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  • 1.
    Rostedt Punga, Anna
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Rostedt Punga: Clinical Neurophysiology. Uppsala Univ Hosp, Uppsala, Sweden.
    Jabre, Joe F.
    Univ Calif Los Angeles, David Geffen Sch Med, Dept Neurol, Los Angeles, CA 90095 USA.
    Amandusson, Åsa
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Rostedt Punga: Clinical Neurophysiology. Uppsala Univ Hosp, Uppsala, Sweden.
    Facing the challenges of electrodiagnostic studies in the very elderly (>80 years) population2019In: Clinical Neurophysiology, ISSN 1388-2457, E-ISSN 1872-8952, Vol. 130, no 7, p. 1091-1097Article in journal (Refereed)
    Abstract [en]

    Objective: Studies on electrodiagnostic (EDX) methods usually exclude the very elderly. This also holds true for studies of normal EDX values. We analyzed the outcome and diagnostic value of EDX and collected reference data in a large cohort of patients >= 80 years of age. Methods: Referral information, ICD-10 diagnoses and EDX data were retrieved from all patients >= 80 years of age referred for EDX studies at our department in 1995-2015. Normative data, including reference ranges, were obtained using the extrapolated norms (e-norms) method. Results: 1966 unique patients (2335 examinations) were included. Only 11% were considered to have normal findings. 66% had pathological EDX findings in accordance with the indication for referral. Carpal tunnel syndrome was by far the most common diagnosis. Normative data retrieved using e-norms were similar to those of reference values from healthy subjects regarding EMG multiMUP data, but typically provided a wider normality window when applied to nerve conduction parameters. Conclusions: EDX studies are valuable in the diagnostic work-up of very elderly patients. Using the e-norms method may be a useful alternative when obtaining reference values in this age group. Significance: Our findings provide additional insights to the challenges of evaluating very elderly patients with neuromuscular disease and underline the importance of including this growing part of the patient population in EDX research.

  • 2.
    Sabre, Liis
    et al.
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Rostedt Punga: Clinical Neurophysiology.
    Evoli, Amelia
    Catholic Univ, Dept Neurol, Rome, Italy.
    Rostedt Punga, Anna
    Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Rostedt Punga: Clinical Neurophysiology.
    Cognitive dysfunction in mice with passively induced MuSK antibody seropositive myasthenia gravis2019In: Journal of the Neurological Sciences, ISSN 0022-510X, E-ISSN 1878-5883, Vol. 399, p. 15-21Article in journal (Refereed)
    Abstract [en]

    Recent reports on cognitive dysfunction, in addition to skeletal muscle fatigue, in muscle-specific tyrosine kinase antibody seropositive (MuSK+) myasthenia gravis (MG) patients led us to study cognition in mice with MuSK+passive transfer MG (PTMG). Twelve 7-week-old female wild-type C57BL/6J mice were passively immunized with IgG from MuSK+ MG patients and 12 control mice received intraperitoneal saline injections. Mice were evaluated with clinical, neurophysiological and behavioral tests (Barnes maze (BM) and novel object recognition (NOR)), and the muscles were immunostained to evaluate the neuromuscular junction in the end of the study. Two-thirds of the immunized mice developed clinically distinct MuSK + PTMG. MuSK + PTMG mice spent less time exploring the novel object in the NOR test (MuSK+ mice 36.4% +/- 14.0 vs controls 52.4% +/- 13.0, p = .02), unrelated to the muscle weakness and regardless of rodents' innate preference of novelty. In the BM test, control mice were more eager to use the direct strategy than the MuSK+ mice (MuSK+ 17.3% vs controls 29.5%, p = .02). Our findings shed new light on cognition dysfunction in human MuSK + MG patients and indicate that recognition memory in the perirhinal cortex could be affected in MuSK + MG.

  • 3.
    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.

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