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Childhood disintegrative disorder

Childhood disintegrative disorder (CDD) is a rare condition characterized by late onset (>3 years of age) of developmental delays in language, social function, and motor skills. Researchers have not been successful in finding a cause for the disorder. more...

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CDD has some similarity to autism, but an apparent period of fairly normal development is often noted before a regression in skills or a series of regressions in skills. Many children are already somewhat delayed when the illness becomes apparent, but these delays are not always obvious in young children.

The age at which this regression can occur is defined variously, and can be from age 2-10 with the definition of this onset depending largely on the opinion.

Regression can be very sudden, and the child may even voice concern about what is happening, much to the parent's surprise. Some children describe or appear to be reacting to hallucinations, but the most obvious symptom is that skills apparently attained are lost. This has been described by many writers as a devastating condition, affecting both the family and the individual's future. As is the case with all PDD categories, there is considerable controversy around the right treatment for CDD.

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Diagnosed and manage autistic children
From Nurse Practitioner, 6/1/03 by Blackwell, James

Autism is one of the most common developmental disabilities in children-more common than Down syndrome-yet many health care professionals aren't aware of its effects. Autism affects anywhere from 1 in 200 to 1 in 1,000 children.1-5 Family and pediatric nurse practitioners can expect to diagnose or treat at least one case during their careers.4,6 As primary care providers (PCPs), nurse practitioners (NPs) should screen all children for developmental delays. The family may also look to NPs to help guide them through the maze of treatment options that are available once the diagnosis of autism has been confirmed.

Awareness of current screening, diagnostic, and treatment methods increases the chance of early intervention, which can lead to better outcomes.

* Profiling the Disorder

Autism is a lifelong condition that is about four times more prevalent in boys and does not seem influenced by racial, ethnic, or psychosocial status. The Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-TR) lists autistic disorder as one of five disorders with similar symptoms under the umbrella diagnosis of pervasive developmental disorders.7 The other four are pervasive developmental disorder-not otherwise specified (PDD-NOS), Asperger syndrome, Rett's disorder, and childhood disintegrative disorder.

Researchers see autism as a biologically-based disorder of the brain defined by the presence or absence of a constellation of symptoms.7 Structural abnormalities usually exist, especially in the size and number of Purkinje and neuronal cells within the cerebellum, which brain scans detect.8,9 Evidence of these abnormalities implies that the etiology occurs early in prenatal development.2'10 Patterns of autism or related disabilities exist in some families and suggest a genetic basis. Although genetic research looks promising, a direct link hasn't been identified and environmental influences may also play a role.

Despite publicized claims that vaccinations can trigger autism, researchers have yet to prove any connection.11 Some parents associate vaccinations, particularly the measles-mumps-rubella (MMR) vaccination, or their constituent substances, such as mercury, with the onset of autistic symptoms. A substantial increase in autism diagnoses, which may partly owe to wider recognition and broader diagnostic criteria, fuel this controversy. Several studies on this issue are underway, and their findings could allay parents' fears. Parents with strong objections to vaccinations may choose to delay them until after their child is 24 months old, or request separate administration of combined vaccinations.

* Diagnosis Delays

Although symptoms typically appear by age 3, autism often remains undiagnosed until age 4 or even later, although an increasing number of children are diagnosed before the age of 3. Parents and childcare workers usually first notice delays in language, play, or social interaction when affected children are between the ages of 18 to 30 months. Some parents may notice symptoms earlier. Diagnostic delays often occur because of the diverse expression of autism, unavailability of appropriate screening tools, and concerns about labeling or incorrect diagnosis.3,12

Delays in diagnosis may also occur because fewer than 30% of primary care providers perform standardized screening for developmental delays during well-child visits.3 In more than 1,200 United Kingdom families who felt "something was wrong" with their child, providers diagnosed fewer than 10 percent of the children during initial presentation.13

Families will look to providers to guide them through treatment options when they have a confirmed diagnosis. Many parents reported that providers didn't offer any information about their child's problems or names of support groups, nor did providers refer them for early intervention. Many said schools and other parents, not health care workers, provided the most assistance.13

* Signs and Symptoms

Children with autism often present with a wide variety of symptoms and characteristics that range from mild to severe (see Table: "Common Characteristics of Children with Autism"). Although no single developmental deficit or behavior characteristic exists for all children with autism, most will have some degree of impairment in the ability to use joint attention (the ability to use eye contact and pointing for the social purpose of sharing experiences with others) and pretend play.1

Children with autism have deficiencies in verbal and nonverbal communication, social interaction, and play. They may also perform repetitive body movements, have unusual responses to people or attachments to objects, resist changes in routines, and behave aggressively or injure themselves. Affected children may exhibit any combination of these behaviors with any degree of severity. When they reach school age, children with autism have a variable range of academic skills.14

The prognosis for children with autism is often uncertain. Some symptoms may lessen as the child ages, others may require specialized support and supervised, structured care. Studies show that the most important factor in determining intellectual progress is the overall level of ability.15

* Screening and Diagnosis

Early diagnosis and intervention may produce better outcomes, but diagnosing autism during a primary care visit can be challenging. Early intervention begins with proper screening. First, providers should screen all children for developmental progress. Developmental surveillance "should include social-emotional milestones in addition to the more traditional motor, cognitive, and language ones."1

Researchers have found that the traditional screening tool, the Denver Developmental Screening Test-II (DDST-II), is lacking in sensitivity and specificity. Researchers report that the Ages and Stages Questionnaire, BRIGANCE Screens, Child Development Inventories, and Parents Evaluations of Developmental Status are more sensitive for general screening.3

Parental concerns regarding speech and language development, behavior, or other developmental issues help detect global deficits and should prompt providers to perform more comprehensive testing. Absence of babbling by 12 months, gesturing by 12 months, single words by 16 months, and two-word spontaneous phrases by 24 months may signal a developmental disability, as is loss of language or social skills at any age.3

Take a medical history, which should include the child's prenatal, labor and delivery, and early neonatal periods, as well as language and motor developmental milestones. Ask parents when they first became concerned about their child and why. Ask them about unusual aspects of the child's early development and concerns they may have.

Evaluate the child's medical conditions, such as hearing or visual impairments, Fragile X syndrome, mental retardation, and use of behavior-modifying medications or interventions. Obtain previous educational evaluations, standardized rating scales, teacher or care provider reports, and other pertinent information.

Conduct a physical examination, including a detailed neurologic examination, to help identify treatable conditions or those frequently associated with autism, such as seizure disorders, Fragile X syndrome, or tuberous sclerosis. Order laboratory examinations if the child's history and clinical presentation indicate them.

All children with developmental delays, particularly language and social delays, should receive a formal audiologic and visual evaluation, comprehensive speech and language evaluation, and serum lead level test. Consider ordering other studies if the child's history and physical exam merit them and if the results will aid in genetic counseling and management options.

The child should be screened specifically for autism if he fails a routine developmental surveillance. This may be done with either an autism screening instrument (see Table: "Autism Diagnostic Tools") or, if you can't obtain an autismspecific instrument, systematically inquire about language and social-emotional development, joint attention, and pretend play with a screening questionnaire (see Table: "Important Screening Questions for Parents").

If the child fails the autism-specific screen, a formal evaluation should be performed. This evaluation should differentiate autism from other developmental disorders. Diagnosis stems from observations of the child's communication, behavior, and development and occurs when a specific number of characteristics are present in inappropriate ranges for that age. A multidisciplinary team, which may include a developmental pediatrician, child psychologist, speech-language expert, and an occupational and physical therapist with autism knowledge, should evaluate the observations, which should take place in home, school, and clinical settings.

A specialist or team of specialists should complete psychological and psychiatric examinations. Specialists should be knowledgeable regarding autism and other pervasive developmental disorders, as well as mental retardation, selective mutism, obsessive-compulsive disorder, and social anxiety disorder.

Cognitive ability needs to be assessed with separate estimates of verbal IQ (VIQ) and nonverbal (performance) IQ (PIQ). Many children will demonstrate a pattern of PIQ higher than VIQ;3 the PIQ-VIQ split, however, is severity-dependent. Formal intelligence testing can be challenging, and results may not be reliable.6

DSM criteria are geared to children 3 years and older and preliminary diagnoses may need revision if additional symptoms appear. Other screening tools exist for autism or are in development (see Table: "Sample of Common Autism Screening Tools").

At this time, a multidisciplinary team establishes optimal intervention strategies. Because of the required time, special training, and multidisciplinary approach, many PCPs may refer the child to more experienced clinicians.

Several diagnostic tools can help practitioners make an accurate diagnosis. Autism specialists typically use one or more diagnostic tools (see Table: "Autism Diagnostic Tools"). The child should receive a re-evaluation within one year and regularly thereafter to track developmental changes.

* Treating Autism

Pediatric literature doesn't contain any medical cures, guidelines, or consensus for treating autism.6 However, most current treatments focus on improving overall function. Typically, this goal involves enrolling the child in an appropriate and intensive early intervention program to promote development of communication, social, adaptive, behavioral, and academic skills. These programs can also decrease maladaptive and repetitive behaviors, and help the family manage stress by providing information about community resources, such as respite care and support groups.

Clinicians should tailor interventions to developmental and behavioral needs as well as the family's coping style and resources. Interventions may include parent education and support, highly structured social play, individualized school-based education, behavior management and training, medical treatment, and respite. Parents of children who have just received an autism diagnosis may find help through talking with other parents of autistic children.16 Help parents find a "parent to parent" organization, and give them the contact information for a local Autism Society of America chapter.

The federal government mandates age-appropriate early intervention and school programs, which are often based on a developmental delay rather than a specific diagnosis. The federal Individuals with Disabilities Education Act of 1990 (and subsequent revisions), mandates "appropriate" educational plans for children with developmental disabilities or delays and allocates specific rights to their parents. Services include speech-language therapy and occupational and physical therapy for preschool and school-aged children.

Several treatments consistently benefit children with autism. The treatments mentioned here don't indicate exclusive treatment, nor do they serve as a standard of care. Al-though specialists will likely direct the care of a child with autism, remain actively involved, even if you disagree with the family's treatment decisions.

Behavioral Technology and Social Skills Training

Behavioral training and management is the cornerstone of treating autism at any age. Behavior management and structured teaching of skills help to prevent undesirable behaviors and help caregivers more clearly teach and give directions to children with autism. Discrete trial training is one form of applied behavior analysis. It involves intensive, one-on-one teaching so children learn small parts of behaviors or skills in short, repeated sessions. As a child learns skills, the teacher adds new ones in carefully planned sequences.

The "social story" uses scripted scenarios to help children with autism understand and follow specified social protocols.17 Another approach uses symbols or pictures to demonstrate appropriate and inappropriate behaviors.

Children with autism should be in childcare centers and classrooms with typically-developing children so they can model desirable behaviors. A teacher can extend this modeling by creating a "circle of friends," in which she invites children to join a circle with an autistic child. The teacher then encourages the children to express concerns about the difficulties the child is having and help create solutions.

Habilitative Services

Communication training involving preverbal and verbal communication and occupational and physical therapy are also essential for managing autism. These are most effective when interwoven throughout a total program. Because children with autism often have a core deficit in functional communication, they typically need intensive intervention to develop receptive and expressive communication.

Based upon behavioral principles, speech therapy may focus on acquiring language skills or helping the child communicate more effectively by correcting errors in semantics or pragmatics. Gestural, verbal, pictorial, and technological systems and discrete trial training can effectively teach language. Each child needs an individualized program, with family and teacher support, to learn communication. Careful assessment is critical, as well as skilled training and commitment of the adults who will use the system with the child.14 Occupational and physical therapy can help address any coordination concerns and motor deficits.

Alternative Therapies

Unconventional treatments abound partly because no medical cure exists for autism. Many parents pursue alternative therapies because of a desire to try almost anything that might help, claims of improvements from other families, or rising skepticism regarding scientifically-based treatments. Become familiar with the more popular alternative therapy treatments for autism, such as unconventional diets, vitamin supplementation, sensory integration, and vision treatments. Approach parents' choices with objectivity and compassion.

Tell parents that many people are urging scientific evaluation of treatments. Advise that they should be skeptical of any treatment-especially those touted as a "cure," a method that will lead to "recovery," or one that will benefit all who have autism. Encourage parents to speak with a health care provider and ask themselves these questions before starting any proposed treatment:18

* Could the treatment harm my child?

* How will failure of the treatment affect my child and family?

* Does the treatment have scientific validation?

* Do specified assessment procedures exist?

* Can we integrate the treatment into the child's current program?

* Changing Landscape

The diagnosis, management, and understanding of autism changes dramatically as more children receive diagnoses and more researchers study the disorder. Periodically update your knowledge about autism to provide early diagnosis, implement appropriate interventions, and coordinate the system of care. For more information about autism, consult local and national resources, such as the Autism Society of America (http://www.autism-society.org). NP

REFERENCES

1. American Academy of Pediatrics: Technical report: The pediatrician's role in the diagnosis and management of autistic spectrum disorder in children. Peds 2001; 107(5):1221-26.

2. Bristol MM, Cohen DJ, Costello E), et al.: State of the science in autism: Report to the National Institutes of Health. J Autism and Dev Disord 1996;26(2):121-54.

3. Filipek PA, Accardo PJ, Ashwal S, et al: Practice parameter: Screening and diagnosis of autism. Neur 2000;55:468-79.

4. Hyman SL, Levy SE: Autistic spectrum disorders: When traditional medicine is not enough. Cont Ped 2000;101-16.

5. Autism Society of America: Advocate;35(3):3. Bethesda, MD: Autism Society of America, 2002.

6. American Psychiatric Association: Diagnostic and statistical manual of mental disorders, 4^sup th^ ed., text revision. Washington, DC: American Psychiatric Association, 2000.

7. Kanner, L: Autistic disturbances of affective contact. Nervous Child 1943;2:217-50.

8. Courchesne E, Yeung-Courchene R, Press GA, et. al: Hypoplasia of cellebellar vermal lobules VI and VII in autism. N Engl J Med 1988; 318:1349-54.

9. Courchesne E, Townsend J, Saitoh O: The brain in infantile autism: posterior fossa structures are abnormal. Neur 1994;44:214-23.

10. Kemper TL, Bauman M: Neuropathology of infantile autism. J Neuropathol Exp Neurol 1998;57:645-52.

11. Towbin KE, Mauk JE, Batshaw ML: Pervasive developmental disorders. In Children with disabilities, 5^sup th^ ed., ML Batshaw (ed.). Baltimore: Paul H. Brookes Publishing Co., 2002.

12. Volkmar F, Cook E, Pomeroy J, et al: Practice parameters for the assessment and treatment of children, adolescents, and adults with autism and other pervasive developmental disorders. J Am Acad Child Adolesc Psychiatry 1999;38(12 Suppl):55S-76S.

13. American Academy of Pediatrics: Policy statement: Auditory integration training and facilitated communication for autism. Peds 1998;102(2):431-3.

14. Howlin P, Moore A: Diagnosis of autism: A survey of over 1200 patients in the UK. Autism 1997;1:135-62.

15. Scott J, Clark C, Brady M: Students with autism: Characteristics and instruction programming. San Diego, CA: Singular Publishing Group, 2000.

16. Wing L: The autistic spectrum: A parents' guide to understanding and helping your child. Berkeley, CA: Ulysses Press, 2001.

17. Santelli B, Ginsberg C, Sullivan S, Niederhauser C: A collaborative study of parent to parent programs. In Families and positive behavior support: Addressing problem behavior in family contexts, JM Lucyshyn, G Dunlap, R Albin (eds.). Baltimore: Paul H. Brooks Publishing Co., 2002.

18. Lorimer PA, Simpson RL, Myles BS, Ganz JB; The use of social stories as a preventative behavioral intervention in a home setting with a child with autism. J Pos Behavior Interventions 2002;4(1):53-60.

19. Freeman BJ: Principals for evaluating treatment of autism. J Autism Dev Disord 1997;27(6);641-51.

20. Baron-Cohen S, Allen J, Gillberg C: Can autism be detected at 18 months? The needle, the haystack, and the CHAT. Brit Jour Psychiatry 1992;161:839-43.

James Blackwell, MS, FNP, APRN, BC

Carol Niederhauser, BA

ABOUT THE AUTHORS

James Blackwell is a Nurse Practitioner at the Carolinas Healthcare System Department of Internal Medicine in Charlotte, North Carolina and Carol Niederhauser is program coordinator at the South Carolina Autism Society. Views expressed in this article are those of the author and do not necessarily reflect the views of the South Carolina Autism Society.

Copyright Springhouse Corporation Jun 2003
Provided by ProQuest Information and Learning Company. All rights Reserved

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