Neuromuscular disorders cause respiratory failure when they significantly impair the respiratory muscle pump. This is a complex system involving the diaphragm, intercostal, neck, shoulder girdle, abdominal wall and possibly paraspinal muscles. The dilator muscles of the palate, pharynx and larynx maintain a patent airway and air conduit. The diaphragm is the main muscle of inspiration, but is aided by the parasternal intercostals, and additionally by the accessory respiratory muscles in forceful inspiration, diaphragmatic weakness or compromised diaphragmatic function as is the case in lung emphysema. Exhalation is passive, but forceful expiration and cough require the abdominal wall and a portion of the intercostals. This chapter focuses on the physiology of the muscles involved in respiration, and the recognition of developing neuromuscular respiratory failure, its clinical evaluation and assessment, and basic principles of management.

Managing the mechanical ventilator in critical illness is far from formulaic. Criteria of intubation are rarely contemplated when a patient is struggling to maintain a patent airway. Once the airway is secured, adjustments in ventilator settings and modes are continuously made, and there is a fair amount of trial and error. Weaning from the ventilator is not standardized (and probably never will be), and protocols (if there are any) are based on consultant preferences and mostly experience. The consensus statement of the European Society of Intensive Care Medicine on mechanical ventilation (MV) in acute brain injury has clearly shown that evidence for certain approaches was either insufficient or lacking and that a substantial amount of research is needed to demonstrate the feasibility, safety, and efficacy of different management approaches in this category of patients.

This chapter reviews considerations in each intervention during the patient’s clinical trajectory of ventilation in the neurosciences ICU. The reader will find that early intubation and mechanical ventilation are initiated because patients cannot protect their airways or have insufficient respiratory drive to maintain oxygenation and normocarbia.

The future challenges for neurorespiratory medicine are significant with respect to the consequences of medical progress, demographic changes and epidemiological forecasts. The treatment of a variety of neurological diseases like genetic disease, inflammatory diseases and stroke improves steadily, and life supporting technologies and care structures are undergoing an evolution, too. To agree on a subset of principles of care, to define professions and qualifications needed to care for patients with respiratory impairment due to neurological disease and to strive for further medical and technological progress are key necessities. These are discussed in this chapter, and a number of suggestions for further research are advanced.

Respiratory regulation comprises respiratory rhythmogenesis, formation of the respiratory motor pattern, control of blood oxygen and carbon dioxide, increase of minute ventilation during physical activity, adaptation of respiration to the sleep-wake cycle, coordination of breathing with swallowing, cough, sneezing, choking and voluntary activity such as speech or singing. Other factors such as growth and maturation, emotion, pregnancy, injury, disease, body temperature, pain and aging lead to changes in respiration. The presence of a respiratory rhythm generator in the brainstem is now known to be a common feature of all vertebrates. Knowledge about respiratory regulation is mainly derived from animal models, but respiratory regulation in humans is subject to an increasing number of physiological, electrophysiological, neuroradiographic, histopathological and genetic studies. This chapter provides an overview of respiratory regulation, focused on neuroanatomical, neurophysiological and clinical apsects.

In times past, an inquisitive physician-scientist must have pondered these questions: How do we unknowingly breathe? What brain structures control our breathing? Why is breathing so perfectly rhythmic? Is there a lung-brain communication, and if so, how? But an even more fundamental question must have been: how much brain injury can one sustain before breathing stops?

It took two centuries (more or less) to answer the above-mentioned questions and gradually add small pieces to a large (still incomplete) puzzle. The respiratory center in the brainstem was identified and characterized in the late 1800s and early 1900s. Similarly, the function of the respiratory muscles and its neural connection with cranial nerves (CN) became better known.

This chapter recounts the history of the neurology of breathing and, thus, the discovery of the respiratory center and the respiratory mechanics. Sections of the chapter review experimental and clinical discoveries of those parts of the central and the peripheral nervous system involved with breathing while acknowledging their interplay.

This chapter discusses the role of palliative care in the management of respiratory problems in neurological disease. To realize the right to live and to enjoy equal participation for neurological patients with respiratory symptoms may be complex and require extensive human, technical and financial resources, and, especially in low- and mid-income countries these resources may not be present. National and cultural differences in the role of palliative care are discussed, furthermore specific problems of palliative care in respiratory therapy such as correct indications, informed consent issues, therapy restriction physician-assisted suicide and euthanasia, in care settings such as critical care. The authors suggest a pathway to decision-making and introduce treatment strategies with a focus on respiratory symptoms.