PERVASIVE DEVELOPMENTAL DISORDERS
DSM-IV
299.00 Autistic disorder
299.80 Rett’s disorder
299.80 Asperger’s disorder
299.10 Childhood disintegrative disorder
299.80 Pervasive developmental disorder NOS (including atypical autism)
The category of pervasive developmental disorders is organized in terms of the qualitative degree of impairment in social and communicative functioning. Autism comprises extremely varied manifestations that encompass deficits in cognition, social awareness, communication, affective expression, and motor control. Because of the continuity of the symptomatology, the term autistic spectrum disorder (ASD) is recognized in the current literature. ASD may occur with or without a neurological substrate, as in the case of idiopathic autism. Age of onset and whether the child developed normally from birth through the first 5 years of life are factors that help differentiate between Rett’s disorder or childhood disintegrative disorder. The intractable anxiety, mood liability, perseveration of thought and behavior, and odd social presentation of these children may mimic other psychiatric disorders, including anxiety disorders, schizophrenia, obsessive-compulsive disorder, and the manic phase of bipolar affective disorder. When neurological impairment coexists with the diagnosis, the individual is often low functioning.
ETIOLOGICAL THEORIES
Psychodynamics
Autistic children are fixed in the presymbiotic stage of development. These children do not achieve a symbiotic attachment, nor do they differentiate self from mother. Psychotic-like behaviors are based on abnormal primary development rather than on a regression from a higher level of functioning. Children with autism lack the intuitive skills to engage in and sustain meaningful social contact, particularly in new situations, and they have a marked inability to generalize.
Biological
Neurological evaluation, including family history, electroencephalogram (EEG), magnetic resonance imaging (MRI), karyotyping, and positron emission tomography (PET), reveals strong evidence of a familial pattern of organic neurological impairment and psychiatric illness. Several research studies estimate the coexistence of neuropsychiatric illness in extended family members to be as high as 50% in individuals with ASD.
Research to confirm brain anatomical abnormalities suggests that neurons in the amygdala (the area responsible for processing emotions and behavior) and the hippocampus (involved in learning and memory) are smaller, more densely packed in some areas, and have shorter, less-developed branches than normal. Low blood circulation in some parts of the cerebral cortex during certain intellectual functions and a reduced number of cells relaying inhibitory messages have been demonstrated. It has been hypothesized that these severe developmental disorders of childhood are the result of a disturbance in the central nervous system integration and in the biological process of maturation. Predisposing organic factors include maternal rubella, phenylketonuria, encephalitis, meningitis, hydrocephalus, hypothyroidism, and tuberous sclerosis.
Family Dynamics
This disorder has been viewed in the past as a result of a severe disturbance in parent-child interaction. Lack of bonding and stimulation as well as maternal deprivation have been listed as causative factors. More recently, dysfunctional parenting has been seen less as contributing to the disorder (not accepted) and more as a response to the disturbed behavior.
CLIENT ASSESSMENT DATA BASE
Activity/Rest
Problems in sleeping
Ego Integrity
Detached, separated from work, withdrawn, restive, may be passive
Verbal/nonverbal communication may be incongruent
Demonstrates repetitive stereotypical motor behaviors (hand flicking, head banging, complex whole-body movements)
Elimination
Disturbances in bowel and bladder functioning
Food/Fluid
Disturbed eating patterns
Hygiene
Generally dependent
Eccentric preoccupation with one area of hygiene and neglect of another (e.g., showering repeatedly but never brushing teeth)
Neurosensory
Abnormalities noted in almost every sphere of development
Delayed motor, perceptual, cognitive, and language development
Soft neurological signs are often seen (e.g., slight tremors, slowed responses)
Varied/bizarre responses to the environment, with resistance or extreme behavioral reactions to minor occurrences; ritualistic behaviors; extreme fascination with moving objects; special interests in music (although heightened hearing/inability to filter or dampen sounds may result in intolerance)
Bizarre facial expressions
Alterations in mood—lacking the gradations in range of fear, sadness, joy
Unreasonable insistence on following routines in precise detail; marked distress over changes in trivial aspects of environment
Difficulty communicating verbally, with delays in/no development of speech; may mimic sounds made by others, incorrect use of words, echolalia, inability to understand abstract terms; consistent reversal of pronouns “I” and “you”
May show periods of extreme agitation in which behavior becomes disruptive and unmanageable
Does not initiate social imitative play appropriate for stage of development
Safety
Self-mutilative behaviors (e.g., head banging, hair pulling)
Lack of appropriate fear/ignoring signs of danger (e.g., running into street with heavy traffic); fearing harmless objects such as shrinking from touch, going limp or stiffening when held (autism), putting people off by abrupt/awkward approaches (Asperger’s)
Social Interactions
Poor eye contact, impaired responsiveness/communication when interacting with others
Severely disturbed/impaired development in social relationships; may be barely able to distinguish parents from strangers (autism)
Marked impairment in use of nonverbal gestures associated with social interactions
Lack of social or emotional reciprocity, does not express pleasure toward or in response to other people’s happiness; indifference or aversion to physical contact
Teaching/Learning
High association with mental retardation; normal or high verbal intelligence (Asperger’s)
Onset during infancy or early childhood before age 3 (autism) with telltale symptoms possibly noted during first months; marked regression following at least 2 years of apparently normal development, and before age 10 (disintegrative disorder), predominantly males; diagnosis of Rett’s made at about 5 months of age, occurring only in females
DIAGNOSTIC STUDIES
Neurological examination to determine presence and/or extent of organic impairment.
BEAM/PET Scans: May reveal abnormalities in cerebellum (regulates motion and some aspects of memory) and the limbic region (controls much of emotional life).
EEG: May be abnormal, reflecting presence/extent of organic impairment.
Psychological Testing/Intelligence Quotient (IQ): Provides information about cognitive and personality functioning; IQ below 70 may be noted.
Biochemical Studies: Abnormalities not consistently noted.
Laboratory Tests: As indicated by antipsychotic drug therapy.
Auditory Testing: To rule out deafness as a cause of speech problems.
Vision Testing: To differentiate responses to auditory and visual stimuli as abnormal reactions versus distorted perceptions.
Developmental Testing (e.g., Denver Developmental): May reveal delays.
Determine physical causes for disturbances in age-appropriate functions and behaviors (e.g., toileting problems).
NURSING PRIORITIES
1. Facilitate control/decrease of behavioral symptoms.
2. Enhance communication skills and social interaction.
3. Promote family involvement in treatment process and acceptance of child’s disability.
DISCHARGE GOALS
1. Current behavior problems or troublesome symptoms for which treatment is being sought are effectively managed.
2. Treatment within the community is maintained; institutional placement is avoided, when possible.
3. Family verbalizes knowledge about resources to meet the need for a long-term structured therapeutic program.
4. Plan is in place to meet needs after discharge.
DSM-IV
299.00 Autistic disorder
299.80 Rett’s disorder
299.80 Asperger’s disorder
299.10 Childhood disintegrative disorder
299.80 Pervasive developmental disorder NOS (including atypical autism)
The category of pervasive developmental disorders is organized in terms of the qualitative degree of impairment in social and communicative functioning. Autism comprises extremely varied manifestations that encompass deficits in cognition, social awareness, communication, affective expression, and motor control. Because of the continuity of the symptomatology, the term autistic spectrum disorder (ASD) is recognized in the current literature. ASD may occur with or without a neurological substrate, as in the case of idiopathic autism. Age of onset and whether the child developed normally from birth through the first 5 years of life are factors that help differentiate between Rett’s disorder or childhood disintegrative disorder. The intractable anxiety, mood liability, perseveration of thought and behavior, and odd social presentation of these children may mimic other psychiatric disorders, including anxiety disorders, schizophrenia, obsessive-compulsive disorder, and the manic phase of bipolar affective disorder. When neurological impairment coexists with the diagnosis, the individual is often low functioning.
ETIOLOGICAL THEORIES
Psychodynamics
Autistic children are fixed in the presymbiotic stage of development. These children do not achieve a symbiotic attachment, nor do they differentiate self from mother. Psychotic-like behaviors are based on abnormal primary development rather than on a regression from a higher level of functioning. Children with autism lack the intuitive skills to engage in and sustain meaningful social contact, particularly in new situations, and they have a marked inability to generalize.
Biological
Neurological evaluation, including family history, electroencephalogram (EEG), magnetic resonance imaging (MRI), karyotyping, and positron emission tomography (PET), reveals strong evidence of a familial pattern of organic neurological impairment and psychiatric illness. Several research studies estimate the coexistence of neuropsychiatric illness in extended family members to be as high as 50% in individuals with ASD.
Research to confirm brain anatomical abnormalities suggests that neurons in the amygdala (the area responsible for processing emotions and behavior) and the hippocampus (involved in learning and memory) are smaller, more densely packed in some areas, and have shorter, less-developed branches than normal. Low blood circulation in some parts of the cerebral cortex during certain intellectual functions and a reduced number of cells relaying inhibitory messages have been demonstrated. It has been hypothesized that these severe developmental disorders of childhood are the result of a disturbance in the central nervous system integration and in the biological process of maturation. Predisposing organic factors include maternal rubella, phenylketonuria, encephalitis, meningitis, hydrocephalus, hypothyroidism, and tuberous sclerosis.
Family Dynamics
This disorder has been viewed in the past as a result of a severe disturbance in parent-child interaction. Lack of bonding and stimulation as well as maternal deprivation have been listed as causative factors. More recently, dysfunctional parenting has been seen less as contributing to the disorder (not accepted) and more as a response to the disturbed behavior.
CLIENT ASSESSMENT DATA BASE
Activity/Rest
Problems in sleeping
Ego Integrity
Detached, separated from work, withdrawn, restive, may be passive
Verbal/nonverbal communication may be incongruent
Demonstrates repetitive stereotypical motor behaviors (hand flicking, head banging, complex whole-body movements)
Elimination
Disturbances in bowel and bladder functioning
Food/Fluid
Disturbed eating patterns
Hygiene
Generally dependent
Eccentric preoccupation with one area of hygiene and neglect of another (e.g., showering repeatedly but never brushing teeth)
Neurosensory
Abnormalities noted in almost every sphere of development
Delayed motor, perceptual, cognitive, and language development
Soft neurological signs are often seen (e.g., slight tremors, slowed responses)
Varied/bizarre responses to the environment, with resistance or extreme behavioral reactions to minor occurrences; ritualistic behaviors; extreme fascination with moving objects; special interests in music (although heightened hearing/inability to filter or dampen sounds may result in intolerance)
Bizarre facial expressions
Alterations in mood—lacking the gradations in range of fear, sadness, joy
Unreasonable insistence on following routines in precise detail; marked distress over changes in trivial aspects of environment
Difficulty communicating verbally, with delays in/no development of speech; may mimic sounds made by others, incorrect use of words, echolalia, inability to understand abstract terms; consistent reversal of pronouns “I” and “you”
May show periods of extreme agitation in which behavior becomes disruptive and unmanageable
Does not initiate social imitative play appropriate for stage of development
Safety
Self-mutilative behaviors (e.g., head banging, hair pulling)
Lack of appropriate fear/ignoring signs of danger (e.g., running into street with heavy traffic); fearing harmless objects such as shrinking from touch, going limp or stiffening when held (autism), putting people off by abrupt/awkward approaches (Asperger’s)
Social Interactions
Poor eye contact, impaired responsiveness/communication when interacting with others
Severely disturbed/impaired development in social relationships; may be barely able to distinguish parents from strangers (autism)
Marked impairment in use of nonverbal gestures associated with social interactions
Lack of social or emotional reciprocity, does not express pleasure toward or in response to other people’s happiness; indifference or aversion to physical contact
Teaching/Learning
High association with mental retardation; normal or high verbal intelligence (Asperger’s)
Onset during infancy or early childhood before age 3 (autism) with telltale symptoms possibly noted during first months; marked regression following at least 2 years of apparently normal development, and before age 10 (disintegrative disorder), predominantly males; diagnosis of Rett’s made at about 5 months of age, occurring only in females
DIAGNOSTIC STUDIES
Neurological examination to determine presence and/or extent of organic impairment.
BEAM/PET Scans: May reveal abnormalities in cerebellum (regulates motion and some aspects of memory) and the limbic region (controls much of emotional life).
EEG: May be abnormal, reflecting presence/extent of organic impairment.
Psychological Testing/Intelligence Quotient (IQ): Provides information about cognitive and personality functioning; IQ below 70 may be noted.
Biochemical Studies: Abnormalities not consistently noted.
Laboratory Tests: As indicated by antipsychotic drug therapy.
Auditory Testing: To rule out deafness as a cause of speech problems.
Vision Testing: To differentiate responses to auditory and visual stimuli as abnormal reactions versus distorted perceptions.
Developmental Testing (e.g., Denver Developmental): May reveal delays.
Determine physical causes for disturbances in age-appropriate functions and behaviors (e.g., toileting problems).
NURSING PRIORITIES
1. Facilitate control/decrease of behavioral symptoms.
2. Enhance communication skills and social interaction.
3. Promote family involvement in treatment process and acceptance of child’s disability.
DISCHARGE GOALS
1. Current behavior problems or troublesome symptoms for which treatment is being sought are effectively managed.
2. Treatment within the community is maintained; institutional placement is avoided, when possible.
3. Family verbalizes knowledge about resources to meet the need for a long-term structured therapeutic program.
4. Plan is in place to meet needs after discharge.
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