Practical approach to respiratory emergencies in neurological diseases

  • Fabrizio Racca
  • Andrea Vianello
  • Tiziana Mongini
  • Paolo Ruggeri
  • Antonio Versaci
  • Gian Luca Vita
  • Giuseppe VitaEmail author
Review Article


Many neurological diseases may cause acute respiratory failure (ARF) due to involvement of bulbar respiratory center, spinal cord, motoneurons, peripheral nerves, neuromuscular junction, or skeletal muscles. In this context, respiratory emergencies are often a challenge at home, in a neurology ward, or even in an intensive care unit, influencing morbidity and mortality. More commonly, patients develop primarily ventilatory impairment causing hypercapnia. Moreover, inadequate bulbar and expiratory muscle function may cause retained secretions, frequently complicated by pneumonia, atelectasis, and, ultimately, hypoxemic ARF. On the basis of the clinical onset, two main categories of ARF can be identified: (i) acute exacerbation of chronic respiratory failure, which is common in slowly progressive neurological diseases, such as movement disorders and most neuromuscular diseases, and (ii) sudden-onset respiratory failure which may develop in rapidly progressive neurological disorders including stroke, convulsive status epilepticus, traumatic brain injury, spinal cord injury, phrenic neuropathy, myasthenia gravis, and Guillain–Barré syndrome. A tailored assistance may include manual and mechanical cough assistance, noninvasive ventilation, endotracheal intubation, invasive mechanical ventilation, or tracheotomy. This review provides practical recommendations for prevention, recognition, management, and treatment of respiratory emergencies in neurological diseases, mostly in teenagers and adults, according to type and severity of baseline disease.


Neurological diseases Respiratory failure Hypercapnia Hypoxemia Invasive mechanical ventilation Noninvasive ventilation 



Amyotrophic lateral sclerosis


Acute respiratory distress syndrome


Acute respiratory failure


Ataxia telangiectasia


Central nervous system


Cough peak expiratory flow




Myotonic dystrophy type 1


Duchenne muscular dystrophy


Emergency room


Endotracheal intubation


Facioscapulohumeral muscular dystrophy


Forced vital capacity


Guillain–Barré syndrome


Glasgow coma scale


Intensive care unit


Invasive mechanical ventilation


Infantile-onset Pompe disease


Myasthenia gravis


Maximum inspiratory pressure


Noninvasive ventilation


Neuromuscular disorders


Parkinson’s disease




Respiratory failure


Spinal cord injury


Status epilepticus


Spinal muscular atrophy


Traumatic brain injury


Upper airway obstruction


Funding information

No specific grant was received from any funding agency in the public, commercial, or not-for-profit sectors.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Fondazione Società Italiana di Neurologia 2019

Authors and Affiliations

  1. 1.Department of Anaesthesia and Intensive CareSant’Antonio e Biagio e Cesare Arrigo HospitalAlessandriaItaly
  2. 2.Respiratory Pathophysiology DivisionUniversity of PaduaPaduaItaly
  3. 3.Neuromuscular Center, Department of NeurosciencesUniversity of TurinTurinItaly
  4. 4.Unit of Pneumology, Department BIOMORFUniversity of MessinaMessinaItaly
  5. 5.Intensive Care Unit, AOU Policlinico “G. Martino”MessinaItaly
  6. 6.Nemo Sud Clinical Centre for Neuromuscular DisordersMessinaItaly
  7. 7.Unit of Neurology and Neuromuscular Diseases, Department of Clinical and Experimental MedicineUniversity of MessinaMessinaItaly

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