Respiratory Disorders Associated With Cerebral Palsy and Neurodegenerative Diseases

Laura Beth Mann Dosier, MD, and Richard M. Kravitz, MD, FAAP

Introduction/Etiology/Epidemiology

•Cerebral palsy (CP) is not a disease per se but is the consequence of perinatal insults, such as hypoxia, infection, prematurity, and intrauterine growth retardation.

•There can be many manifestations of CP, depending on the severity of damage present; these may include

—Central nervous system (CNS) complications (seizures, developmental delay)

—Musculoskeletal complications (spasticity, hypotonia, contractures, scoliosis)

—Respiratory complications

•Neurodegenerative diseases represent a separate and distinct group of illnesses that have multiple different etiologic origins.

•CP and neurodegenerative diseases have a common final pathway that can lead to respiratory complications.

•The most common respiratory problems in children with CP and neurodegenerative diseases include

—Airway clearance abnormalities (weakened or ineffective cough)

—Aspiration (with or without gastroesophageal reflux [GER])

▪Recurrent aspiration may lead to bronchiectasis.

—Recurrent pneumonia

—Asthma (independent of aspiration)

—Restrictive lung disease

—Sialorrhea

—Sleep disturbances

▪Snoring

▪Obstructive and central apnea

—Upper-airway obstruction

•Muscle tone abnormalities can lead to

—Ineffective or diminished cough

—Altered swallowing function

▪Inability to handle oral secretions

▪Higher risk for aspiration of secretions and food

—Altered upper-airway tone with subsequent collapse and obstruction

▪Obstructive sleep apnea

▪Hypoventilation

▪Fragmented sleep

•CNS-related dysfunction

—Altered sleep patterns (reversed day-night cycles)

—Insomnia

—Excessive tiredness

•Orthopedic complications

—Scoliosis

▪Development of restrictive lung disease

▪Altered airway clearance

▪Abnormal insertion of the esophagus through the diaphragm and loss of lower esophageal sphincter tone, which leads to GER.

•Gastrointestinal complications

—Increased GER

—Secondary aspiration

Clinical Features of Pulmonary Comorbidities

•Symptoms

—Coughing or wheezing

▪May be related to aspiration, exacerbation of bronchiectasis, or comorbid asthma

—Feeding-related symptoms

▪Choking with subsequent coughing and/or wheezing and aspiration

—Upper-airway symptoms

▪Noisy breathing

—Sleep-related symptoms

▪Snoring

▪Apneas

▪Gasping respirations while asleep

•Signs

—Scoliosis

—Wet voice (especially with feeding)

—Altered cough (usually weak in quality)

—Chest congestion (rattling sound)

—Stridor

—Stertor

—Snoring

•Chest imaging (chest radiography and computed tomography) may show