC isoforms.

    Conclusion: The present results provide evidence of specific ageing- and gender-related differences in regulation of muscle contraction at the cellular level. It is suggested that these cellular changes have a significant impact on muscle function and the ageing-related motor handicap.

  • 277.
    Zetterberg, Lena
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Physiotheraphy.
    Halvorsen, Kjartan
    Färnstrand, Catarina
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
    Aquilonius, Sten-Magnus
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Neurology.
    Lindmark, Birgitta
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Physiotheraphy.
    Physiotherapy in Cervical Dystonia: Six experimental single-case studies2008In: Physiotherapy Theory and Practice, ISSN 0959-3985, E-ISSN 1532-5040, Vol. 24, no 4, p. 275-290Article in journal (Refereed)
    Abstract [en]

    The aim of the study was to explore the outcome of a physiotherapy program targeted to improve the quality of life of people with cervical dystonia (CD) by reducing pain, improving awareness of postural orientation, increasing muscle strength, and reducing the effort of moving the head and neck. In six single case studies, the primary outcome measure for each case was the Cervical Dystonia Questionnaire (CDQ) to measure the impact of the program on the individuals' quality of life. Secondary outcome measures were identified for the different components of the physiotherapy program: Visual Analogue Scale (pain); Postural Orientation Index (postural orientation awareness); and Movement Energy Index (effort of moving head and neck). Each of the cases had the severity of their problems scored on the Toronto Western Spasmodic Torticollis Scale. The study period was 26 weeks: 2 weeks' baseline period, 4 weeks' treatment period, and 20 weeks' follow-up. All measures except the Movement Energy Index (MEI) and CDQ-24 were taken three times per week for the first 6 weeks of the study and then once at 3 and 6 months. The MEI was taken once a week during the pretreatment and the treatment periods and during the first 2 weeks of follow-up and also after 3 and 6 months of follow-up. The CDQ-24 was taken once in the pretreatment period, once after completion of treatment, once 2 weeks after treatment, and once at 3 and 6 months of follow-up. Five of the six case studies reported an increase in quality of life at 6-month follow-up, as measured on the CDQ-24. Three of the six cases reported a reduction in pain and severity of the dystonia and had improved scores on the postural orientation measure at 6-month follow-up. All six patients had a reduction in the movement energy scores, but this was not significant. The outcomes of the six case studies would suggest that further investigation is required to show the effectiveness of physiotherapy programs in the management of CD.

3456 251 - 277 of 277
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