Cerebral Palsy: A Multisystem Review: Page 3 of 3
Cerebral Palsy: A Multisystem Review: Page 3 of 3
General. Management of the child with CP begins with a comprehensive medical evaluation by a multidisciplinary team that includes a pediatrician; a pediatric neurologist; physical, occupational, and speech therapists; an audiologist; an ophthalmologist; a social worker; and possibly a neurosurgeon and orthopedic surgeon. Family members are the most important part of the treatment team. Hearing tests and early referrals to audiologists and speech therapists are necessary to prevent delays in language and learning skills. Use of electronic communication devices may help alleviate frustration caused by communication difficulties. Screening for visual defects and ophthalmologic referrals should take place within the first year of life. Cognitive delays warrant careful assessment, initiation into early intervention programs, and the development of an individualized education plan in the face of severe motor and communication difficulties.
The goals of overall therapy should be aimed at maximizing the patient's level of function. Important areas of focus include ambulation, cognitive skills, activities of daily living, personal hygiene, and rehabilitation into society.15 The primary care physician will likely play the central role in coordinating care, but the pediatric neurologist will be called upon for expert guidance.
Neurologic. Pharmacotherapeutic resources are available to manage symptoms such as spasticity and sleep disturbances and to manage seizures and movement disorders in general. Oral antispasticity medications include benzodiazepines, baclofen, a2-adrenergic agonists, and dantrolene sodium (Dantrium).33 Injectable medications include botulinum toxin A (Botox).
Invasive procedures include placement of an intrathecal infusion pump for continuous delivery of baclofen to the CNS for the treatment of severe spasticity. Several studies have shown that intrathecal baclofen therapy is safe and effective in children with CP.34-36
Another neurosurgical procedure for spasticity is highly selective dorsal root rhizotomy. This procedure attempts to cut fewer than 50% of the sensory fascicles from each cauda equine nerve root, guided by intraoperative neuromuscular testing to identify the most abnormal nerve fascicles that should undergo ablation.37-39
Few studies have directly compared the safety, efficacy, and long-term outcomes or relative costs of dorsal root rhizotomy and the baclofen pump. Therefore, the decision to use either of these interventions must be made on a case-by- case basis.40 Seizure control with antiepileptic drugs or use of a ketogenic diet or surgery also should be considered on a case-by-case basis, depending on patient presentation and seizure type.
Therapies for dystonia include anticholinergics and dopamine agonists. Sleep problems, which can be attributed to pain and motor dysfunction, can be managed with melatonin and other sleep aids and by simply establishing an appropriate bedtime routine.
Musculoskeletal. Management of the musculoskeletal sequelae of CP should include the participation of the pediatric orthopedic surgeon and physical therapist. Treatment should be tailor-made for the specific orthopedic abnormality present. Consultation with the specialists will determine optimum timing and type of treatment.
Oromotor. Of various strategies studied to reduce plaque and gingivitis in patients with CP, an electric toothbrush was found most effective in optimizing oral hygiene.41 The use of cryotherapy to enhance mouth opening by providing a temporary reduction in masseter spasticity has been shown to improve oral access and thus facilitate preventative dental care and other dental procedures.42
Occupational and behavioral therapy using oral stimulation techniques, such as stroking, tapping, and blowing exercises, has been shown to improve drooling.43 The centrally acting antispastic agent modafinil (Provigil)44 and anticholinergic medications such as scopolamine, glycopyrrolate (Robinul), and benzotropine improve drooling but are associat- ed with significant systemic adverse effects.45 Ultrasound-guided intraglandular injection of botulinum toxin A can successfully reduce drooling without major adverse effects.45 Surgical options include submandibular gland excision, parotid duct ligation, duct rerouting, and transtympanic neurectomy.46
Placement of gastrostomy tubes (G-tubes) for non-oral feeding should be considered in patients at high risk for chronic undernourishment because of severe impairment of oromotor function. Oral feeding interventions for such patients have not been shown to be effective in promoting feeding efficiency or weight gain.47 Although concerns have been raised, no correlation between respiratory problems and G-tube placement has been found.48
The placement of a G-tube has been shown to improve the caregiver's quality of life as measured by significantly reduced length of feeding times, increased ease of drug administration, and reduced parental concern regarding the child's nutritional status.49 Consultation with a nutritionist may be of benefit for the child with CP who experiences either poor weight gain or obesity.
Cardiopulmonary. Any evidence of heart or lung disease should be aggressively evaluated. Pediatric cardiology consultation should be sought for definitive diagnosis and treatment of the cardiac abnormality. Effective pulmonary toileting and administration of antibiotics, including anaerobic coverage, are essential for the treatment of pneumonia in the patient with CP.
GI. In patients with GERD in whom medical management with proton pump inhibitors fails, Nissen fundoplication may be undertaken. A study50 of gastrojejunal tube placement suggested that the surgical procedure may be equally helpful in improving GERD. Administering baclofen orally or via a nasogastric tube to reduce symptoms of GERD also has been shown to reduce the frequency of emesis and the total number of events of acid reflux.51
Treatment of chronic constipation demands a multifaceted approach that includes a regular toileting schedule, proper toilet positioning, dietary modifications, and medication. In patients with impacted stool, it is important to begin with a "clean-out" regimen using either oral osmotic agents or serial enemas. After the impacted stool is removed, a maintenance program using titrated agents such as polyethylene glycol to achieve daily soft stool should be continued on a long-term basis. Oral mineral oil should not be used because of risk of aspiration.
Genitourinary. Highly selective dorsal root rhizotomy may improve spasticity and bladder storage capacity in appropriate patients. Neurogenic detrusor overactivity--the most common finding in patients with lower urinary tract symptoms--can most often be controlled with a clean intermittent catheterization protocol and concomitant anticholinergic medication.28
In regard to precocious puberty, a pediatric endocrinologist may be consulted. Gonadotropin-releasing hormone agonists remain the mainstay of treatment.52
Older female patients in particular have unique medical needs, including contraceptive care and breast cancer screening. Mammography is underutilized by women with CP.53 Barriers to care include communication problems, transport, and physical limitations requiring adaptation of mammographic technique. Similar barriers also have been associated with access to contraceptive care in this patient population.54 Those patients who are taking antiepileptic agents for management of concomitant seizure disorders and who request contraceptive care should be counseled about the decreased effectiveness of oral contraceptives when used with certain antiepileptic drugs. The increased risk of thrombosis in women who have limited mobility also should be considered before initiating oral contraceptives.55
Behavioral. An integrative team approach, including monthly meetings between the rehabilitation therapist, child psychiatrist, developmental pediatrician, psychologist, and a preventative medicine specialist, has been shown to improve standardized measures of family stress associated with the parents' attitude toward the child with a disability. It also has shown significant improvements in the subscales of the Vineland Adaptive Behavior Scales assessment of the child.56
Mood and behavioral disturbances are treated on a case-by-case basis, with behavior modifications and medication that depend upon the individual patient's circumstances. If pain is the possible trigger for the behavior change, for example, management should focus on uncovering and treating the underlying cause.
1. Zaffuto-Sforza CD. Aging with cerebral palsy. Phys Med Rehabil Clin N Am. 2005;16:235-49.
2. Curless RG. Diagnostic problems in three pediatric neurology practice plans. Pediatr Neurol. 1998;19:272-274.
3. Odding E, Roebroeck ME, Stam HJ. The epidemiology of cerebral palsy: incidence, impairments and risk factors. Disabil Rehab. 2006;28:183-191.
4. Nelson KB, Ellenberg JH. Antecedents of cerebral palsy: multivariate analysis of risk. N Engl J Med. 1986;315:81-86.
5. Toso L, Poggi S, Park J, et al. Inflammatory- mediated model of cerebral palsy with developmental sequelae. Am J Obstet Gynecol. 2005;193: 933-941.
6. Ashwal S, Russman BS, Blasco PA, et al; Quality Standards Subcommittee of the American Academy of Neurology; Practice Committee of the Child Neurology Society. Practice parameter: diagnostic assessment of the child with cerebral palsy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2004;62:851-863.
7. Palmer FB. Strategies for early diagnosis of cerebral palsy. J Pediatr. 2004;145:S8-S11.
8. Khan A, Vasudevan A. Lesch-Nyhan Syndrome. Consultant for Pediatricians. 2006;5:322.
9. Hadders-Algra M. General movements: A window for early identification of children at high risk for developmental disorders. J Pediatr. 2004; 145:S12-S128.
10. Accordo J, Kammann H, Hoon A. Neuroimaging in cerebral palsy. J Pediatr. 2004;145:S19-S27.
11. Flett PJ. Rehabilitation of spasticity and related problems in childhood cerebral palsy. J Pediatr Child Health. 2003;39:6-14.
12. Sanger TD, Delgado MR, Gaebler-Spira D, et al. Classification and definition of disorders causing hypertonia in childhood. Pediatrics. 2003;111: e89-e97.
13. Walker WD, Johnson CP. Mental retardation: overview and diagnosis. Pediatr Rev. 2006;27:204-239.
14. Green L, Greenberg GM, Hurwitz E. Primary care of children with cerebral palsy. Clin Fam Pract. 2003;5:467-491.
15. Krigger KW. Cerebral palsy: An overview. Am Fam Physician. 2006;73:91-100.
16. Deluca PA. The musculoskeletal management of children with cerebral palsy. Pediatr Clin North Am. 1996;43:1135-1150.
17. Sakai T, Yamada H, Nakamura T, et al. Lumbar spinal disorders in patients with athetoid cerebral palsy: a clinical and biomechanical study. Spine. 2006;31:E66-E70.
18. Henderson RC, Kairalla JA, Barrington JW, et al. Longitudinal changes in bone density in children and adolescents with moderate to severe cerebral palsy. J Pediatr. 2005;141:69-75.
19. Dabney KW, Lipton GE, Miller F. Cerebral palsy. Curr Opin Pediatr. 1997;9:81-88.
20. Cooley WC. Providing a primary care medical home for children and youth with cerebral palsy. Pediatrics. 2004;114:1106-1113.
21. Su JM, Tsamtsouris A, Laskou M. Gastroesophageal reflux in children with cerebral palsy and its relationship to erosion of primary and permanent teeth. J Mass Dent Soc. 2003;52:20-24.
22. Reilly S, Skuse D, Poblete X. Prevalence of feeding problems and oral motor dysfunction in children with cerebral palsy: a community survey. J Pediatr. 1996;129:877-882.
23. Stallings VA, Charney EB, Davies JC, Cronk CE. Nutrition-related growth failure of children with quadriplegic cerebral palsy. Dev Med Child Neurol. 1993;35:126-138.
24. Chong SK. Gastrointestinal problems in the handicapped child. Curr Opin Pediatr. 2001;13:441-446.
25. Del Guidice E, Staiano A, Capano G, et al. Gastrointestinal manifestations in children with cerebral palsy. Brain Dev. 1999;21:307-311.
26. Bohmer CJ, Klinkenberg-Knol EC, Niezen-de Boer MC, Meuwissen SG. Gastroesophageal reflux disease in intellectually disabled individuals: how often, how serious, how manageable? Am J Gastroenterol. 2000;95:1868-1872.
27. Park ES, Park CI, Cho SR, et al. Colonic transit time and constipation in children with spastic cerebral palsy. Arch Phys Med Rehabil. 2004;85:453-456.
28. Karaman MI, Kaya C, Caskurlu T, et al. Urodynamic findings in children with cerebral palsy. Int J Urol. 2005;12:717-720.
29. Worley G, Houlihan CM, Herman-Giddens ME, et al. Secondary sexual characteristics in children with cerebral palsy and moderate to severe motor impairment: a cross-sectional survey. J Pediatr. 2002;110:897-902.
30. Murphy N, Elias ER. Sexuality of children and adolescents with developmental disabilities. J Pediatr. 2006;118:398-403.
31. Guidry Tyiska C. Working With Victims of Crime With Disabilities. Washington, DC: Office for Victims of Crime, US Dept of Justice; 2001. Available at: www.ojp.usdoj.gov/ovc/publications/factshts/disable.htm. Accessed November 7, 2006.
32. McDermott S, Coker AL, Mani S, et al. A population-based analysis of behavior problems in children with cerebral palsy. J Pediatr Psychol. 1996;21:447-463.
33. Verrotti A, Greco R, Spalice A, et al. Pharmacotherapy of spasticity in children with cerebral palsy. Pediatr Neurol. 2006;34:1-6.
34. Albright AL, Gilmartin R, Swift D, et al. Long-term intrathecal baclofen therapy for severe spasticity of cerebral origin. J Neurosurg. 2003;98: 291-295.
35. Gerszten PC, Albright AL, Barry MJ. Effect on ambulation of continuous intrathecal baclofen infusion. Pediatr Neurosurg. 1997;27:40-44.
36. Gerszten PC, Albright AL, Johnstone GF. Intrathecal baclofen infusion and subsequent orthopedic surgery in patients with spastic cerebral palsy. J Neurosurg. 1998;88:1009-1013.
37. Peacock WJ, Staudt LA. Selective posterior rhizotomy: evolution of theory and practice. Pediatr Neurosurg. 1991-1992;17:128-134.
38. Staudt LA, Nuwer MR, Peacock WJ. Intraoperative monitoring during selective posterior rhizotomy: technique and patient outcome. Electroencephalogr Clin Neurophysiol. 1995;97:296-309.
39. Staudt LA, Peacock WJ. Dorsal rhizotomy for spasticity. West J Med. 1995;162:260.
40. von Koch CS, Park TS, Steinbok P, et al. Selective posterior rhizotomy and intrathecal baclofen for the treatment of spasticity. Pediatr Neurosurg. 2001;35:57-65.
41. Bozkurt FY, Fentoglu O, Yetkin Z. The comparison of various oral hygiene strategies in neuromuscularly disabled individuals. J Contemp Dent Pract. 2004;5:23-31.
42. dos Santos MT, de Oliveira LM. Use of cryotherapy to enhance mouth opening in patients with cerebral palsy. Spec Care Dentist. 2004;24:232-234.
43. Yam WKL, Yang HLC, Abdullah V, Chan CYL. Management of drooling for children with neurological problems in Hong Kong. Brain Develop. 2006;28:24-29.
44. Hurst D, Cedrone N. Modafinil for drooling in cerebral palsy. J Child Neurol. 2006;21:112-114.
45. Jongerius PH, van den Hoogen FJ, van Limbeek J, et al. Effect of botulinum toxin in the treatment of drooling: a controlled clinical trial. Pediatrics. 2004;114:620-627.
46. Tilton AH. Approach to the rehabilitation of spasticity and neuromuscular disorders in children. Neurol Clin. 2003;21:853-881.
47. Rogers B. Feeding methods and health outcomes of children with cerebral palsy. J Pediatrics. 2004;145:S28-S32.
48. Sullivan PB, Juszczak E, Bachlet AM, et al. Gastrostomy tube feeding in children with cerebral palsy: a prospective, longitudinal study. Dev Med Child Neurol. 2005;47:77-85.
49. Sullivan PB, Juszczak E, Bachlet AM, et al. Impact of gastrostomy tube feeding on the quality of life of carers of children with cerebral palsy. Dev Med Child Neurol. 2004;46:796-800.
50. Wales PW, Diamond IR, Dutta S, et al. Fundoplication and gastrostomy versus image-guided gastrojejunal tube for enteral feeding in neurologically impaired children with gastroesophageal reflux. J Pediatr Surg. 2002;37:407-412.
51. Kawai M, Kawahara H, Hirayama S, et al. Effect of baclofen on emesis and 24-hour esophageal pH in neurologically impaired children with gastroesophageal reflux disease. J Pediatr Gastroenterol Nutr. 2004;38:317-323.
52. Heger S, Sippell WG, Partsch CJ. Gonadotropin-releasing hormone analogue treatment for precocious puberty: twenty years of experience. Endocr Dev. 2005;8:94-125.
53. Poulos AE, Balandin S, Llewelyn G, et al. Women with cerebral palsy and breast cancer screening by mammography. Arch Phys Med Rehabil. 2006;87:304-307.
54. Sulpizi LK. Issues in sexuality and gynecologic care of women with developmental disabilities. J Obstet Gynecol Neonatal Nurs. 1996;25:609-614.
55. Owens K, Honebrink A. Gynecologic care of medically complicated adolescents. Pediatric Clin North Am. 1999;46:631-642.
56. McDermott S, Nagle R, Wright HH, et al. Consultation in paediatric rehabilitation for behaviour problems in young children with cerebral palsy and/or developmental delay. Pediatr Rehabil. 2002;5:99-106.