Abstract
To maintain adequate gas exchange and alveolar ventilation, the respiratory system requires an intact parenchyma and a respiratory pump. The failure of any of these components can result in respiratory failure. Neurological disorders affect the respiratory system either through the central nervous system (altering the ventilatory centers, swallowing, and respiratory movements) or through the peripheral nervous system (altering the respiratory pump and its regulatory feedback to the central system). The degree of impairment will depend on the disease, the severity of nerve or muscle involvement, and the timing of the diagnosis. A complete neurological assessment will provide a systemic approach, which must involve a multidisciplinary team. The respiratory system should be evaluated from the clinical and physiological standpoints on a regular basis, following guidelines that include spirometry, static pressure measures, peak flow cough, sleep studies, chest imaging, and bronchoscopy, among other evaluations. The treatment must focus on slowing down the progressive deterioration of musculoskeletal and respiratory function, thus improving the quality of life.
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Allen J. Pulmonary complications of neuromuscular disease: a respiratory mechanics perspective. Paediatr Respir Rev. 2010;11:18–23.
Arens R, Muzumdar H. Sleep, sleep disordered breathing, and nocturnal hypoventilation in children with neuromuscular diseases. Paediatr Respir Rev. 2010;11:24–30.
Bach JR, Saltstein K, Sinquee D, Weaver B, Komaroff E. Long term survival in Werdnig–Hoffmann disease. Am J Phys Med Rehabil. 2007;86:339–45.
Benditt JO, Boitano LJ. Pulmonary issues in patients with chronic neuromuscular disease. Am J Respir Crit Care Med. 2013;187(10):1046–55.
Hukins CA, Hillman DR. Daytime predictors of sleep hypoventilation in Duchenne muscular dystrophy. Am J Respir Crit Care Med. 2000;161(1):166–70.
Hull J, Aniapravan R, Chan E. British Thoracic Society guideline for respiratory management of children with neuromuscular weakness. Thorax. 2012;67:i1–i40.
Katz SL. Assessment of sleep-disordered breathing in pediatric neuromuscular diseases. Pediatrics. 2009;123:S222–5.
Kennedy JD, Martin AJ. Chronic respiratory failure and neuromuscular disease. Pediatr Clin N Am. 2009;56:261–73.
Khirani S, Colella M, Caldarelli V, Aubertin G, Boulé M, Forin V, Ramirez A, Fauroux B. Longitudinal course of lung function and respiratory muscle strength in spinal muscular atrophy type 2 and 3. Eur J Paediatr Neurol. 2013;17:552–60.
Khirani S, Ramirez A, Aubertin G, Boulé M, Chemouny C, Forin V, Fauroux B. Respiratory muscle decline in Duchenne muscular dystrophy. Pediatr Pulmonol. 2014;49:473–81.
Kwon YH, Lee HY. Differences of respiratory function in children with spastic diplegic and hemiplegic cerebral palsy, compared with normally developed children. J Pediatr Rehabil Med. 2013;6:113–7.
Mellies U, Ragette R, Schwake C, et al. Long-term noninvasive ventilation in children and adolescents with neuromuscular disorders. Eur Respir J. 2003;22:631–6.
Panitch HB. The pathophysiology of respiratory impairment in pediatric neuromuscular diseases. Pediatrics. 2009;123:S215–8.
Rajatonirina S, Razanajatovo NH, Ratsima EH, et al. Outcome risk factors during respiratory infections in a paediatric ward in Antananarivo, Madagascar 2010–2012. PLoS One. 2013;8:1–9.
Sharma GD. Pulmonary function testing in neuromuscular disorders. Pediatrics. 2009;123:S219–21.
Vega-Briceño LE, Contreras I, Prado F. Evaluación respiratoria de la enfermedad neuromuscular en niños. Rev Neumol Pediatr. 2007a;2(1):6–10.
Vega-Briceño LE, Prado F, Bertrand P, Sánchez I. Soporte ventilatorio no invasivo en enfermedades neuromusculares. Rev Neumol Pediatr. 2007b;2:15–20.
Wallgren-Pettersson C, Bushbyb K, Melliesc U, Simonds A. Ventilatory support in congenital neuromuscular disorders—congenital myopathies, congenital muscular dystrophies, congenital myotonic dystrophy and SMA (II). Neuromuscul Disord. 2004;14:56–69.
Yang ML, Finkel RS. Overview of paediatric neuromuscular disorders and related pulmonary issues: diagnostic and therapeutic considerations. Paediatr Respir Rev. 2010;11:9–17.
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Escobar Henríquez, R., Chateau Infante, B. (2020). Respiratory Complications in Children with Neurological Diseases. In: Bertrand, P., Sánchez, I. (eds) Pediatric Respiratory Diseases. Springer, Cham. https://doi.org/10.1007/978-3-030-26961-6_26
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DOI: https://doi.org/10.1007/978-3-030-26961-6_26
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