4.5 Review

Hypoventilation syndrome in neuromuscular disorders

Journal

CURRENT OPINION IN NEUROLOGY
Volume 34, Issue 5, Pages 686-696

Publisher

LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/WCO.0000000000000973

Keywords

neuromuscular hypoventilation; pathophysiology; respiratory muscle training; respiratory muscle weakness

Funding

  1. DGM-Deutsche Gesellschaft fur Muskelkranke e.V
  2. Sanofi-Aventis Germany GmbH
  3. CSLBehring GmbH
  4. Alexion Pharma GmbH
  5. Desitin Germany
  6. Akcea GmbH
  7. Sanofi Genzyme
  8. Aspire LLC

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This article summarizes the current aspect of hypoventilation syndrome in neuromuscular disorders, including pathophysiology, clinical symptoms, assessment, and treatments. Recent findings show that new therapeutic agents have positive effects on the respiratory system, while symptomatic therapies like mechanical ventilation and respiratory muscle training are crucial for improving patients' quality of life.
Purpose of review Hypoventilation syndrome in neuromuscular disorders (NMDs) is primarily due to respiratory muscle weakness and results in increased morbidity and mortality. This article highlights current aspects of neuromuscular hypoventilation syndrome, including pathophysiology, clinical symptoms, assessment, respiratory involvement in various NMD, and causal and symptomatic treatments with an emphasis on recent research and advances. Recent findings and Summary New therapeutic agents have been developed within the last years, proving a positive effect on respiratory system. Symptomatic therapies, including mechanical ventilation and cough assistance approaches, are important in NMD and respiratory muscle training may have benefit in strengthening respiratory muscles and should be offered patients with respiratory muscle weakness the same way as physiotherapy. Correct respiratory assessments and their correct interpretation are hallmarks for early diagnosis of hypoventilation syndrome and treatment.

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