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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
  • 74 Downloads

Abstract

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.

Keywords

Neurological diseases Respiratory failure Hypercapnia Hypoxemia Invasive mechanical ventilation Noninvasive ventilation 

Abbreviations

ALS

Amyotrophic lateral sclerosis

ARDS

Acute respiratory distress syndrome

ARF

Acute respiratory failure

AT

Ataxia telangiectasia

CNS

Central nervous system

CPEF

Cough peak expiratory flow

DM

Dermatomyositis

DM1

Myotonic dystrophy type 1

DMD

Duchenne muscular dystrophy

ER

Emergency room

ETI

Endotracheal intubation

FSHD

Facioscapulohumeral muscular dystrophy

FVC

Forced vital capacity

GBS

Guillain–Barré syndrome

GCS

Glasgow coma scale

ICU

Intensive care unit

IMV

Invasive mechanical ventilation

IOPD

Infantile-onset Pompe disease

MG

Myasthenia gravis

MIP

Maximum inspiratory pressure

NIV

Noninvasive ventilation

NMDs

Neuromuscular disorders

PD

Parkinson’s disease

PM

Polymyositis

RF

Respiratory failure

SCI

Spinal cord injury

SE

Status epilepticus

SMA

Spinal muscular atrophy

TBI

Traumatic brain injury

UAO

Upper airway obstruction

Notes

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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