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Childh d A i fChildhood Apraxia of SpeechSpeech
Cheryl D Tierney MD MPHCheryl D. Tierney, MD, MPHAssistant Professor Of Pediatrics
Penn State Hershey Children's HospitalDepartment of Pediatrics
Division of Human Genetics, Growth and Development
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There are no disclosures related to today’s
t tipresentation
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Objectives•Relate key speech and language concepts
Objectives
necessary to understand Childhood Apraxia of Speech (CAS)
•Discuss evidence available to guide treatment of Childhood Apraxia of SpeechChildhood Apraxia of Speech
•Review considerations of care in the child with Childhood Apraxia of Speech
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Case of Henry
Henry is a 2 1/2 year old who presents today for a WCC. Hi f il j t d d i t bli hi ith
y
His family just moved and is establishing care with you.As recommended by the AAP Practice Guidelines (2006) you administer a developmental surveillance tool andyou administer a developmental surveillance tool and review it before you enter the room. You notice mom indicates a concern with Henry’s
i ti H i i ST th h EI f 18communication. He was receiving ST through EI from 18 months but progress was noted to be slow.Because of this, mom wonders if she should try and get EI , y gstarted here or just wait and see?
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Conceptual Framework: What is speech and what is language?language?
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Conceptual Framework: What is speech and what is language?
Dysfluency
language?
Dysfluency
Articulation Disorder / Phonological Disorder
Childhood Apraxia of Speech
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DysfluencyDysfluency
Stuttering: Interruptions in the normal flow of speechStuttering: Interruptions in the normal flow of speech
Typical •Whole word or phrase repetitions
Less Typical •Repetition of sounds •Repetitions of syllablesp
•Interjections •Revisions•Hesitations
•Repetitions of syllables •Prolongation
Etiology unknown•Hesitations Etiology unknownAges affected: 2-5 yrsOften resolves on its own Exacerbating factors include Stress, Fatigue & Language complexity
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Articulation/Phonological Di dDisorder
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Articulation Disorder•A person cannot produce the sounds necessary for
Articulation Disorder
correct speech.
•Predictable: Unable to make: p, b, m by 3 years Unable to make: d, n, k by 4 years Unable to make sh and th by 6 years Unable to make sh and th by 6 years Unable to make s by age 7
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Phonological DisorderPhonological Disorder
Poor use of certain speech sounds expected for age
o Predictable errors, consonants more commono M > F o 3% of preschool children and 2% of children 6 - 7yr o Prognosis is generally good for normal or near-o Prognosis is generally good for normal or near-
normal speech
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Childhood Apraxia of S hSpeech
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Childhood Apraxia of SpeechChildhood Apraxia of Speech
Neurological speech sound disorder That affects a child’s ability to coordinate the muscles of the tongue, lips, mouth and jaw to
make accurate and reproducible speech dsounds
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GeneticsForkhead Box Promoter Gene7q31, at the SPCH1
locus Promoter Gene (FOXP2)
locus
Gene Mutation or deletionleads to decreased protein productionp p
FOXP2 Protein
*
Protein
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Genetics
FOXP2 is required for proper brain and lung q p p gdevelopment.
-mice studies
FOXP2 may regulate genes involved in l ti itneuroplasticity: songbird studies bat studies
Haesler S, Wada K, Nshdejan A, Morrisey EE, Lints T, Jarvis ED, Scharff C (March 2004). "FoxP2 expression in avian vocal learners and non-learners". J. Neurosci. 24 (13): 3164–75. doi:10.1523/JNEUROSCI.4369-03.2004.
Teramitsu I White SA (July 2006) "FoxP2 regulation during undirected singing in adult songbirds" J Neurosci 26 (28): 7390 4Teramitsu I, White SA (July 2006). FoxP2 regulation during undirected singing in adult songbirds . J. Neurosci. 26 (28): 7390–4. doi:10.1523/JNEUROSCI.1662-06.2006.
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Genetics: Human correlates
Apraxia in humans has been linked to mutations in the FOXP2 gene.
fMRI analysis of these individuals performing silent verb generation and spoken word repetition tasksand spoken word repetition tasks showed under-activation of Broca's area and in the putamen. p
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Suggestive History
•Feeding problems
gg y
•Feeding problems•Prolonged drooling •Hard time blowing bubbles•Trouble drinking through a strawTrouble drinking through a straw•Weak kisses
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Distinguishing characteristicsg gPhonological Disorders CAS
Consonant omissions and substitution errors
Consonant omissions and substitution errors
common uncommon
D l ti t ifi T i l d l tiDeletions not specific to position
Terminal deletionscommon
Accuracy unrelated to # syllables in words
Accuracy reduced with longer utterances# syllables in words with longer utterances
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Features• Limited babbling as an infant
Featuresg
• Delayed onset of speechRestricted sound inventory• Restricted sound inventory
• lots of words.....all with the same sound• ‘word approximations’• word approximations
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Features•Error (un)predictability
V l i t•Vowel errors prominent
•Effect of increased complexityEffect of increased complexity
•Dysdiadokokinesis effect/ / / / / / / / / / /k //pa/, /pa/, /pa/ versus /pa/ /ta/ /ka/
•Disturbances of prosody•Disturbances of prosody
•Groping (age effect)
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Back to Our Case:You ask some more questions and you find that she has
few words, many of which are word approximations ex da for daddy, ma for mommy, ba for bottle
She is frustrated and gets angry easily when she is notShe is frustrated and gets angry easily when she is not understood.
She is wearing a bib around her neck that you remove when you do your physical exam
• History suggests a possible speech disorder, or maybe apraxiaapraxia...
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An approach: My child isn’t t lki• Identify all domains of developmental delay first
talking…• Identify all domains of developmental delay first
Refer to Early Intervention Does the profile you see fit a speech disorder?
• Refer for confirmatory testing Tx needs to be geared to the dx in terms of
frequency intensity and approachfrequency, intensity and approach• Consider the co-morbidities possible
Ex. Apraxia in children with autism• Consider alternative and augmentative
communication devices (AAC) for voice and for keeping up with learning while speech developskeeping up with learning while speech develops
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Once upon a time..p
EVALUATE AND TREATEVALUATE AND TREATDX SPEECH DELAY
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Developmental DomainsMotor
fine motortgross motor
speech
Cognitivevisual motorplayadaptive skillsadaptive skills
Communicationnonverbal skillsreceptive languageexpressive languagespeech social interactions
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Speech and Language p g gEvaluation•Possible speech disorder r/o apraxia of speech
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The EvaluationExpressive & Receptive L
The Evaluation
Language
Speech
Phonemic awareness and early literacy (for ages 4-5 and older)literacy (for ages 4-5 and older)
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How Many Treatment Sessions Are Required t I M Child' S h?
Phonological disorder
to Improve My Child's Speech?
ApraxiaPhonological disorder 21-42 sessions (x 29)
Apraxia144-168 sessions(x 151)
1/2 3/4
Children with apraxia of speech required 81% more individual treatment sessions than the children with severe phonological disorders in order to achieve a similar functional outcome.
Thomas F. Campbell, "Functional Treatment Outcomes in Young Children with Motor Speech Disorders," Clinical Management of Motor Speech Disorders in Children, edited by Anthony J. Caruso and Edythe A. Strand, Thieme Medical Publishers Inc., New York, 1999.
similar functional outcome.
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Treatment Evidence Summary•Although there are differences in definitions of
Treatment Evidence Summary
intensive remediation for children with apraxia, there appears to be emerging consensus within the literature that therapy should be conducted at leastliterature that therapy should be conducted at least three to five times weekly, in sessions lasting between 30 and 60 minutes each, and that thebetween 30 and 60 minutes each, and that the intervention should be conducted on an individual basis (1:1).
Penelope K. Hall, Linda S. Jordan, Donald A. Robin, Developmental Apraxia of Speech: Theory and CClinical Practice , 2nd Edition, page 200, Pro-ed Publishers, Texas, 2007.
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What evidence exists to guide treatment?
• CAS over-rely on their own imprecise auditory feedback . Slowing down the rate of articulation can help correct this over
treatment?
o Slowing down the rate of articulation can help correct this over-reliance and may be a new angle for intervention [1].
• There is a lack of dissociation and decreased coordination amongThere is a lack of dissociation and decreased coordination among tongue tip, lower lip and jaw movements compared with normal age-matched peers [2]
• Therapeutic emphasis on training more difficult speech targets yields generalization to simpler sound productions[3].
[1]Ballard KJ, Robin DA, McCabe P, McDonald J. A treatment for dysprosody in childhood apraxia of speech. J Speech Lang Hear Res 2010; 53:1227-45[2]Terband H, Maassen B, van Lieshout P, Nijland L. Stability and composition of functional synergies for speech movements in children with developmental speech disorders. J Commun Disord 2010; 44:59-74.[3]Iuzzini J, Forrest K. Evaluation of a combined treatment approach for childhood apraxia of speech. Clin Linguist Phon 2009; 24:335-45..
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Treatment programs availableg
•Multisensory programs work bestMultisensory programs work best.
PROMPT•PROMPT•Moving Across SyllablesW d Fli•Word Flips
•Kaufman Praxis
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Apraxia's link to Literacy
Phonological Awareness is knowing that sentences are d f d d d f ll bl d
y
made up of words, words are made up of syllables and syllables are made up of sounds.
Phonemic awareness is required foro Rhymingo Manipulating sounds
"take the c in cat and put a b --- What do you get?"
Phonetics is how we learn to read
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Vocabulary size is strongly related to phonological awarenessphonological awareness
*Velleman SL. Lexical and phonological development in children with childhood apraxia of speech--a commentary on Stoel-Gammon's 'Relationships between lexical and phonological development in young children' J Child Lang; 38:82 6children . J Child Lang; 38:82-6.
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Lexicon phonological Lexicon p gawareness
decreased production of words and fewer vocalizations reduced
1
vocalizationspractice and feedback
decreased skill and
2
decreased skill and motivation
3
higher order literac
decreased vocabulary learning
4
literacy
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What are the long term outcomes f hild ith CAS?for children with CAS?
Children with apraxia, even if they are completely remediated by school entry, are at increased risk of language based learning problems (dyslexia)language based learning problems (dyslexia)
R l ti i t d til i t dRe-evaluation is suggested until age appropriate and reading
• every 6 months under age 3 yearsevery 6 months under age 3 years• yearly from 3 onward• through early literacy years
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So........
Identifying children with a speech / language delay in
So........
practice is important but only part of the story
Speech and language development are different and p g g punderstanding the differences helps to formulate an informed differential diagnosis
Diagnosis (working or confirmed) is very helpful in guiding treatment and advocating for such will only improve ultimate developmental potential for the children we serve
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Back to our case….
We suspected a speech disorder. We requested an evaluation. Henry’s speech pathologist completed the PLS5 and the Kaufman to confirm normal receptive language, moderately delayed expressive language and g g , y y p g gfindings consistent with childhood apraxia of speech.
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Back to our case….
He was treated 3 times per week with an evidence based treatment approach utilizing PROMPT methods by a certified and experienced therapist. He had a combination of school based and private therapy so that p pythe family could learn how to be involved and carry over the goals of therapy at home.
Henry loved using an Ipad with apraxia applications toHenry loved using an Ipad with apraxia applications to practice at home.
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Back to our case…
3 ½ years later Henry returns for his kindergarten physical exam and mother delights in telling you that he is doing fabulously In fact he is already reading simpleis doing fabulously. In fact, he is already reading simple books and loves rhyming games on his mother’s Ipad.
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Conclusions
Childhood apraxia of speech is a significant and not
Conclusions
uncommon speech sound disorder in children
Diagnosis can be suspected by the pediatrician and g p y pconfirmation with testing is needed by an SLP experienced in apraxia to confirm an ultimate di idiagnosis
Once diagnosed, specialized intervention, education and monitoring will be needed to help children with CAS reach their ultimate communication potential.
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Thank you!!y