OBM Neurobiology is an international peer-reviewed Open Access journal published quarterly online by LIDSEN Publishing Inc. By design, the scope of OBM Neurobiology is broad, so as to reflect the multidisciplinary nature of the field of Neurobiology that interfaces biology with the fundamental and clinical neurosciences. As such, OBM Neurobiology embraces rigorous multidisciplinary investigations into the form and function of neurons and glia that make up the nervous system, either individually or in ensemble, in health or disease. OBM Neurobiology welcomes original contributions that employ a combination of molecular, cellular, systems and behavioral approaches to report novel neuroanatomical, neuropharmacological, neurophysiological and neurobehavioral findings related to the following aspects of the nervous system: Signal Transduction and Neurotransmission; Neural Circuits and Systems Neurobiology; Nervous System Development and Aging; Neurobiology of Nervous System Diseases (e.g., Developmental Brain Disorders; Neurodegenerative Disorders).

OBM Neurobiology publishes research articles, technical reports and invited topical reviews. Although the OBM Neurobiology Editorial Board encourages authors to be succinct, there is no restriction on the length of the papers. Authors should present their results in as much detail as possible, as reviewers are encouraged to emphasize scientific rigor and reproducibility.

Free Publication in 2019
Current Issue: 2019  Archive: 2018 2017

Special Issue

Non-invasive Positive Pressure Ventilation in a Patient with the Severe Form of X-linked Myotubular Myopathy

Submission Deadline: May 20, 2019 (Open)               Submit Now

Guest Editor

Kenji Inoue, MD
Department of Pediatrics, Shiga Medical Center for Children, Shiga, Japan
Email: [email protected]
Website: http://www.pref.shiga.lg.jp/mccs/shinryo/shinke/index.html
Research Interests: Developmental disorders, Neuromuscular, and Epilepsy

About this topic

The severity of X-linked myotubular myopathy (XLMTM) ranges from mild to severe, depending on the level of ventilatory support required. Patients with the severe form of XLMTM usually die within the first year of life due to respiratory failure. Most survivors need tracheostomies, and there have only been a few reports about the use of non-invasive positive pressure ventilation (NPPV) in patients with the severe form of XLMTM because of the severity of the associated respiratory failure. Therefore, the precise NPPV settings employed in patients with the severe form of XLMTM were not described in any of the previous reports, while in patients with spinal muscular atrophy type I, the key to success of NPPV was reported to be the use of high-span PIP. What setting of NPPV is appropriate in patients with the severe form of XLMTM? Can the high-span PIP be employed to patients with the severe form of XLMTM? In this special issue, we aim to the introduction of a research or a case report in respiratory management with NPPV in patients with severe form of XLMTM, as well as in patients with other severe form of neuromuscular disorders.