Neurodevelopmental Disorders

Neurodevelopmental Disorders

This group of conditions has its onset in developmental period. (0-18)

The disorders typically manifest early in development, often before the child enters grade school, and are characterized by developmental deficits that produce impairments of personal, social, academic, or occupational functioning.

Neurodevelopmental Disorders continued

•These disorders may be very specific limitations in learning or control of executive functions to global impairment of social skills or intellectual abilities.

•Originally these disorders were categorically defined, but more recently there is shift to measurement that defines the range of severity and demonstrating that clear boundaries are difficult to delineate.

•These disorders often co-occur with each other, Intellectual Developmental Disorder may appear with Autism Spectrum Disorder, or Attention Deficit Disorder may be present with Oppositional Defiant Disorder.

Types of Neurodevelopmental Disorders

1. Intellectual Developmental Disorder

2. Communication Disorders

3. Autism Spectrum Disorder

4. Attention-Deficit/Hyperactivity Disorder

5. Specific Learning Disorders

6. Motor Disorders

7. Other Neurodevelopmental Disorders

The diagnosis of Intellectual Developmental Disorder

(Intellectual Disability) replaces the previous diagnosis---mental retardation.

Intellectual Developmental Disorder has an onset during the developmental period with characteristic deficits in cognitive functioning and adaptive behaviors.

Rosa's Law

-On October 5, 2010, U.S. President Barack Obama officially signed bill S. 2781 into federal law.

-Rosa's Law removes the terms "mental retardation" and "mentally retarded" from federal health, education and labor policy and replaces them with people first language "individual with an intellectual disability" and "intellectual disability."

DSM IV-TR Criteria of IDD

•Significantly sub-average general intellectual functioning

•score of 70 or below on individual measure of intelligence)

•Significant limitations in two levels of adaptive functioning

•communications, self-care, home living, social/interpersonal skills, self-direction, functional academic skills, work leisure, health, safety)

•Onset before age 18

Severity Criteria DSM IV-TR

•Levels of Severity in DSM IV-TR

(Percent Diagnosis)

IQ Measure

•Mild (85%) 70 to 50-55

•Moderate (10%) 50-55 to 35-40

•Severe (3 to 4%) 35-40 to 20-25

•Profound (1 to 2 %) Below 20-25

Intellectual Developmental Disorder DSM 5 Criteria

Intellectual developmental has an onset during the developmental period that includes both intellectual and adaptive functioning deficits in conceptual, social and practical domains.

Intellectual Developmental disorder DSM 5-TR Criteria continued

•The following three criteria must be met (A, B, C)

•A. Deficits in intellectual functions (reasoning, problem solving, planning, abstract thinking, judgment, academic learning, and learning from experience.)

•B. Deficits in adaptive functioning that results in failure to meet developmental and sociocultural standards for personal independence and social responsibility.

•C. Onset of intellectual & adaptive deficits in developmental period.

•Defined on the basis of adaptive functioning and not by IQ. Adaptive function determines the level of supports required and are less valid in the lower end of the IQ range.

Specify level of severity for IDD

•Intellectual Developmental Disorder requires a severity diagnosis that is coded on ICD– 10 as (corrected coding)

•F 70--Mild;

•F 71--Moderate;

•F 72--Severe;

•F73--Profound.

•Severity is defined on the basis of adaptive functioning and not IQ scores, because adaptive functioning determines required level of supports.

Tests of Adaptive Functioning

•Woodcock-Johnson Scales of Independent Behavior:

•This test measures independent behavior in children.

•Vineland Adaptive Behavior Scale (VABS):

•This test measures the social skills of people from birth to 19 years of age. This test is not administered directly to the child. Instead, questions are directed to primary caregivers and other people familiar with the child. The test contains four sections. These are communication; daily living skills; socialization; and motor skills. This test is also used for children with behavioral disorders, and physical handicaps.

•The Diagnostic Adaptive Behavior Scale (AAIDD, 2013):

•This test measures adaptive behavioral skills. There are three main categories of these skills. This includes conceptual, social, and practical life skills. This test is very helpful for determining the intensity and types of supports needed to maximize independent functioning and quality of life. However, a more useful and appropriate test for that purpose is the Supports Intensity Scale (AAIDD, 2004).

Domains of Assessment

Levels of severity are identified across three domains

1. Conceptual domain

Competence in memory, language, reading, writing, math, acquisition of knowledge, problem solving & judgment.

2. Social domain

Empathy, feelings, thoughts, emotions of others, social judgment, friendship, communication skills

3. Practical domain

Learning & management skills, personal care, money & self management, work or school organization, daily living skills.

Intellectual Developmental Disability Prevalence

Intellectual Developmental Disability has an overall general population prevalence of approximately 10 per 1,000 persons, however the global prevalence differs by country and level of development, being 16 per 1,000 in middle-income countries and 9 per 1,000 in high-income countries.

The prevalence varies by age, being higher in youth than adults. In the United States, prevalence per 1,000 population does not vary significantly by ethno-racial groups.

Specific Issues in IDD

•Onset of the disorder is before 18 & may vary according to the type of brain disorder & dysfunction.

•Within two years many developmental disorders indicate delayed motor, language & social milestones.

•Mild levels of IDD may not be readily identifiable until 5 & the beginning of more formal learning activities.

•Developmental/genetic disorders may have facial stigmata or specific behavioral appearance.

•Many disorders of intellectual development are nonprogressive, however in some there are periods of worsening and then stabilization (e.g., Rett syndrome, Sanfilippo syndrome, Down syndrome)

•Conditions of cognitive functioning may be influenced by other changes in hearing, vision, motor functioning.

Cultural Issues of IDD

•Several factors may influence differences in ethno-racial groups.

•Environmental factors, such as perinatal injury, chronic social deprivation, may influence diagnosis.

•Access to health care & socioeconomic status all impact on diagnosis of IDD

•In Australia 39 per 1,000 persons are the statistics of the Aboriginal population, whereas 16 per 1,000 are the statistics for more affluent, non-Aboriginal youth.

•Factors that impact on assessment differences, linguistic backgrounds, socioeconomic status, cultural & community setting, environmental experiences, cultural beliefs about supernatural influences on personal functioning.

Global Developmental Delay (F88)

•For individuals under 5 when assessment cannot be adequately assessed in early childhood.

-Milestones not met, but cannot be tested. Reassessment necessary.

Unspecified Intellectual Disability (F79)

•Individuals over age 5 when unable to be assessed locally.

-Reserved for unusual or extraordinary circumstances.

Language Disorder (F80.2)

Essential features are difficulties of acquisition and use of language due to deficits in the comprehension or production of vocabulary, sentence structure, discourse.

Expressive ability refers to production of vocal, gestural or verbal signals, whereas receptive ability refers to ability to process and receive language signals

Language Disorder--F80.2 Criteria

•A. Persistent difficulties in the acquisition and use of language across modalities

•Reduced vocabulary

•Limited sentence structure

•Impairment in discourse

•B. Language abilities are substantially and quantifiably below those expected for age resulting in limitations

•C. Onset in early developmental period

•D. Difficulties not attributable to hearing or other sensory impairment or medical condition

Language Disorder--F80.2 Characteristics

•Vocabulary & grammar are limited thus negatively impacting the child's discourse.

•Vocabulary is smaller than expected & less varied

•Sentences are shorter & less complex

•Deficits in vocabulary make language comprehension difficult

•Word finding problems, impoverished verbal definitions, poor understanding of synonyms.

•Difficulty with word or number lists, difficulty with new sequences of sounds.

•All these deficits are below age & developmental milestones for language.

Speech Sound Disorder (F80.0)(previously Phonological Disorder)

•A. Persistent difficulty with speech sound production that interferes with speech intelligibility or prevents verbal communication or messages

•B. Disturbance causes limitation in effective communication that interferes with social participation, academic achievement, or occupational performance

•C. Onset of symptoms in early developmental period

•D. No due to congenital or acquired conditions

Speech Sound Disorder (F80.0) Characteristics

•Children with this condition have difficulty with articulation of speech sounds & do not follow the developmental pattern for their age group.

•Intelligible speech should emerge at around 3 years.

•By age 5 years, children should be producing speech sounds clearly & accurately according to their cultural & community norms.

•Misarticulation of the "late eight" (l, r, s, th, ch, dzh, & zh) are not unusual until the child reaches age 8 years.

•Treatment is effective with most children unless a child also has a language disorder.

Childhood-Onset Fluency Disorder (F80.81) (Stuttering)

Disturbance in normal fluency and time patterning of speech uncharacteristic of developmental level

Childhood-Onset Fluency Disorder (F80.81) (Stuttering) criteria

•A. Disturbances inappropriate for the individual's age and language skills, persists over time, and characterized by frequent and marked occurrences (one or more of following)

•Sound and syllable repetitions

•Sound & prolongations of consonants & vowels

•Broken words

•Audible or silent blocking

•Circumlocutions

•Words produced with excess of physical tension

•Monosyllabic whole word repetitions

•B. Causes anxiety about public speaking and limitations in effective communication

•C. Early developmental period

•Later onset is adult-onset fluency disorder

•D. Not speech-motor or sensory deficit or neurological insult or other medical condition.

Social (Pragmatic) Communication Disorder (F80.99)

Primary difficulty with pragmatics or the social use of language & communication as manifested by deficits in understanding and following social rules of verbal and nonverbal communication

Social (Pragmatic) Communication Disorder (F80.99) Criteria

A. Persistent difficulty in the social use of verbal & nonverbal communication

Deficits in using communication for social purposes

Impairment of the ability to change communication to match the context of listener

Difficulties following rules for conversation or story telling

Difficulties in understanding what is not explicitly stated & nonliteral or ambiguous meanings of language

Idioms

Humor

Metaphors

B. Deficits in functional limitations in effective communication, social participation, social relationships, academic achievement or occupational performance

C. Onset in early developmental period

D. Not attributable to neurological or medical disorder. Not better explained by autism, intellectual disability, or other mental disorder

Social Communication Disorder (F80.99) characteristics

•By age 4 or 5, children should possess adequate speech & language skills to permit identification of specific deficits in social communication.

•Autism spectrum disorder issue ma be differentiated by presence of autism's restrictive & repetitive patterns of behavior, interests & activities.

•Only diagnose this disorder if the above symptoms are not revealed on examination.

Autism Spectrum Disorder (F84.0)

The essential feature of Autistic Disorder is persistent deficits in social communication & social interaction across a number of contexts.

Autism Spectrum Disorder replaced:

-Autistic disorder (autism),

-Asperger's disorder,

-Childhood Disintegrative Disorder, and

-Pervasive Developmental Disorder -Not Otherwise Specified

Autism Spectrum Disorder (F84.0) criteria

A. Persistent deficits in social communication and social interaction in multiple contexts as manifest by all of the following

B. Restricted, repetitive patterns of behavior, interests, activities

C. Present in early development but may not fully manifest until social demands exceed capabilities

D. Clinically significant impairment social, occupational, or other areas

Criteria A. Deficits in social communication and social interaction across multiple contexts are required for this diagnosis and manifest by all following

•Deficits in social emotional reciprocity

•Deficits in nonverbal communication behaviors used for social interaction

•Deficits in developing, maintaining, and understanding relationships

•Severity is based on social communication impairment & restricted, repetitive patterns of behavior

Criteria B. Restricted, repetitive patterns of behavior, interests, or activities manifest by two of the following

•Stereotyped or repetitive motor movements, use of toys or speech

•Insistence on sameness, inflexible adherence to routines or ritualized patterns of verbal or nonverbal behavior

•Highly restricted, fixated interests that are abnormal in intensity or focus

•Hyper-or hypo-reactive to sensory input or unusual interest in sensory aspects of the environment

Some Specifiers for Autism Spectrum

•With or without accompanying intellectual impairment,

•With or without accompanying language impairment,

•Associated with another neurodevelopmental, mental, behavioral disorder,

•Associated with known medical or genetic condition or environmental factor (code to identify associated genetic or medical condition)

•With catatonia- additional code autism (F06.1)

Specifiers for Autism Spectrum

Severity

•Mild

-Requiring support

•Moderate

-Requiring substantial support

•Severe

-Requiring very substantial support

Prevalence of Autism Spectrum Disorder

-Frequencies for autism spectrum disorder in U.S. between 1% to 2%.

-Frequencies in African Americans appear to be at1.1% & Latinx children (0.8%) with White children (1.3%) even when SES is considered.

-Prevalence of autism spectrum disorder in non-U.S. countries is 1% of the population (0.62%) a global prevalence without much variation based on geographic regional or ethnicity.

-Diagnosed 3 to 4x more often in males than females.

-Globally, the male: female ratio is 3:1, with concerns about under-recognition & diagnosis in females.

More specifiers of Autism Spectrum Disorder

•Specifier, related to language, should be assessed & described. Examples of descriptors:

-No intelligible speech, single words only, or phrase speech.

-Expressive & descriptive language should be considered separately

-Specifier, genetic, medical or environmental factors applied when condition know (associated-not necessarily causation)

-Genetic: Rett syndrome, Down syndrome

-Medical: epilepsy

-Exposure: Fetal valproate syndrome, Fetal alcohol syndrome, Fetal Rubella

Associated features of Autism Spectrum Disorder

1. Theory of mind deficits

-Inability to understand others perspectives

2. May be slow to talk with language comprehension deficits

3. Executive function deficits may be present

•Difficulty with big picture, focus on/in details of task

4. Motor deficits

•Odd gait, clumsiness, walking on tiptoes, space issues

5. Self-injury may be present

•Head banging (common), biting self

6. Symptoms usually recognized in second year of life (12 to 24 months)

7. Rule out visual impairments & hearing impairments

Attention Deficit/Hyperactivity Disorder

•A. Essential feature is persistent pattern of inattention and/or hyperactivity-impulsivity that interferes with functioning or development.

•Several inattentive and hyper-impulsive symptoms present prior to age 12.

•Present in two or more contexts/setting

•B. Several inattentive or hyperactive-impulsive symptoms prior to 12

•C. Several inattentive or hyperactive-impulsive symptoms present in two or more contexts

•D. Clear evidence that the symptoms interfere with or reduce social, occupational, or academic functions

•E. Do not occur exclusively during course of schizophrenia or other psychotic disorder or another mental disorder

Inattention in ADHD

Six (6) of following symptoms have persisted for at least 6 months to a degree inconsistent with developmental level

Fails to give close attention to details or makes careless mistakes

Difficulty sustaining attention in tasks or play activities

Does not seem to listen when spoken to directly

Does not follow through on instructions or fail school-work

Difficulty organizing tasks and activities

Avoids, dislikes, or is reluctant to engage in tasks that require sustained mental efforts

Loses things necessary for tasks or activities

Easily distracted by extraneous stimuli or unrelated thoughts

Forgetful in daily activities

Hyperactivity in ADHD

Six or more of the following symptoms have persisted for at least 6 months to a degree that is inconsistent with developmental level (not sole manifestation of ODD)

Fidgets with or taps hands or feet or squirms in seat

Leaves seat in situations when remaining seated is expected

Runs about or climbs in situations where it is inappropriate

Unable to play or engage in leisure activities quietly

Often on the go, as if driven by a motor

Talks excessively

Blurts out answer before question has been completed

Difficulty waiting for turn

Interrupts or intrudes on others

Specifiers for ADHD

•(F90.2) Combined presentation

•(F90.0) Predominantly inattentive presentation

•(F90.1) Predominantly hyperactive/impulsive presentation

•In partial remission

•Level of severity

•Mild

-Few symptoms in excess of diagnosis

•Moderate

•Severe

-Many symptoms in excess of diagnosis or several severe symptoms

•Note: Can be diagnosed with autism spectrum disorder

Other Specified Attention-Deficit/Hyperactivity Disorder 314.01 (F90.8)

•Presentations in which the symptoms of ADHD cause stress and impairment but full criteria are not met.

Unspecified Attention-Deficit/Hyperactivity Disorder 314.01 (F90.9)

•Full criteria for ADHD not met but distress and impairment are present, but clinician chooses not to specify the reason

Factors in ADHD

•Prevalence

-Worldwide about 7.2% children. From 0.1 to 10.2 % of children & adolescents. More common in foster or correctional settings. In adults, occurs in 2.5 percent.

•Neurodevelopmental disorders

-Children with Fragile X syndrome or 22q11 deletion syndrome are more likely to have inattention & hyperactivity but should be diagnosed with ADHD if meet full criteria.

•Heritability at 74%.

•More frequent in males than females in general population, ratio 2:1 & in adults 1.6:1.

•Cultural factors

-African American & Latinx diagnosis lower than for White populations

Some Stimulant Medications for ADHD

Amphetamine & dextroamphetamine

Adderall

Adderall XR (extended release)

Methylphenidate

Concerta (extended release)

Daytrana (patch)

Desoxyn

Metadate ER & CD

Methylin

Ritalin

Ritalin SR & LA (extended release)

JORNAY PM (extended release)

Dextroamphetamine

Dexedrine

Dextrostat

Dexmethylphenidate

Focalin

Focalin XR (extended release)

Lisdexaminefetamine dimesylant

Vyvanse

Some Non-Stimulant Medications for ADHD

Atomoxetine (Strattera)

class of medications known as selective norepinephrine reuptake inhibitors

Guanfacine (Intuniv)

used alone or in combination with other medications to treat high blood pressure & may treat ADHD by affecting the part of the brain that controls attention and impulsivity.

Clonidine (Kapvay)

alpha2 agonists on PFC

Specific Learning Disorder

Persistent difficulties in learning and using academic skills (reading, spelling, writing, mathematics) persisting for 6 months.

Specific Learning Disorder Criteria

•A. Difficulties learning and using academic skills by presence of one of the following persisting for 6 months

Inaccurate or slow & effortful word reading

Difficulty understanding meaning of what is read

Difficulties with spelling

Difficulties with written expression

Difficulties with mastering number sense, number facts, or calculations

Difficulties with math reasoning

B. Affected academic skills are substantially and quantifiably below those expected for the individual's chronological age, and cause significant interference with academic or occupational performance

C. Learning difficulties began in school age, but may not manifest until the demands for those skills exceeded the individual's capacities

D. Learning difficulties not better accounted for by intellectual disabilities, uncorrected visual or auditory acuity, other mental or neurological disorders.

Specifiers: Specific Learning Disorder

•Specifiers:

•(F81.0) with impairment in reading

-reading accuracy, rate and fluency, comprehension

-Dyslexia term referring to problems in accurate or fluent word recognition, poor decoding, & poor spelling.

•(F81.81) with impairment in written expression

-spelling accuracy, grammar and punctuation, written expression and organization

•(F81.2) with impairment mathematics

-number sense, memorization, arithmetic facts, fluent calculation, math reasoning

Severity Specifiers: Specific Learning Disorder

•Mild

-some difficulty in one or two domains, but of mild enough severity that the individual may be able to compensate or function well when provided with accommodations

•Moderate

-marked difficulty in learning skills in one or more domains, so ha the individual not likely to become proficient without intensive and specialized teaching. Accommodations necessary for accuracy and efficiency.

•Severe

-difficulty is severe affecting several academic domains. Unlikely to acquire skills with specialized teaching, not able to competently or efficiently complete activities even with accommodations

Features of Specific Learning Disabilities

•May demonstrate above-average drawing, design, or visuospatial abilities with poor reading comprehension

•May demonstrate mechanical giftedness or aptitude

•Cognitive deficits appear in reading words, difficulty acquiring math skills

•Other disorders may or may not be present

•May demonstrate baby talk, mispronounce words, have trouble with letters or even trouble learning to count.

•Adolescence reading may remain slow and effortful.

•Anxiety disorders, somatic complaints & panic disorder may manifest

Prevalence of Specific Learning Disorders

•Prevalence in U.S., Brazil & Northern Ireland, 5% to 15% of school aged children.

•Occurs across linguistic & ethno-racial backgrounds.

•In English clinical symptoms of difficulty reading is inaccurate and slow reading of single words.

•Spanish, German (direct mapping with sounds & letters) or Chinese, Japanese (non-alphabetic languages) slow but accurate reading.

•More common in males than females, ratios 2:1 or 3:1

•Associated with depression, feelings of self-deprecation, suicidal thoughts

•Underemployment & lower incomes may result.

Developmental Coordination Disorder (F82)

•A. Acquisition & execution of coordinated motor skills below expected for given age

•B. Persistent & significantly interferes in daily living for chronological age

•C. Onset in early developmental period

•D. Motor deficits not explained by intellectual disability, visual impairment or neurological disorders.

Features of developmental coordination disorder

•Developmental coordination disorders show additional motor activities, sometimes suppressed.

-Choreiform movements

•These overflow movements are neurodevelopmental immaturities/neurological soft signs rather than neurological abnormalities

•Prevalence in 5 to 11-year-olds is 5% to 8% cross nationally.

-UK 1.8% to 3%

-Canada, Sweden, Taiwan 7% to 8%

-Males more than females, 2:1 and 7:1

Stereotypic Movement Disorder (F98.4)

•A. Repetitive, seemingly driven and purposeless motor behaviors

•B. Repetitive motor behavior interferes with social, academic, or other activity and may result in self injury

•C. Onset in early development

•D. Motor behavior not attributable to the effects of substance or other mental disorder

Stereotypic Movement Disorder (F98.4) specifiers

Specify:

With self-injurious behavior

Without self-injurious behavior

Specify if associated with medical condition or genetic disorder

Lesch-Nyhan syndrome, intellectual developmental disorder, intrauterine alcohol exposure

Specify:

Mild

Symptoms easily suppressed by sensory stimulus or distraction

Moderate

Symptoms require explicit protective measures & behavioral modification

Severe

Continuous monitoring & protective measures are required to prevent serious injury

Tourette's Disorder (F95.2)

A. Both multiple motor and one of more vocal tics have been present at some time during illness

B. Ticks may wax & wane in frequency but have persisted for more than one year since onset

C. Onset before 18

D. Disturbance not due to substances or other medical conditions

Persistent (Chronic) Motor or Vocal Tic Disorder (F95.0)

A. Single or multiple motor or vocal tics that have been present during the illness, but not both motor and vocal

B. The tics may wax and wane in frequency but have persisted for more than 1 year since first tic onset

C. Onset before 18

D. Disturbance not attributable to the effects of substance or other medical condition (e.g., Huntington's disease, postviral encephalitis)

E. Not meet criteria for Tourette's

•Specify if:

-Motor tics only

-Vocal tics only

Provisional Tic Disorder (F95.0

•A. Single or multiple motor and /or vocal tics

•B. The tics been present for less than 1 yea since first tic onset

•C. Onset before age 18

•D. Disturbance not attributable to the effects of substance or other medical condition (e.g., Huntington's disease, postviral encephalitis)

•E. Criteria never met for Tourette's or persistent motor or vocal tic disorder

Various Tics

There are two types of tics:

—Physical tics (motor tics) - motor movements, such as blinking, jerking the head or any part of the body.

—Phonic tics (vocal tics) - uttering sounds, such as grunts or squeaks, and words or phrases.

There are two main classifications of ticks:

—Simple ticks - this may involve moving just one muscle, or uttering a single sound. Movements are sudden, short lived, and often repetitive.

—Complex ticks –

—the physical movements are more complex, and/or the phonic tics involve a more sophisticated set of utterances, including long phrases. Complex tics involve several muscle groups.

—Echopraxia (imitate movements); Copropraxia (sexual or taboo gestures); Palilalia (repeating one’s own sounds); Echolalia (last heard words); Coprolalia (socially inappropriate, obscenities, or slurs)

Tics

Simple physical tics

•Eye blinking

•Eye darting

•Grinding the teeth

•Head jerking

•Neck twisting

•Nose twitching

•Rolling the eyes

•Rotating the shoulders

•Shoulder shrugging

•Sticking the tongue out

Complex tics

•Copropraxia - making obscene gestures

•Echopraxia - miming the movements of other people

•Flapping

•Head shaking

•Hitting things

•Jumping or hopping

•Kicking things

•Shaking

•Smelling objects

•Touching oneself

•Touching others

Features of Tic Disorders

•Tics are common in childhood.

•Prevalence in U.S. 3 per 1000. Lower diagnosis among African Americans or Latinx. Estimated prevalence in Canada of Tourette's from 3 to 9 per 1,000 school age children.

•Globally, males are more affected than females, ratio 2:1 to 4:1.

•Onset between 4 to 6 years with eye blinking often initial symptom. Peak severity between 10 to 12 years. Severity declines in adolescence.

•Heritability of tic disorders 70% to 85%, but no difference in male and female heritability. Genetic variance with OCD, ADHD and other neurodevelopmental disorder (autism spectrum disorder)

Other Neurodevelopmental Disorders

•Other Specified Tic Disorder (F95.8)

•Unspecified Tic Disorder (F95.9)

•Other Specified Neurodevelopmental Disorder (F88)

•Unspecified Neurodevelopmental Disorder (F89)