Development of Augmented-Reality-Based Magnetic Field Visualization System as an Educational Tool.

Electromagnetism is a difficult subject to learn because the phenomenon is not observed directly and does not have a perceivable, concrete image. The visualization of the phenomenon will greatly help beginners understand electromagnetics. This paper proposes an augmented-reality-based visualization system of the magnetic flux density around current-generating objects. The system can be realized with a portable magnetic field sensor and a familiar device-a smartphone. The effectiveness of the system is verified through experiments, and our findings suggest that the system can effectively help an operator gain an intuitive understanding of the magnetic flux density.

Psychiatric and neurodevelopmental aspects of Becker muscular dystrophy.

Little is known about the relationship between Becker Muscular Dystrophy (BMD) and mental disorders. This study aimed to clarify whether BMD is a risk factor for psychiatric diseases. We asked genetically or immunohistochemically confirmed BMD patients to participate in the study interview. Participants who consented to psychiatric tests underwent further assessments of intellectual, psychological, and neurodevelopmental disorders. In total, 76 (73%) of 105 BMD patients (median age, 37 years) completed the interview. Of these, 6 had developmental disorders (mental retardation, pervasive developmental disorder), 33/76 (43%) experienced bullying in school, 11 exhibited problematic behaviors such as cutting class and violent incidents, and 16 had psychiatric disorders (schizophrenia spectrum, 5; depressive spectrum, 4; stress-related disorders, 3; obsessive-compulsive and related disorders, 2; somatic symptom and related disorders, 2; bipolar and related disorders, 1). Mean IQ was normal, whereas 13/40 (32.5%) of participants were in a depressive state. High trait anxiety was found in 20/40 (50%) of patients, while 15/40 (38.5%) were in an anxious state. Review of MRI data from 14 participants revealed brain atrophy caused solely by BMD and unrelated to any other complication. Our findings suggest that BMD patients are at risk of developing psychiatric disorders. Physical handicap or bullying may influence their mental state, as many of them have high trait anxiety. Parents, teachers, and supporters should be mindful of the daily environment of BMD patients and provide support to help them cope with stress.

Social involvement issues in patients with Becker muscular dystrophy: A questionnaire survey of subjects from a patient registry.

BACKGROUND: Little is known about the relationship between Becker Muscular Dystrophy (BMD) and developmental problems, school life, employment, and mental problems. We aimed to clarify whether BMD is a risk factor for developmental disorders, problematic behavior, psychiatric diseases, and other social difficulties in school life and employment. METHODS: Adults with genetically or immunohistochemically confirmed BMD from the Registry of Muscular Dystrophy in Japan (REMUDY) were asked to complete a questionnaire regarding patient history, school life, employment, and mental problems. RESULTS: In total, 125 (68.3%) of 183 participants with BMD (median age, 37.2 years) completed the questionnaire. Of these, ten had developmental disorders (mental retardation, autism, and speech disturbance). Fifty-eight (44%) experienced bullying in school, and 39 felt the reason for bullying was physical handicap. Sixteen participants experienced problematic behavior such as cutting class, domestic violence, violent incidents, suicide attempts, or self-mutilation. Employment histories were noted by 92 (73%), of whom 15 could not continue to work due to physical handicaps. Fifteen participants had psychiatric disorders, with 5, 3 and 1 having neurosis, depression, and bipolar disorder, respectively. The other 6 participants with psychiatric disorders did not specify their diagnoses. Patients carrying a Dp140 expression change had significantly more incidences of developmental disorders, but not bullying, problematic behavior, workplace difficulties, or psychiatric disorders. CONCLUSIONS: Patients with BMD risk bullying and workplace difficulties, as well as developing psychiatric disorders. Parents, teachers, and supporters should be mindful of the daily environment of BMD patients and provide support to help them cope with stress.

[Two cases of nemaline myopathy presenting with hypertrophy of distal limbs with prominent asymmetry].

Nemaline myopathy commonly presents with symmetrical proximal weakness. Here we report two cases of nemaline myopathy presenting with distal dominant involvement with prominent asymmetry. Case 1 was a 37-year-old man who recalled frequently falling down and had right calf atrophy since he was 3-years-old. He had right calf muscle atrophy and weakness and steppage gait; his cardiopulmonary function was normal. Case 2 was a 35-year-old man with right calf muscle atrophy and weakness since childhood. He had right dominant distal leg weakness and atrophy together with respiratory failure and started noninvasive positive pressure ventilation. He also developed cardiomyopathy and died from acute respiratory failure due to pneumonia at age 39. Both cases harbored compound heterozygous nebulin (NEB) mutations with c.20131 C>T:p.Arg6711Trp and a nonsense mutation. Nemaline myopathy associated with NEB mutations can present as distal dominant myopathy with prominent asymmetry.

Two cases of sporadic late onset nemaline myopathy effectively treated with immunotherapy.

Sporadic late onset nemaline myopathy (SLONM) associated with monoclonal gammopathy of undetermined significance (MGUS) is an adult onset myopathy with poor clinical outcomes, requiring high-dose intravenous melphalan with autologous peripheral blood stem cell transplantation (HDM-SCT). Here we report two cases of SLONM associated with MGUS in which improvements were achieved only with immunotherapy. A 39-year-old woman had a two-year history of dropped head syndrome and progressive proximal weakness. On admission, she was able to walk with assistance and had lordosis with camptocormia. Combination therapy with plasmapheresis and intravenous immunoglobulin in addition to intravenous methylprednisolone pulse therapy ameliorated camptocormia and proximal weakness after one year. A 51-year-old man had difficulty in raising his arms and required walking assistance prior to visiting our hospital. He had proximal weakness and atrophy, winged scapulae, and gait disturbance. After combination immunotherapy, no progression was observed for 13 years. In both cases, patients did not desire to undergo HDM-SCT, and IgG kappa monoclonal protein was positive, of which the levels were normalized after immunotherapy. Combination immunotherapy can be a possible alternative to HDM-SCT in patients with SLONM. Both patients showed myogenic changes with abundant fibrillation, and needle EMG revealed positive sharp waves. Case 1 showed high signal intensities in MRI STIR/T2WI in muscles showing weakness. These findings are commonly observed in patients with myositis, suggesting that, without muscle biopsy, SLONM may be misdiagnosed as myositis. Muscle biopsy revealed scattered fibers with nemaline bodies without type 2B deficiency, which are important pathological findings that differentiate SLONM from congenital nemaline myopathy.