Disease/Medical Condition

CEREBRAL PALSY Date of Publication: Dec. 20, 2017

(also known as “CP”; the spastic form of CP is also known as “spastic ”; includes “ spastic CP”, “dyskinetic/choreo-athetoid CP”, “ataxic CP”, and “mixed CP .”)

Is the initiation of non-invasive dental hygiene procedures* contra-indicated? No.

■ Is medical consult advised? No, — assuming patient/client is already under medical care for , which is well managed, and — assuming that seizure disorder (if any) is well controlled.

Is the initiation of invasive dental hygiene procedures contra-indicated?** Possibly, but not typically.

■ Is medical consult advised? ...... See above. Yes, if patient/client has a history suggestive of a need for pre-procedure medication for calming, involuntary muscle movement control, seizure control, or other behavioural challenges. ■ Is medical clearance required? ...... Possibly (e.g., if there are significant involuntary muscle movements creating a safety concern for the dental hygienist and/or the patient/client, or if there is significant risk of seizure). ■ Is antibiotic prophylaxis required? ...... No. ■ Is postponing treatment advised? ...... Possibly (e.g., if there are significant involuntary muscle movements creating a safety concern for the dental hygienist and/or the patient/client; also depends on severity and level of control of co-morbid seizure disorder, as well as presence/absence of oral pathology — such as tooth fractures — that may need to be addressed prior to dental hygiene treatment).

Oral management implications

■ Most persons with mild or moderate forms of cerebral palsy can be treated successfully in the general practice dental hygiene setting. However, provision of oral care often requires adaptation of routine skills. ■ Patients/clients in wheelchairs may be more easily treated in the wheelchair deploying wheelchair-lock wheels. A sliding board can be used to support back, head, and neck, while reclining the wheelchair if possible. ■ If a patient/client needs to be transferred from a wheelchair to the dental chair, the dental hygienist should ask about preferences such as pillows, padding, or other aids to ease the transfer. Placing the dental chair at a 45 degree angle can help protect the airway by avoiding the supine position. ■ Patients/clients taking muscle relaxants (e.g., and benzodiazepines) or anti-spasmodics (e.g., ) are at increased risk of hypotension, dizziness, and/or ataxia. To reduce the likelihood of a fall, the patient/client should be assisted to and from the chair. The dental hygienist should also be cautious when adjusting the dental chair, with inclination occurring slowly for re-equilibration. ■ The degree of intellectual disability, if any, varies with each patient/client. Therefore, explanation of procedures and dental hygiene education should be tailored according to the individual needs of the patient/client. The dental hygienist should be empathetic regarding the patient/client’s frustrations and concerns. Consistency in all aspects of oral care (including the same dental hygienist and operatory over time) contributes to improved cooperation. ■ Dysarthria1 is common, and the dental hygienist should be patient and allow time for the patient/client to express himself/ herself. ■ Limbs should not be forced into unnatural positions. The patient/client should be allowed to settle into a position that is comfortable and does not interfere with dental hygiene treatment.

1 is difficult or unclear articulation of speech due to impaired movement of the muscles used for speech production.

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Disease/Medical Condition

CEREBRAL PALSY

(also known as “CP”; the spastic form of CP is also known as “spastic paralysis”; includes “ spastic CP”, “dyskinetic/choreo-athetoid CP”, “ataxic CP”, and “mixed CP ”)

Oral management implications (cont’d)

■ The patient/client’s involuntary muscle movements may create a safety issue for the dental hygienist, and proactive measures should be taken to address this. The dental hygienist should try to anticipate the patient/client’s movements, blending professional movements with those of the patient/client or working around them. ■ For the most part, uncontrolled body movements should be tolerated, rather than attempting to stop them. Firm, gentle pressure can be applied to calm shaking limbs, particularly in children. ■ Lights, sounds, and sudden movements that trigger primitive reflexes2 or uncontrolled movements3 should be minimized. The patient/client should be informed about a stimulus before its appearance. Although relaxation will not stop involuntary body movements, it may reduce intensity and frequency. ■ The tonic labyrinthine reflex may be prevented by keeping the patient/client’s head supported and flexed, maintaining the chair in the upright position, and folding the patient/client’s hands at midline. Management involves bringing arms forward, separating legs, and massaging shoulders. ■ The asymmetric tonic neck reflex may be prevented by using rear operating position and stabilizing the head in midline. Management involves placing face in midline and helping flex extended arm and leg. ■ The startle reflex may be prevented by informing the patient/client before lowering, raising, or tilting the dental chair. ■ The patient/client’s head should be softly cradled during treatment. If the patient/client’s head needs to be turned, this should be done gently and slowly. ■ An early morning appointment, before eating or drinking, benefits a patient/client with a gagging problem. Hyperactive gag and bite reflexes necessitate gentle, slow introduction of dental hygiene instruments into the mouth. A mouth prop may be helpful. The chin should be placed in a neutral or downward position to mitigate hyperactive gag reflex. ■ Appointments should ideally be short with frequent breaks. Muscle relaxants may be indicated if long procedures are needed. If extensive dental treatment is required, persons with CP may need sedation, general anaesthesia, or hospitalization4. ■ Patients/clients who are tube-fed are at high risk of aspiration in the dental chair. Therefore, such patients/clients should be positioned as upright as possible, utilizing low amounts of water with high volume suction. ■ Seizure management protocol should be in place prior to arrival of patients/clients at risk. ■ Gastroesophageal reflux is common, and therefore the dental hygienist should be alert for teeth sensitivity and signs of erosion. ■ Xerostomia can be managed with saliva substitutes. ■ Malocclusion should prompt orthodontic needs assessment, followed by treatment as feasible. ■ Mouth guards should be considered for treatment of bruxism if gagging and dysphasia permit comfortable and practical use.

2 Primitive reflexes are common and may complicate oral care. There are 3 main types of such reflexes: asymmetric tonic neck reflex (when a patient/client’s head is turned away from the midline, the arm and leg on that side extend and stiffen while the contralateral arm and leg flex); tonic labyrinthine reflex (when the neck is tilted backwards losing support or is extended while a patient/client is lying on his or her back, the legs and arms extend and stiffen while the back and neck arch); and startle reflex (any surprising stimulus can trigger uncontrolled, often forceful movements involving the whole body). 3 Emotional stress can worsen involuntary movements in dyskinetic CP, including grimacing and squirming. 4 While local anaesthetics can generally be used without adverse reaction, some muscle relaxants and anticholinergics used in management of CP can cause (CNS) depression and potentiate other CNS depressants used in dentistry. Therefore, conscious sedation is generally not recommended. However, general anaesthesia, with appropriate precautions, may be required to accomplish restorative or surgical treatment.

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Disease/Medical Condition

CEREBRAL PALSY

(also known as “CP”; the spastic form of CP is also known as “spastic paralysis”; includes “ spastic CP”, “dyskinetic/choreo-athetoid CP”, “ataxic CP”, and “mixed CP ”)

Oral management implications (cont’d)

■ Manual dexterity should be assessed to develop an oral self-care plan. An adapted toothbrush or electric toothbrush, as well as floss holder, may be indicated. ■ The caregiver should be engaged regarding oral home care, especially so in the case of a child with CP5. Brushing in a supine position with a mouth prop may prove useful. Rinsing with fluoride or chlorhexidine should be monitored. ■ Persons with CP are prone to falls or accidents that result in trauma and injury to the mouth. Therefore, the dental hygienist can be proactive in suggesting a tooth-saving kit (e.g., in group homes) and in giving specific directions on what to do if a permanent tooth is knocked out. ■ Because physical abuse often presents as oral trauma and occurs more commonly in persons with developmental disabilities than the general population, the dental hygienist should be alert to suspicious oral trauma during the examination. Findings should be noted in the chart and any suspected abuse or neglect in children should be reported to the Children’s Aid Society as required by law6.

Oral manifestations

■ The more severe the neurological insult, the higher is the risk of dental and other oral disease. ■ Most oral clinical findings result from disturbances of the oral musculature. Spasticity and rigidity of the muscles of the mouth, tongue, and pharynx occur in spastic CP. ■ Abnormal functioning of the tongue, lips, and cheeks can inhibit oral clearance of food, and this may be exacerbated by the consumption of a soft, carbohydrate rich diet. ■ Malocclusion results from dysfunction of the facial, masticatory, and lingual musculature, which is exacerbated by tongue thrusting. Overjet and overbite occur with increased incidence (i.e., Class II Div I malocclusion). ■ An open bite with protruding anterior teeth is common and usually associated with tongue thrusting (especially in dyskinetic CP). ■ Enamel hypoplasia and delayed eruption of permanent teeth are developmental manifestations. ■ Other issues include mouth breathing, hypoactive or hyperactive gag and bite reflexes, oral hypersensitivity (to touch, taste, or smell), dysphagia, dysarthria, sialorrhea, drooling, exaggerated and prolonged bite reflexes, bruxism, food pouching, and temporomandibular joint disorders. ■ Dental caries and periodontal disease occur at elevated rates due to poor oral hygiene and complications of physical abilities and malocclusion. ■ Dental caries and periodontal disease occur at elevated rates due to poor oral hygiene and complications of physical abilities and malocclusion. ■ Dental erosion, thermal sensitivity, and pain can result from gastroesophageal reflux (GERD). ■ Gingival hyperplasia may occur in patients/clients taking phenytoin for seizure disorder. Other side effects of anticonvulsants include xerostomia dysgeusia, and stomatitis.

5 For young children with CP, caregivers should learn to gently cleanse the incisors daily with a soft cloth or an infant soft toothbrush. For older children who are unwilling or physically unable to cooperate, the dental hygienist should teach proper brushing techniques to the caregiver in conjunction with ways to safely restrain the child when necessary. For example, the child may be placed in the caregiver’s lap to stabilize the head with one hand while using the other hand to brush the teeth. An older child may recline on a chair or bed while the caregiver angles the head backward with one hand while the teeth are brushed with the other hand. 6 in accordance with the Ontario’s Child and Family Services Act

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Disease/Medical Condition

CEREBRAL PALSY

(also known as “CP”; the spastic form of CP is also known as “spastic paralysis”; includes “ spastic CP”, “dyskinetic/choreo-athetoid CP”, “ataxic CP”, and “mixed CP ”)

Oral manifestations (cont’d)

■ Xerostomia may result from /muscle relaxants7 used to control spasticity and rigidity or from anticholinergics8 or central nervous system agents9 used to manage involuntary body movements or drooling. ■ Excessive drooling may be caused by benzodiazepines10, which are used to relax muscles, decrease muscle , relieve anxiety, and manage agitation and seizures. ■ Fractures of the anterior maxillary teeth are common due to uncoordinated ambulation and seizures that result in frequent falls. This situation is worsened by the lack of lip protection that occurs with protrusive teeth. ■ Patients/clients who are tube-fed often have low caries, rapid accumulation of calculus, GERD, and oral hypersensitivity. ■ Patients/clients with concurrent pica11 may exhibit related damage to oral soft tissue and teeth.

Related signs and symptoms

■ CP encompasses a group of chronic neurologic disorders caused by non-progressive damage to the developing that originates during the prenatal or perinatal period or during first few years of life12. Physical disability results. ■ In addition to the posture and movement disorders that impair functional mobility and typify cerebral palsy, persons with CP may also have disturbances of sensation, perception, communication, and cognition, depending on the nature of the brain abnormality. ■ The 4 major CP subtypes13 are:  spastic (the most common form, characterized by muscle stiffness, resistance to passive movement, , and sudden, involuntary muscle contractions or spasms)14;  dyskinetic/choreo-athetoid (involuntary, uncontrolled muscle movement that may be slow and writhing; often results in children having very weak muscles and feeling “floppy” when carried)15;

7 such as , baclofen, and clonidine 8 such as benztropine, glycopyrrolate, and trihexyphenidyl 9 such as levodopa (which is converted to dopamine in the brain) 10 such as , alprazolam, and triazolam 11 Pica is the compulsive eating of non-edible substance, such as dirt, sand, and paint chips. 12 Most congenital cases are caused by damage to the brain during pregnancy or delivery. Congenital risk factors are premature birth, multiple births, maternal infections, maternal exposure to toxic substances, and blood type incompatibility. Acquired cases during the first few months or years of life may be caused by brain infections (e.g., meningitis) or head injury (e.g., from fall, motor vehicle collision, or child abuse). It is rarely possible to diagnose CP in infants less than 6 months of age except in very severe cases; in many cases, diagnosis is not possible until 12 months, informed collectively by signs of delayed motor milestones, abnormal , persistence of primitive reflexes, and abnormal postural movement. may be documented by cranial ultrasound, computed tomography (CT scan), or magnetic resonance imaging (MRI). 13 The location of the brain injury influences how movement is affected. 14 Spastic CP is caused by damage to the frontal lobe motor area of the cerebral cortex, and it can involve different parts of the body. In , both legs are affected more than the arms (most common in premature babies). In hemiplegia, the leg and arm on one side of the body are affected (occurs in babies who have had a or trauma to one side of the brain). In quadriplegia, all four limbs are affected, as may be the muscles of the face, mouth, and torso (most common in babies who have experienced oxygen deprivation in the brain). 15 Dyskinetic CP is caused by damage to the basal ganglia (the parts of the brain responsible for smooth coordinated movements and body posture). This form of CP usually only involves the motor centres of the basal ganglia without intellectual impairment.

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Disease/Medical Condition

CEREBRAL PALSY

(also known as “CP”; the spastic form of CP is also known as “spastic paralysis”; includes “ spastic CP”, “dyskinetic/choreo-athetoid CP”, “ataxic CP”, and “mixed CP ”)

Related signs and symptoms (cont’d)

 ataxic (poor coordination/balance sometimes accompanied by tremors; patients/clients typically walk with a wide-based gait and may have problems with precise or quick movements)16; and  mixed (often spastic-athetoid, usually initially manifesting with spasticity, with involuntary movement increasing as the child develops). ■ Prevalence in children is less than 1% (with boys being affected slightly more than girls)17, but incidence is increasing due to modern medical technology that improves survival in the perinatal period. More than 2600 new diagnoses occur annually in Ontario, and more than 34,000 Ontarians live with CP, with an average age of 30 years. While some children die in infancy, most persons with CP have a normal or near-normal life expectancy. ■ Some affected persons appear to have few obvious effects, whereas others may be severely incapacitated necessitating mobility devices and personal attendants to assist them with activities of daily living18. ■ Delayed motor development and persistent primitive reflexes are common. ■ Speech/communication disorders (such as dysarthria in dyskinetic CP) and/or vision19/hearing impairment may result from the brain injury that caused CP. ■ Intellectual disability is present in about 60% of persons with CP. ■ Seizure disorder is present in 30% to 50% of children with CP. ■ Associated medical problems include difficulty eating, poor nutrition, regurgitation, vomiting, aspiration, breathing problems, poor bowel and bladder control, and pressure sores.

References and sources of more detailed information

■ CDHO Advisory http://www.cdho.org/Advisories/CDHO_Advisory_Cerebral_Palsy.pdf ■ Sehrawat N, Marwaha M, Bansal K, Chopra R. Cerebral Palsy: A Dental Update. International Journal of Clinical Pediatric Dentistry. 2014;7(2):109-118. doi:10.5005/jp-journals-10005-1247. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4212167/ ■ Jan BM, Jan MM. Dental health of children with cerebral palsy. Neurosciences. 2016;21(4):314-318. doi:10.17712/ nsj.2016.4.20150729. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5224428/ ■ National Institute of Dental and Craniofacial Research, National Institutes of Health https://www.nidcr.nih.gov/oralhealth/Topics/DevelopmentalDisabilities/PracticalOralCarePeopleCerebralPalsy.htm ■ National Institute of Neurological Disorders and Stroke, National Institutes of Health https://www.ninds.nih.gov/Disorders/All-Disorders/Cerebral-Palsy-Information-Page

16 Ataxic CP is caused by damage to the cerebellum (the part of the brain responsible for balance and coordination of voluntary movement). 17 CP affects about 1 in every 400 babies to some degree, with this statistic increasing up to 1 in 3 for premature births. 18 Management approaches to CP and its associated conditions include ; occupational therapy; speech and language therapy; biofeedback; , casts, and splints; medications (such as drugs to control seizures, spasticity, or spasms); (such as orthopaedic surgery and soft tissue surgery to counter effects of spasticity or to enable the child to move more easily, as well as to reduce spasticity); adaptive equipment (such as mobility aids and communication devices); and daily living aids (such as large-handled eating utensils). 19 Strabismus (in which eyes are crossed or misaligned) is the most common visual impairment in persons with CP. Visual motor skills development, such as hand-eye coordination, may lag that of other people. cont’d on next page...

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Disease/Medical Condition

CEREBRAL PALSY

(also known as “CP”; the spastic form of CP is also known as “spastic paralysis”; includes “ spastic CP”, “dyskinetic/choreo-athetoid CP”, “ataxic CP”, and “mixed CP ”)

References and sources of more detailed information (cont’d)

■ Medscape http://www.medscape.com/viewarticle/836976 (Oral Health and the Child With Cerebral Palsy). ■ School of Dentistry University of Washington http://dental.washington.edu/wp-content/media/sp_need_pdfs/CP-Dental.pdf ■ Ontario Federation for Cerebral Palsy https://www.ofcp.ca http://ofcp.ca/wp-content/uploads/2016/05/what-is-CP-2015.pdf ■ Institute for Clinical Evaluative Sciences + Ontario Brain Institute https://www.ices.on.ca/~/media/Files/.../Brain-Disorders-in-Ontario/Full-Report.ashx ■ Medscape http://www.medscape.org/viewarticle/576698_4 (Spasticity: A Clinical Review) ■ Darby M (ed.) and Walsh M (ed.). Dental Hygiene: Theory and Practice (4th edition). St. Louis: Elsevier Saunders; 2015.

* Includes oral hygiene instruction, fitting a mouth guard, taking an impression, etc. ** Ontario Regulation 501/07 made under the Dental Hygiene Act, 1991. Invasive dental hygiene procedures are scaling teeth and root planing, including curetting surrounding tissue.

Date: September 18, 2017

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