Epilepsy, found in 35%–62% of children with CP,9 is more frequent in children with abnormal neuroimaging findings and greater motor impairment. Nonepileptic paroxysmal events and some movement disorders may create confusion and errors in treatment. Performance of an electroencephalogram (EEG) is indicated in case epileptic seizures are suspected. In this group of patients, the most frequent disorder is status epilepticus (14%–47% of children with CP and epilepsy), and therefore seizures must be treated at an early stage. The management of epilepsy does not differ from the followup in patients without CP, but in follow-up visits, clinicians should always ask about seizures or any “new”, “different” “recurrent” events, uncontrolled movements etc, to identify epilepsy that has gone unnoticed by the parents. Videos may be very useful to understand the presentation of these events and determine whether performance of EEG is indicated.

Intellectual disability (ID), observed in 40%–70% of individuals with CP,10 is strongly associated with the type of CP (more severe ID in spastic versus dyskinetic CP and in quadriplegia versus hemiplegia), the presence of epilepsy and abnormal features on EEG or neuroimaging.

The most frequent language disorders are dysarthria (in 40%) and inability to talk (in 25%).11 Patients with CP may also experience impairment in other areas of communication, such as the development of gestures and facial expressions, receptive and expressive language acquisition and speech production. Personalised treatment must be initiated early (ideally starting before age 2 years) in any child at risk of CP.

Over 50% of patients with CP have mental health disorders,12 chiefly mood and conduct disorders, social impairment, attention deficit and hyperactivity which, added to the other features of the disease, exacerbate problems with schooling and adaptation. On the whole, they cause significant anxiety and hinder integration of the child in the community, so it is important to identify these disorders, not underestimate them and offer any resources that may be needed. Followup by school counsellors, adaptations at the level required by the child and support from a speech therapist or speech-language pathologist (SLP) or educational therapist (ET) are indispensable in these patients. These children are more likely to be the target of bullying or harassment, so it is important to watch for red flags that may indicate their presence.

Movement disorders (dystonia, chorea, athetosis and ballism), described in up to 40% of patients,13 are more frequent in dyskinetic CP and often associated with spasticity. They cause significant functional impairment, as they may interfere with, impede or possibly preclude performance of activities of daily living (ADLs), and may also cause pain. The most frequent one is dystonia, characterised by involuntary muscle contractions, sustained or intermittent, that cause twisting movements or abnormal posture caused by the simultaneous contraction of agonist and antagonist muscles. Physical therapy is the cornerstone of treatment. Trihexyphenidyl is usually the first-line drug used in pharmacotherapy.

Spasticity occurs in 85% of children with CP14 and results in functional impairment in ADLs (walking, eating, washing, dressing), in addition to possibly causing muscle pain, spasms and abnormal postures. In some cases, spasticity may be beneficial, as the increased muscle tone in the lower limbs helps maintain the standing position, contributing to the maintenance of muscle mass and bone density. Spasticity can be measured with the modified Ashworth scale and the Tardieu scale (Table 4). A personalised treatment plan must be developed, with realistic goals established in agreement with the patient and caregiver. Possible goals may include optimising functioning, improving hygiene, alleviating pain or preventing complications to improve the quality of life of the patient and the family. The coordinating paediatrician must know the different treatment options: nonpharmacological treatments such as physical therapy, occupational therapy and assistive devices, oral pharmacological treatment including oral therapy with baclofen (first-line treatment for generalised spasticity) or local parenteral treatment with botulinum toxin, and surgical intervention.

Most are secondary to spasticity, which creates fixed contractures that give rise to osteoarticular deformities (thumb-in-palm, wrist and elbow flexion, scoliosis, hip displacement/dislocation, clubfoot) that worsen the clinical condition of the child and may require orthopaedic surgery.15

Between 80% and 90% of patients with CP have chronic gastrointestinal disorders,16 chiefly, on one hand, malnutrition and impaired growth, and on the other, the main associated gastrointestinal disorders: dysphagia, gastro-oesophageal reflux (GOR) and constipation.17

The aetiology of malnutrition in children with CP is multifactorial, and this problem is identified in 60%–90% of affected patients. The factors underlying undernutrition are decreased intake, micronutrient deficiency and in rare cases increased nutritional requirements. The energy requirement can be estimated using the Schofield equation, as done in the general population, although it is important to take into account that non-ambulatory children that depend on assistive devices may have an energy expenditure that is 70% the expenditure observed in their healthy peers.

Dysphagia affects between 40% and 90% of children with CP.18 The development of oral motor skills depends on neural maturation and requires the coordination of muscle groups that are under the control of cranial nerves, the brainstem and the cerebral cortex. Children with CP have weak sucking, poor suck-swallow coordination, inefficient lip closure and ineffective chewing that often results in an increased duration of feeding and in some cases an insufficient energy intake, which would result in malnutrition. On the other hand, oropharyngeal dysphagia carries a risk of bronchopulmonary aspiration of food that can worsen the respiratory condition of the patient. Therefore, assessing the safety and effectiveness of oral feeding is of vital importance. The assessment should include questions about the duration of meals (with durations > 30min considered a possible warning sign) or the development of cough, cyanosis, sweating or dyspnoea while eating.

The aetiology of GOR is multifactorial: abnormal posture, dysmotility due to neurologic impairment with increased frequency of transient lower oesophageal sphincter relaxation and increased abdominal wall pressure secondary to spasticity and scoliosis. Gastro-oesophageal reflux is found in nearly half of children with CP and should be suspected in patients with food refusal, vomiting, drooling, respiratory complications or other nonspecific symptoms such as irritability, anaemia or dental abnormalities. In mild cases, clinicians ought to recommend thickening any consumed fluids and staying upright after meals. If this does not achieve control of symptoms, proton pump inhibitors are the first-line agents used for pharmacological treatment.19

Lastly, posture and muscle tone abnormalities, deficient fluid intake, immobility or the use of pharmaceuticals lead to constipation in approximately half of patients with CP. The initial approach to its management is disimpaction (at which time, foods with a high fibre content should be avoided), followed by maintenance therapy with polyethylene glycol or lactulose in addition to dietary measures (adequate intake of fibre and fluids).16

Children with CP are at risk of low bone mineral density (BMD) and osteoporosis. Immobility, inadequate intake of micronutrients (calcium and vitamin D), undernutrition and anticonvulsant therapy may contribute to their development. Pathologic fractures may occur in up to 20% of these patients, most frequently involving the distal femur. Early detection is important, as most cases are asymptomatic (up to 80% of vertebral fractures).

Dual-energy x-ray absorptiometry (DEXA) is the most widely used technique to measure BMD. Low BMD is defined as a BMD z-score for age of less than –2 (adjusted for height if the height z-score of the patient <–2). The diagnosis of osteoporosis requires presence of low BMD combined with a history of clinically significant fractures (2 or more long bone fractures by age 10 years or 3 or more long bone fracture at any age up to 19 years) or vertebral compression fractures in the absence of local disease or high-energy trauma.

When it comes to the measures used to improve BMD, the current evidence shows that calcium and vitamin D supplementation may help and that bisphosphonates probably help, while there is insufficient evidence to support high-impact physical activity (running, jumping) for this purpose.

Oral calcium supplementation in children with CP with a normal BMD and without bone fragility should be considered if calcium intake is insufficient, although it is always preferable to improve calcium intake through dietary sources. In patients with low BMD, bone fragility or osteoporosis, supplementation with calcium and vitamin D is indicated.19

Children with CP are more likely to develop caries, malocclusion and periodontal disease compared to the general population (incidence of up to 90%).20

They are one of the main causes of morbidity and mortality in children with CP. Many factors are at play in respiratory complications in patients with CP21 (Fig. 2).22

Figure 2.

Factors involved in respiratory insufficiency in children with CP.

GOR, gastro-oesophageal reflux; SAHS, sleep apnoea-hypopnoea syndrome.

(0.22MB).

Respiratory manifestations vary with the age of the patient, and it is more frequent for infants to experience difficulty feeding, aspiration and apparent life-threatening events and for older children to experience persistent cough, noisy breathing and recurrent respiratory infections. It is also important to be watchful for manifestations suggestive of sleep apnoea-hypopnoea.

Between 40% and 75% of children with CP have some degree of visual impairment.23 Clinicians must be alert to recognise the signs of possible vision impairment: nystagmus (fusion maldevelopment nystagmus or lasting 15–30s in vesticulo-ocular reflex response tests), unusual behaviour, absence of ocular reflexes, purposeless eye movements and lack of visual curiosity and attention.24,25

The reported prevalence of hearing loss in children with CP ranges from 4% to 13%.25 In children with CP, the newborn hearing screening should include otoacoustic emissions and an auditory brainstem response test. Red flags of hearing loss include poor response to auditory stimuli, unusual behavioural responses and abnormal language development.

Up to 60% of patients with CP experience voiding dysfunction, enuresis, urinary urgency, incontinence or neurogenic bladder.26 The most specific warning signs are constant dripping of urine, need of abdominal contraction for initiation of urination or weak urine stream and polydipsia.

In all patients, treatment focuses on pursuing continence, regardless of disease severity, through behavioural and dietary measures (fluid intake of 1500mL/m2, treatment of constipation, control of schedules and posture, enuresis alarm, etc), pharmaceuticals such as oxybutynin in case of incontinence secondary to detrusor hypertonicity or desmopressin once daytime continence is achieved and nocturnal enuresis persists, intermittent catheterization or injection of botulinum toxin.27

The reported prevalence of drooling ranges from 10% to 58%.28 In the assessment of affected patients, clinicians should determine its frequency, severity and impact on the quality of life of children and their caregivers.

The main goals of treatment of drooling are: reducing its impact on physical, social and mental health, improve quality of life for patients and caregivers and reduce the burden experienced by caregivers.

Treatment consists of:

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  Teaching sensory awareness and oral motor skills: this is the cornerstone of management for children able to follow directions and cooperate with treatment.

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  Pharmacotherapy: to reduce production of saliva. The first-line agents are trihexyphenidyl (the drug of choice for dyskinetic forms of CP), glycopyrronium bromide or scopolamine, with atropine reserved for second-line treatment.

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  Botulin toxin infiltration.

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  Surgery.

Sleep disorders are found in 25% of children with CP compared to 5% of the general population. The main sleep disorders in these patients are difficulty falling and staying asleep, difficult morning awakening, nightmares and sleep anxiety.29 In many cases, these problems are due to environmental factors, hunger or thirst, and in many others to respiratory problems, need for repositioning due to immobility, seizures, pain or drug side effects, among other reasons.

The mainstay of treatment is sleep hygiene. If a treatable cause cannot be identified, melatonin can be tried out, especially in patients that have difficulty falling asleep, avoiding routine use of sedatives.

Pain is a frequent symptom, and some studies report a prevalence of up to 32% in children30 and 74%31 in youth with CP. The identification, assessment and management of pain in children with CP continues to pose a significant challenge mainly due to deficient exploration during the history taking, communication problems in the patients and the limited availability of instruments to measure pain. Thus, clinicians must routinely assess the presence of pain and its possible triggers.

While there is no consensus on which instrument is best to assess pain, of all that are currently available we recommend the revised Face, Legs, Activity, Cry, Consolability (r-FLACC) scale32 due to its ease of use and feasibility.

The most frequent mechanisms33 that generate pain in children with CP include nociceptive—somatic (spasticity, hip subluxation, fracture, etc) or visceral (constipation, GOR, gastric ulcer, etc)—and neuropathic, and we must not forget procedural pain (physical therapy, botulin toxin injections, etc). Children with CP often experience pain from several sources.

When it comes to pain management, the first step should be a comprehensive evaluation to attempt to identify the cause and treat it. If a cause cannot be established, clinicians should consider a stepped-approach trial of simple analgesia (such as paracetamol and/or ibuprofen) for mild to moderate pain.34

Establish a care plan for the patient that includes support for caregivers and education to teach them the necessary skills to play an active role in the lifelong development of the individual with CP. To this end,

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  Inform the primary care nurse of the case.

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  Refer to social worker at the primary care centre to make initial contact with community-based social services.

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  Determine the need to refer to community-based child and adolescent mental health services on a case-by-case basis.

Having discussed the different comorbidities found in children with CP, we must not neglect to highlight the fragile condition of these patients, so that while they may be stable for periods of variable duration, they are more vulnerable to experiencing decompensations due to intercurrent conditions that exacerbate the underlying disease. Therefore, it is important to take these resources into and make the necessary referrals in order to adjust treatment goals to the best interest of the patient in each situation.