Intellectual Disability Treatment & Management
- Author: Ari S Zeldin, MD, FAAP, FAAN; Chief Editor: Amy Kao, MD more...
See the list below:
- Early identification of children with developmental delays is necessary to begin receiving early intervention services for children from birth to 3 years of age and early childhood education services for children aged 3-5 years, which are known to improve outcomes.
- The mainstay of treatment of MR/ID is developing a comprehensive management plan for the condition. The complex habilitation plan for the individual requires input from care providers from multiple disciplines, including special educators, language therapists, behavioral therapists, occupational therapists, and community services that provide social support and respite care for families affected by MR/ID.
- Preventive care: Unfortunately, routine preventive care for children and adults with MR/ID is lacking. Adaptive equipment (eg, for nonambulatory patients) and extra time (eg, double time slots) may be required to accommodate such patients. In addition, family members or other support persons may be helpful. Written plans (such as the Massachusetts Department of Developmental Services Annual Health Screening Recommendations and Health Record) are helpful for interdisciplinary team communication.
- Physical activity and obesity are major contributors to disease in MR/ID. Very few programs exist that target healthy lifestyles (nutrition/diet, exercise, self-care, stress reduction) in those with MR/ID. Annual counseling and referral on these issues to community agencies and programs is recommended. Medications (eg, antipsychotics) should be titrated to reduce the risk of obesity and metabolic issues.
- Manifestations of pain in people with severe to profound MR/ID include crying, screaming, grimacing, protective postures (eg, arching, fetal position), rocking, and aggression. Parent/caregiver input is key to interpretation of these behaviors, though validated tools have been used as adjuncts (such as the Pediatric Pain Profile).
- Common causes of acute pain include dental caries/abscesses, GERD, constipation, UTI, spasticity (when MR/ID is associated with cerebral palsy), pressure sores, and fractures.
- In addition, neuropathic pain due to dysautonomia or motor spasms may create chronic disturbances. Treatment should be prompt and include NSAIDs or acetaminophen for mild pain, tramadol or equivalent for moderate pain, and opioids for severe pain as indicated, and management of sources of pain. Some suggest use of gabapentin for neuropathic pain if no sources are identified and there is a history of surgery, symptoms suggesting visceral hyperalgesia (eg, associated with feedings or bowel movements), or symptoms of autonomic dysfunction and spasticity.
- Written, verbal and pictoral forms of communication as well as gestures and demonstrations are helpful for those with MR/ID to ensure mutual understanding and improve treatment adherence.
- Sedation/anesthesia: Patients with MR/ID requiring anesthesia may have different reactions than the general population, such as paradoxical reactions to benzodiazepines, and care should be taken to use the lowest dose and titrate slowly.
- Sexuality/abuse: A significantly higher proportion of children and adults with MR/ID have experienced some form of abuse, with some estimates of up to 70%, which contributes to mental health issues. This should be addressed at each medical visit and especially in the setting of changes in behaviors, such as increased aggression.
- No treatments are available specifically for cognitive deficiency. Although the pharmacologic enhancement of cognition (eg, use of donepezil in patients with Down syndrome ) is an area of interest, research on such nootropic (ie, knowledge-enhancing) compounds is limited. Such drugs have not become part of the routine or even experimental clinical management of this population.
See the list below:
- Individuals in the United States older than 18 years are no longer under the guardianship of their biological parents. No exceptions are made for children with MR/ID. Most of these individuals, particularly those in the range of mild MR, are capable of making appropriate legal and medical decisions when adequately and appropriately informed of the decision outcomes.
- Physicians have the duty to ascertain whether patients with MR/ID have the capacity to consent for medical treatments. This may be challenging and outside information and supports (eg, family, caregivers, social workers) may be required to confirm the patients' understanding of the risks, benefits and alternatives to the procedure.
- Some individuals may not be capable of comprehending the implications of the medical or legal matter at hand. In such cases, the decision is best made by a member of the biological family. The family member may obtain guardianship status for power of attorney over these matters. If a family member is unavailable to serve as guardian, then a guardian ad litum can be assigned by the court for assistance in such legal and medical matters. If a patient with MR/ID does not have the capacity to consent, then the patient's assent should be sought if possible.
- Subsequent to the long history of forced sterilization of girls/women with MR/ID, varied federal, state, and local laws regulate sterilization of individuals with MR/ID. The American College of Obstetrician/Gynecologists provides guidance on informed consent for sterilization procedures in patients with ID/MR.
- Complex decisions, particularly those involving end of life, are perhaps best handled with the assistance of the ethics committee of the involved medical institution.
- Failure to identify a genetic cause of MR/ID with risks to other family members or risks to the patient for future medical complications are potential medical/legal pitfalls.
- Perhaps 1 in 8 convicts on death row in the United States has MR/ID. Many persons cannot fully comprehend the Miranda Rights and other critical concepts necessary to maneuver through the criminal justice system.
See the list below:
- Developmental pediatrician or psychologist
- Geneticist and counselor
- Special education/educational therapist
- Occupational, speech and/or physical therapist
- Durable medical equipment providers
- Social services agencies/social workers
Nutritional supplements are of no proven benefit.
Brooks M. FDA OKs Postnatal Blood Test for Intellectual Disabilities. Medscape Medical News. Jan 17 2014. Available at http://www.medscape.com/viewarticle/819387. Accessed: Jan 29 2014.
FDA. FDA allows marketing for first of-its-kind post-natal test to help diagnose developmental delays and intellectual disabilities in children. US Food and Drug Administration. Jan 17 2014. Available at http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm382179.htm.
Courchesne E, Carper R, Akshoomoff N. Evidence of brain overgrowth in the first year of life in autism. JAMA. 2003 Jul 16. 290(3):337-44. [Medline].
Dawson G, Munson J, Webb SJ, Nalty T, Abbott R, Toth K. Rate of head growth decelerates and symptoms worsen in the second year of life in autism. Biol Psychiatry. 2007 Feb 15. 61(4):458-64. [Medline].
Shevell M, Ashwal S, Donley D, Flint J, Gingold M, Hirtz D. Practice parameter: evaluation of the child with global developmental delay: report of the Quality Standards Subcommittee of the American Academy of Neurology and The Practice Committee of the Child Neurology Society. Neurology. 2003 Feb 11. 60(3):367-80. [Medline].
Wigal T, Greenhill L, Chuang S, McGough J, Vitiello B, Skrobala A. Safety and tolerability of methylphenidate in preschool children with ADHD. J Am Acad Child Adolesc Psychiatry. 2006 Nov. 45(11):1294-303. [Medline].
American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders. 5th Edition. Washington, DC: APA Press; 2013.
Birch HG, Richardson SA, Baird D, Horobin G, Illsley R. Mental Subnormality in the Community: A Clinical and Epidemiologic Study. Baltimore: Williams & Wilkins; 1970.
Strauss D, Eyman RK. Mortality of people with mental retardation in California with and without Down syndrome, 1986-1991. Am J Ment Retard. 1996 May. 100(6):643-53. [Medline].
Patja K, Mölsä P, Iivanainen M. Cause-specific mortality of people with intellectual disability in a population-based, 35-year follow-up study. J Intellect Disabil Res. 2001 Feb. 45:30-40. [Medline].
Rutter M, Graham P. Epidemiology of psychiatric disorder. Rutter M, Tizard J, Whitemore P. Education, Health and Behavior. London, England: Longman Group; 1970. 178-201.
American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders. Fourth Edition, Text Revision. Washington DC: American Psychiatric Association; 2000.
Shattuck PT. The contribution of diagnostic substitution to the growing administrative prevalence of autism in US special education. Pediatrics. 2006 Apr. 117(4):1028-37. [Medline].
Kenneth Lyons Jones. Smith's Recognizable Patterns of Human Malformations. 6. Elsevier Saunders; 2006. 160-161.
Kaufmann WE, Abrams MT, Chen W. Genotype, molecular phenotype, and cognitive phenotype: correlations in fragile X syndrome. Am J Med Genet. 1999 Apr 2. 83(4):286-95. [Medline].
Greenberg F, Lewis RA, Potocki L. Multi-disciplinary clinical study of Smith-Magenis syndrome (deletion 17p11.2). Am J Med Genet. 1996 Mar 29. 62(3):247-54. [Medline].
Tassabehji M, Metcalfe K, Fergusson WD. LIM-kinase deleted in Williams syndrome [letter]. Nat Genet. 1996 Jul. 13(3):272-3. [Medline].
Verhoeven WM, Tuinier S, Kuijpers HJ, Egger JI, Brunner HG. Psychiatric Profile in Rubinstein-Taybi Syndrome. A Review and Case Report. Psychopathology. 2009 Nov 20. 43(1):63-68. [Medline].
Marques Pereira P, Schneider A, Pannetier S, Heron D, Hanauer A. Coffin-Lowry syndrome. Eur J Hum Genet. 2009 Nov 4. [Medline].
Amir RE, Van den Veyver IB, Wan M. Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl- CpG-binding protein 2. Nat Genet. 1999 Oct. 23(2):185-8. [Medline].
Axelrad ME, Schwartz DD, Fehlis JE, Hopkins E, Stabley DL, Sol-Church K, et al. Longitudinal course of cognitive, adaptive, and behavioral characteristics in Costello syndrome. Am J Med Genet A. 2009 Dec. 149A(12):2666-72. [Medline]. [Full Text].
Gripp KW, Lin AE, Stabley DL. HRAS mutation analysis in Costello syndrome: genotype and phenotype correlation. Am J Med Genet A. 2006 Jan 1. 140(1):1-7. [Medline].
Autti-Ramo I, Fagerlund A, Ervalahti N. Fetal alcohol spectrum disorders in Finland: Clinical delineation of 77 older children and adolescents. Am J Med Genet A. 2006 Jan 15. 140(2):137-43. [Medline].
Mann JR, McDermott S, Barnes TL, Hardin J, Bao H, Zhou L. Trichomoniasis in pregnancy and mental retardation in children. Ann Epidemiol. 2009 Dec. 19(12):891-9. [Medline].
Moeschler JB, Shevell M,. Clinical genetic evaluation of the child with mental retardation or developmental delays. Pediatrics. 2006 Jun. 117(6):2304-16. [Medline].
Mefford HC, Batshaw ML, Hoffman EP. Genomics, intellectual disability, and autism. N Engl J Med. 2012 Feb 23. 366(8):733-43. [Medline].
Liang JS, Shimojima K, Yamamoto T. Application of array-based comparative genome hybridization in children with developmental delay or mental retardation. Pediatr Neonatol. 2008 Dec. 49(6):213-7. [Medline].
Sagoo GS, Butterworth AS, Sanderson S, Shaw-Smith C, Higgins JP, Burton H. Array CGH in patients with learning disability (mental retardation) and congenital anomalies: updated systematic review and meta-analysis of 19 studies and 13,926 subjects. Genet Med. 2009 Mar. 11(3):139-46. [Medline].
Michelson DJ, Shevell MI, Sherr EH, Moeschler JB, Gropman AL, Ashwal S. Evidence report: Genetic and metabolic testing on children with global developmental delay: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2011 Oct 25. 77(17):1629-35. [Medline].
Bazzano AT, Zeldin AS, Diab IR, Garro NM, Allevato NA, Lehrer D. The Healthy Lifestyle Change Program: a pilot of a community-based health promotion intervention for adults with developmental disabilities. Am J Prev Med. 2009 Dec. 37(6 Suppl 1):S201-8. [Medline].
Kishnani PS, Sommer BR, Handen BL, Seltzer B, Capone GT, Spiridigliozzi GA, et al. The efficacy, safety, and tolerability of donepezil for the treatment of young adults with Down syndrome. Am J Med Genet A. 2009 Aug. 149A(8):1641-54. [Medline].
ACOG Committee Opinion. Number 371. July 2007. Sterilization of women, including those with mental disabilities. Obstet Gynecol. 2007 Jul. 110(1):217-20. [Medline].
Bartlo P, Klein PJ. Physical activity benefits and needs in adults with intellectual disabilities: systematic review of the literature. Am J Intellect Dev Disabil. 2011 May. 116(3):220-32. [Medline].
Ellis JM, Tan HK, Gilbert RE, Muller DP, Henley W, Moy R, et al. Supplementation with antioxidants and folinic acid for children with Down's syndrome: randomised controlled trial. BMJ. 2008 Mar 15. 336(7644):594-7. [Medline]. [Full Text].
MASSACHUSETTS DEPARTMENT OF DEVELOPMENTAL SERVICES. Massachusetts Department of Developmental Services Annual Health Screening Recommendations. Available at http://www.mass.gov/Eeohhs2/docs/dmr/health_screening_checklist.pdf. Accessed: April 1, 2010.
Bull MJ. Health supervision for children with Down syndrome. Pediatrics. 2011 Aug. 128(2):393-406. [Medline].
[Guideline] McCandless SE, Committee on Genetics. Clinical report—health supervision for children with Prader-Willi syndrome. Pediatrics. 2011 Jan. 127(1):195-204. [Medline].
Ahn KJ, Jeong HK, Choi HS. DYRK1A BAC transgenic mice show altered synaptic plasticity with learning and memory defects. Neurobiol Dis. 2006 Jan 30. [Medline].
American Psychiatric Association. APA DSM-5 Development: Proposed Revision: Mental Retardation. American Psychiatric Association DSM-5 Development. Available at http://www.dsm5.org/ProposedRevisions/Pages/proposedrevision.aspx?rid=384. Accessed: March 20, 2010.
Capute AJ, Accardo PJ. Developmental Disabilities in Infancy and Childhood. Vol 1 and 2. Baltimore: Paul H Brookes. 1996: 1-619 and 1-521.
Developmental surveillance and screening of infants and young children. Pediatrics. 2001 Jul. 108(1):192-6. [Medline].
Doheny KF, McDermid HE, Harum K. Cryptic terminal rearrangement of chromosome 22q13.32 detected by FISH in two unrelated patients. J Med Genet. 1997 Aug. 34(8):640-4. [Medline].
Flint J, Wilkie AO, Buckle VJ. The detection of subtelomeric chromosomal rearrangements in idiopathic mental retardation. Nat Genet. 1995 Feb. 9(2):132-40. [Medline].
Kirchhoff M, Gerdes T, Brunebjerg S. Investigation of patients with mental retardation and dysmorphic features using comparative genomic hybridization and subtelomeric multiplex ligation dependent probe amplification. Am J Med Genet A. 2005 Dec 15. 139(3):231-3. [Medline].
Maulik PK, Mascarenhas MN, Mathers CD, Dua T, Saxena S. Prevalence of intellectual disability: a meta-analysis of population-based studies. Res Dev Disabil. 2011 Mar-Apr. 32(2):419-36. [Medline].
Rubin IL, Crocker AC. Medical care for children and adults with developmental disabilities. Second edition. Baltimore, MD: Paul H Brookes Publishing Co, Inc; 2006.
Medina AE, Krahe TE, Ramoa AS. Restoration of neuronal plasticity by a phosphodiesterase type 1 inhibitor in a model of fetal alcohol exposure. J Neurosci. 2006 Jan 18. 26(3):1057-60. [Medline].
Miyake N, Shimokawa O, Harada N. BAC array CGH reveals genomic aberrations in idiopathic mental retardation. Am J Med Genet A. 2006 Feb 1. 140(3):205-11. [Medline].
Reiss S, Aman MG. Psychotropic Medications and Developmental Disabilities: The International Consensus Handbook. The Ohio State University Nisonger Center. 1998. 1-355.
Richardson SA, Koller H. Twenty-Two Years. Cambridge, MA: Harvard University Press. 1996. 1-328.
Schroeder S, Gerry M, Gertz G, Velasquez F. Final Project Report: Usage of the Term "Mental Retardation:" Language, Image and Public Education. June 2002. Available at www.ssa.gov/disability/MentalRetardationReport.pdf.
Volkmar FR, Lewis M. Mental Retardation: Child and Adolescent Psychiatric Clinics of North America. Philadelphia: WB Saunders Company; 1996. 5: 769-993.