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Cerebral Palsy

Background

Medical Management Considerations

Oral Health Considerations

References

Resources for Families

Advisory Committee

Publication Information

BACKGROUND

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Description and Cause

Cerebral palsy is “an umbrella term for a group of non-progressive, but often changing, motor impairment syndromes secondary to lesions or anomalies of the brain arising in the early stages of its development” (Kuban and Leviton 1994). Congenital cerebral palsy (90% of cases) may be caused by inadequate blood or oxygen supply to the fetus, illness during pregnancy, premature birth, birth trauma, etc. Acquired cerebral palsy (10% of cases) may be caused in the first few months after birth by head trauma, infections (encephalitis, meningitis, herpes simplex, cytomegalovirus), exposure to toxic substances, severe malnutrition, child abuse, etc. Evidence suggests that cerebral palsy does not result from the hazards of neonatal intensive care, as has been suggested.

Occurrence

1.5 to 2.5:1,000 children by early school age

Classifications of Cerebral Palsy

Although the classification of types of cerebral palsy is not uniform and there is not a standard upon which all clinical observers agree, it can be classified according to the type of abnormal muscle tone or movement, or according to the region of the body involved.

Note: Definitive diagnosis of cerebral palsy may not be possible until 15 to 24 months of age; however, the presence of spasticity, hypotonia, dystonia or athetosis warrants referral for evaluation and treatment services; and the presence of functional delays warrants referral for early intervention services.

  1. Spastic Cerebral Palsy (50% of cases)

Extremities Involved: diplegia (mainly both lower extremities), quadriplegia (all four extremities plus trunk and neck), or hemiplegia (one arm and one leg on same side of body)

Neurologic Dysfunction: spasticity

Characteristic Features:

  • Abnormal persistence of newborn reflexes
  • Grasp reflex causing tight fists in infants
  • Increasing spasticity and rigidity of limbs
  • Diplegia only: may have normal intelligence, difficulty drawing and writing, cross-over of the legs
  • Quadriplegia only: seizures, severe mental retardation is usual
  • Hemiplegia only: may have average intelligence; seizures
  1. Athetoid Cerebral Palsy (20% of cases)

Note: There are very few new cases of athetoid cerebral palsy because its primary cause, hyperbilirubinemia, can now be identified and treated at birth.

Extremities Involved: quadriplegia

Neurologic Dysfunction: athetosis, hypotonia

Characteristic Features:

  • Weak and “floppy” muscles in infancy
  • Appearance of symptoms after 6 months
  • Abnormal hand positioning when infant reaches for objects
  • Unsteady balance and gait
  • Abnormal, involuntary and uncontrollable movements (twitching, twisting, writhing)
  • Hearing and speech deficiencies
  • Normal intelligence
  1. Ataxic Cerebral Palsy (10% of cases)

Extremities Involved: atonic diplegia or congenital cerebellar ataxia

Neurologic Dysfunction: hypotonia

Characteristic Features:

  • Diplegia only: severe mental retardation
  • Ataxia only: mild mental retardation, disturbed balance and depth perception
  • Difficulty with rapid or fine movements
  • Spasticity in late childhood
  • Tremors, unsteadiness, uncoordination and choreic movements
  1. Mixed Cerebral Palsy: A combination of the three types above.

Common Associations

The likelihood of associated conditions increases with the severity of the underlying Cerebral Palsy and is higher among people with quadriplegia than among those with diplegia or hemiplegia. For example, only a small number of persons with athetoid cerebral palsy have epilepsy because the basal ganglia are primarily involved; in contrast, up to 90% of individuals with spastic quadriplegia have epilepsy because the entire brain is usually affected.

In addition, many of these conditions are secondary results of immobility and therefore may be preventable (such conditions are indicated with a *).

  • Mental retardation
  • Learning disabilities
  • Seizure disorders
  • Vision impairment
  • Speech impairment
  • Hearing impairment
  • Scoliosis
  • Tonal problems
  • Bladder problems (urinary tract infection)
  • Excessive drooling
  • Poor nutrition
  • Depression
  • Joint contractures
  • Hip dislocation
  • *Joint, muscle or other pain
  • *Skin breakdown
  • *Gastrointestinal problems (oral motor dysfunction, gastroesophageal reflux, esophagitis, gastritis, ulcers, constipation)
  • *Pneumonia
  • *Osteoporosis

MEDICAL MANAGEMENT CONSIDERATIONS

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Note: These considerations are in addition to the normal medical care provided to an individual without cerebral palsy. Although many recommendations can be addressed through clinical examination by the primary care provider, a multidisciplinary team approach (primary care physician, neurologist, orthopedist, physical therapist, occupational therapist, pediatric orthotist, nurse, dentist or dental hygienist, social worker) is the standard of care for persons with Cerebral Palsy. Access to specialized multidisciplinary care may be available through California Children Services (CCS) for persons under age 21.

I. Management by Age

Ongoing (all ages)

  • Monitor for pain related to spasticity
  • Maintain a high level of clinical suspicion of seizures
  • Refer to a specialist for discussion of durable medical equipment options such as braces, splints, crutches, wheelchairs, lifts
  • Refer to a specialist for discussion of adaptive equipment such as special cups, special handles for eating utensils and toothbrushes, or for the use of a motorized toothbrush
  • Refer to an oral health professional for evaluation and recommendation of an oral health care plan including the engagement of caregivers in daily disease prevention practices as needed
  • Monitor muscle strength and function
  • Encourage adherence to a bladder voiding schedule
  • Ensure safe physical environments for persons with poor motor control
  • Monitor nutritional status
  • Monitor for scoliosis
  • Treat skin injuries promptly
  • Teach pressure-relieving techniques
  • Encourage and assess self-care skills
  • Monitor speech and language progress
  • Discuss augmented communication options (or refer to speech and language therapist)
  • Emphasize importance of normal, unrestrictive environments
  • Encourage interaction with nondisabled peers

Infancy (Birth to 1 year)

  • Perform complete physical and neurological exam to detect congenital anomalies
  • Recommend physical and occupational therapy evaluation
  • Recommend an oral health assessment by age 1
  • Monitor for gastroesophageal reflux
  • Refer to early intervention programs and cerebral palsy/disability support groups
  • Discuss possibility of SSI enrollment

Childhood (1 to 13 years)

  • Recommend periodic physical and occupational therapy consultation; ongoing therapy as needed
  • Refer to orthopedic surgeon to assess need for surgery or other intervention
  • Recommend speech and language therapy evaluation
  • Recommend the establishment of and communication about an oral health care plan
  • Monitor school progress
  • Recommend and arrange for annual dental care exams and cleanings, caries risk assessment as needed, and daily disease prevention practices

Adolescence and Adulthood (13 years and over)

  • Implement recommendations of speech, physical and occupational therapists
  • Watch for functional losses in movement and mobility
  • Discuss alternative community living resources
  • Discuss long-term financial plans
  • Monitor oral health status, caries risk, and nutrition
  • Monitor prevocational training and vocational activities
  • Discuss community-supported employment opportunities
  • Discuss development of community mobility skills

II. Management of Specific Secondary Conditions

Spasticity

Consider these possible interventions as appropriate:

  • Oral medications (diazepam, dantrolene sodium, baclofen)
  • Intrathecal medications (Baclofen pump)
  • Alcohol (phenol) blocks
  • Botox (Botulinum toxin) injections into affected muscles
  • Nerve blocks
  • Selective dorsal rhizotomy
  • Orthopedic surgery (deep tendon releases, osteotomies)
  • Low intensity, low frequency electrical stimulation

Seizures

  • Maintain a high level of clinical suspicion of seizures
  • Prescribe standard antiepileptic medications
  • Monitor medication levels and effectiveness
  • Prevent status epilepticus through the use of rectal benzodiazepines when necessary
  • Vagal nerve stimulation is an option for refractory epilepsy

Gastrointestinal Conditions

  • Encourage normal GI function through dietary management including adequate nutrition and regular exercise
  • Ensure adequate intake of fiber and fluids for bowel control
  • Assist in the development of a bowel program and encourage regular bowel movements after meals (may require use of suppositories, enemas or manual extraction of stool)
  • Recommend upright positioning during and immediately after feeding
  • Consider small, frequent meals
  • Consider evaluation for feeding/swallowing difficulties, supervision during oral feeding, and swallowing therapy
  • Prescribe anti-ulcer medications, if indicated

Respiratory Conditions

  • Monitor swallowing function
  • Monitor carefully for scoliosis
  • Watch closely for signs of pneumonia
  • Treat pneumonia with antibiotics; use oxygen or assisted ventilation when necessary

ORAL HEALTH CONSIDERATIONS

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A thorough knowledge of the dental implications of cerebral palsy will aid a primary care provider and dental practitioner in preventing dental problems and providing the most appropriate treatment. Treatment options and limitations, as well as daily measures to take to prevent dental problems and maintain oral health, should be clearly explained to persons with developmental disabilities and their families/caregivers. Dental caries is an infectious and transmissible disease. Every person should have an oral health care plan by age 1 to prevent early childhood caries and later periodontal disease.

Dental Conditions Associated with Cerebral Palsy

  • Drooling (10-37%), primarily due to swallowing dysfunction
  • Increase in dental caries due to
  • Poor oral hygiene, physical difficulty with brushing/flossing teeth
  • Soft diet
  • Enamel hypoplasia (incomplete development or underdevelopment of the enamel)
  • Mouth breathing
  • Anti-drooling medications leading to xerostomia
  • Use of sugar-laden foods, e.g. soft drinks, jelly beans, etc., throughout the day and evening as behavioral reinforcers
  • Retaining food in the mouth longer due to dysphagia
  • Use of liquid medications with high sugar content
  • Increased risk of periodontal disease due to
  • Gingival hyperplasia (gum overgrowth) as a result of phenytoin (Dilantin) therapy
  • Poor oral hygiene due to immobility or greater dependence on others and other factors listed above
  • Malocclusions due to decreased muscle guidance of erupting teeth
  • Tongue thrust
  • Dental trauma particularly to anterior teeth, fractured teeth
  • Bruxism (compulsive grinding or clenching of the teeth)
  • Temporomandibular joint (TMJ) disorders
  • Dry lips due to mouth breathing
  • Decreased parotid flow rate

Dental Management

Managing the preventive, restorative, and surgical aspects of dental treatment involve an early (by age 1) and regular oral health evaluation and the establishment of a customized oral health care plan. Dental treatment plans should consider the limited motor skills and degree of control of the upper extremities of the patient. In addition, the patient may or may not be able to provide consent for the dental treatment.

Tooth decay (dental caries) is due to a variety of factors including acidogenic bacteria, presence of fermentable carbohydrates, and impaired ability of the saliva to buffer the acid. Fillings alone do not address the bacteria or the poor buffering capacity of the saliva. An appropriate caries risk assessment and an oral health plan are necessary to address the cause of the oral disease, including periodontal disease.

Clinical Considerations

  • The approach to dealing with dental decay in persons with cerebral palsy differs little from the general population, including the professional application of sealants, except for the need to involve caregivers in supervising or performing preventive procedures for some individuals.
  • Individuals using wheelchairs may need to be transferred from wheelchair to dental chair.
  • To protect the airway in patients with swallowing dysfunction or aspiration problems, patients should not be placed in a supine position; placement at a 45-degree angle is acceptable.
  • Dental chairs should be moved slowly and movements of the individual�fs head should be minimized to reduce spastic muscle responses.
  • Rapid set impression materials are helpful.
  • Stabilizing/protective devices or restraints may be needed due to uncontrollable body movement.
  • Muscle relaxants, sedation, general anesthesia, or hospitalization may be required to provide treatment. If a sedative is needed, one with a muscle relaxant component is a logical choice.
  • Tablets/capsules are often better managed than liquid medications.
  • Since patients may be receiving anti-epileptics, medications for spasticity, and/or other drugs, consider drug interactions prior to using additional medications, sedatives, or anesthetics.
  • Use of removable prosthetic devices is not contraindicated in people with uncontrolled seizures or ataxia provided those appliances are tooth born, well fitting, and have good retention. Both safety issues and concerns of patient self-esteem should be considered.
  • A dentist may consider creating a mouth guard for individuals with seizures to protect the individual�fs teeth in the case of a fall.
  • Many people with cerebral palsy who have uncontrolled head movements can nevertheless be treated in a dental office if the dental provider uses good hand and finger rests and positions the patient to be able to move with them.
  • Preventive measures include topical fluoride applications, dietary counseling, sealants, and periodic professional prophylaxis.

At-Home Care

  • Establish a customized oral health care plan that includes good daily oral hygiene procedures including plaque removal by brushing and flossing and the use of adjunct aids as needed, such as various fluoride products, Xylitol, and fluoride varnish.
  • Modify usual methods of brushing/flossing teeth for individuals who cannot perform these procedures in the usual manner.
  • If patient will not cooperate with brushing, teach caregiver to use desensitization by starting with some wet gauze or a washcloth on the mouth and gradually moving to a soft, dry toothbrush, then a wet toothbrush, and finally a toothbrush with toothpaste.
  • Modify toothbrush, use specially designed toothbrushes or floss holders with modified handles to facilitate grasping. A toothbrush or floss holder handle can be modified to make it easier to grasp by pushing the handle through a rubber ball or by attaching the handle to a bicycle grip with plaster of Paris.
  • Instruct caregivers to position the individual on a couch with their head in the caregivers lap to assist the individuals needing help to remove plaque. Use a lightly moistened brush without toothpaste and use a floss holder for patients who cannot rinse/expectorate or perform these procedures themselves.
  • Promote diet that does not increase the risk of dental decay (e.g., reduces the regular exposure to fermentable carbohydrates, etc.).
  • Consider diet counseling for a patient with significant tooth decay.
  • Consider diet consistency and sugar content of liquid medications.
  • Avoid prolonged use of bottles containing fermentable carbohydrates and provide instructions to never put a child to sleep with a bottle containing fermentable carbohydrates.
  • Use fluoridated water and toothpaste. Recommend daily fluoride tablets or drops if water supply does not contain recommended amount of fluoride.
  • Use gum, mints, or foods containing Xylitol as the primary sugar to reduce caries.
  • Use antimicrobial (Chlorhexidine) mouthwash or spray as an adjunct for some individuals with rampant caries and gingivitis.

REFERENCES

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Peer-reviewed Journal Articles/Academies

Ashwal, S., et al. (2004). Practice Parameters: Diagnostic Assessment of the Child with Cerebral Palsy. Neurology, 62(6), 851-863.

Cooley, WC., et al. (2004). Providing a Primary Care Medical Home for Children and Youths with Cerebral Palsy. Pediatrics, 114(4), 1106-1112.

Dzienkowski, RC, et al. (1996). “Cerebral Palsy: A Comprehensive Review.” Nurse Practitioner, 21(2),45-59.

Grether, J.K., Nelson, KB. (1997). Maternal Infection and Cerebral Palsy in Infants of Normal Birth Weight. Journal of the American Medical Association, 278(3), 207-211.

Kuban, K.C.K., Leviton, A.(1994). Medical Progress: Cerebral Palsy. The New England Journal of Medicine, 330(3) , 188-195.

Michaud, LJ. et al. (2004). Prescribing therapy services for children with motor disabilities. Pediatrics, 113(6), 1836-1838.

Lollar, D. (1994). Preventing Secondary Conditions Associated With Spina Bifida or Cerebral Palsy: Proceedings and Recommendations of a Symposium. Washington, D.C.: Spina Bifida Association of America.

Special Interest Groups/Other Publications

Batshaw, Mark L. and Yvonne M. Perret. (1992). Children with Disabilities(pp. 441-466).. Baltimore: Paul H. Brookes Publishing.

Dorval, Jane. (1994). Achieving and Maintaining Body Systems Integrity and Function: Clinical Issues.” Crystal City, Virginia: Centers for Disease Control and Prevention, Spina Bifida Association of America, United Cerebral Palsy Associations, Inc., & Shepherd Spinal Center.

Eastern Los Angeles Regional Center. (1998). Wellness Initiative (July).

Miller, Freeman and Steven J. Bachrach. (1995). Cerebral Palsy: A Complete Guide for Caregiving. Baltimore: The Johns Hopkins University Press.

RESOURCES FOR FAMILIES

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American Academy for Cerebral Palsy and Developmental Medicine, 847�E98-1635 ,

http://www.aacpdm.org/index?service=page/Home

American Dental Association, 312-440-2500,

http://www.ada.org

Tips for People Who Have Special Needs, http://www.ada.org/public/topics/special_needs.asp#overview

Brain Injury Association of California, 800-457-2443

California Children Services (CCS), 916-654-0499,

http://www.dhs.ca.gov/pcfh/cms/ccs/

California Department of Developmental Services, 916-654-1690,

http://www.dds.ca.gov

California Department of Health Services,

http://www.dhs.ca.gov/

California Regional Centers, 915-654-1958,

http://www.dds.cahwnet.gov/rc/RCInfo.cfm

Easter Seals National Office, 800-221-6827,

http://www.easterseals.com/site/PageServer

Exceptional Parent Magazine, 800-EPARENT,

http://www.eparent.com/

March of Dimes Birth Defects Foundation, 914-428-7100,

http://www.marchofdimes.com/

M.I.N.D. Institute (Medical Investigation of Neurodevelopmental Disorders), 888-883-0961,

http://www.ucdmc.ucdavis.edu/mindinstitute/

National Oral Health Information Clearinghouse, 301-402-7364, TTY 301-656-7581,

http://www.nidcr.nih.gov/

National Oral Health Information Clearinghouse

Oral Conditions in Children with Special Needs, A Guide for Health Care Providers

http://www.nidcr.nih.gov/HealthInformation/SpecialCareResources/SpecialCareinOralHealth.htm

National Rehabilitation Information Center, 800-346-2742,

http://www.naric.com/

Southern Association of Institutional Dentists,

http://saiddent.org/modules.asp

Special Care Dentistry, 312-440-2660,

http://www.scdonline.org

Special Olympics Special Smiles,

http://www.specialolympics.com/Special+Olympics+Public+Website/English/Initiatives/Healthy_Athletes/Special+Smiles/default.htm

A Guide to Good Oral Health for Persons with Special Needs,

http://www.specialolympics.org/Special+Olympics+Public+Website/English/Initiatives/Healthy_Athletes/Special+Smiles/Oral_Health_Guide/default.htm

United Cerebral Palsy Associations, Inc., 800-872-5827,

http://www.ucpa.org

University of Florida, College of Dentistry, Department of Pediatric Dentistry

Oral Health Care for Persons with Disabilities,

http://www.dental.ufl.edu/Faculty/Pburtner/disabilities/Default.htm

University of the Pacific, School of Dentistry

Center for Oral Health for People with Special Needs,

http://www.cfoh.org

or

Community Programs

http://dental.pacific.edu/

ADVISORY COMMITTEE

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Theodore A. Kastner, M.D., M.S.

Felice Weber Parisi, M.D., M.P.H.

Joan M. Reese, M.D., M.P.H.

Larry Yin, M.D., M.S.P.H., F.A.A.P.

PUBLICATION INFORMATION

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Funded by a grant from the California Department of Developmental Services

For more information, contact:

Center for Health Improvement

1330 21st Street, Suite 100

Sacramento, CA 95814

(916) 901-9645

This document does not provide advice regarding medical diagnosis or treatment for any individual case, and any opinions or statements contained in this document are not intended to serve as a standard of medical care. Physicians are encouraged to view the considerations presented in this document in light of evolving scientific information. This document is not intended for use by the layperson. Reproduction of this document may be done with proper credit given to California Department of Developmental Services and the Center for Health Improvement.

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