Apraxia -What’s That?

Answer from a developmental pediatrician

Presented by Marilyn C. Agin , MD, Medical Director, New York City Early Intervention Program and Medical Director, Cherab Foundation Co-Author of The Late Talker book

Presented at the First Apraxia Conference hosted by Cherab Foundation, July 23-24, 2001, Headquarters Plaza Hotel, Morristown, New Jersey and all presented at Research Workshop – September 20-21 and on September 22, 2001 ‘ Fatty Acids in Neurodevelopmental Disorders’ St Anne’s College, Oxford, UK

The First Scientific Conference on Therapy for Verbal Apraxia/Dyspraxia

What’s in a Name and Definitions

What is apraxia, verbal apraxia (or apraxia of speech or verbal dyspraxia), orofacial apraxia and motor apraxia. How is verbal apraxia treated?

Apraxia is a neurogenic impairment involving planning, executing and sequencing motor movements. Verbal apraxia affects the programming of the articulators and rapid sequences of muscle movements for speech sounds (often associated with hypotonia and sensory integration disorder). Oral apraxia involves nonspeech movements (e.g., blowing, puckering, licking food from the lips). Motor apraxia involves the programming of hand or whole body movement.

Neurodevelopmental Evaluation of Verbal Apraxia: History

  • Limited babbling and oral play
  • Late transition to solids, feeding difficulties
  • Drooling that exceeds typical expectations
  • History of accompanying oral apraxia
  • May have elaborate nonverbal or gestural communication
  • First words may emerge on time, but vocabulary growth is slow
  • Increased frustration, behavior problems
  • Family history of speech, language, learning problem

Nerodevelopmental Evaluation: Physical Neurologic Exam

  • Hypotonia (truncal)
  • May have gross and fine motor incoordination
  • Motor planning difficulties
  • Sensory integration/self-regulatory issues
  • Delayed or mixed dominance

Assessment of Respiration and Phonation

  • Postural tone
  • Head and trunk control
  • Respiratory support for phonation
  • Ability to sound play

Oral Motor Assessment

  • Oral hypotonia
  • Drooling
  • Feeding
  • Suck swallow pattern
  • Chewing
  • Facial Expression

Speech/Language/Cognitive Assessment (1)

  • Receptive language > expressive language
  • Normal to near normal cognitive abilities
  • Limited repertoire of consonant sounds (“da” may be generic)
  • Sounds/syllable omissions, vowel distortion, cluster
  • Increased errors with increased length of utterance
  • Inconsistency of errors

Speech/Language/Cognitive Assessment (2)

  • Prosodic disturbances (monotone)
  • Groping “trial and error” behavior (dysfluencies, silent posturing)
  • Expressive language: more limited lexicon, grammatical errors, disordered syntax
  • School age child: learning difficulties — reading, written expression and spelling

Association with Other Disorders
Some examples are:

  • Cerebral Palsy
  • Down Syndrome
  • Other neurologic syndromes
  • Autistic spectrum disorders
  • Role of “motor apraxia” in autism (1)
  • Role of verbal apraxia in speech and language acquisition (2) (little research is available)

(1) Rapin, ed (1996) Preschool Children with Inadequate Communication

(2) Wetherby, et al (2000) Autism Spectrum Disorders

Verbal Apraxia Controversies (1)

Nomenclature:

Name borrowed from adult model
In adults, apraxia is an acquired condition
Stroke or head injury
Affects Broca’s area and sensorimotor cortex of the dominant hemisphere

Verbal Apraxia Controversies (2)

Etiology

Specific site of lesion has not been demonstrated on a consistent basis in children

EEGs suggested that praxis area in young children involved large cortical areas of both hemispheres with lateralization to left hemisphere in later childhood (1)

Other studies (2,3) report “soft signs” on neurologic exam

Early neuro-imaging studies typically negative (4)

Most studies: small samples, outdated (1) Rosenbeck & Wertz (1972)
(2) Yoss & Darley (1974)
(3) Ferry , Hall $ Hicks (1975)
(4) Horowitz (1984)
Verbal Apraxia Controversies (3)

Diagnosis: Exclusive vs. Inclusive

Group of speech researchers see verbal apraxia as solely a motor speech disorder (1, 2)

This renders apraxia a rarity (estimates 1-2%/1000 live birth)

Misses a great many children with more global dyspraxic syndromes associated with verbal apraxia

They propose that verbal apraxia is more like a symptom cluster or even a spectrum disorder (1) Hall et al. (1993) Developmental

(1) Hall et al. (1993) Developmental Apraxia of Speech
(2) Hayden (1998) PROMPT Manual

Appropriate Therapy (1)

Intensive and frequent

Individual (no benefit from group therapy)

Repetitive practice for habituation of motor learning

Multisensory, including touch-cue system (PROMPT)

Core vocabulary

Successive approximations

Melodic, rhythmic (singing rhymes)

Appropriate Therapy (2)

Difficult course resistant to “traditional methods”

Regression and learning to speak one word at a time

Use of “total communication” approach (e.g. sign language, PECS and augmentative communication devices)

Oral motor techniques–if indicated

“Children with apraxia of speech required 81% more individual therapy sessions…to achieve a similar functional outcome” Campbell (1999) Clinical Management of Motor Speech Disorders

Early Diagnosis (1)

Ongoing developmental surveillance and screening by pediatric practitioners Policy statement from the AAPediatrics and the American Academy of Neurology-CNS

Dispel the myth that all “late talkers” (with no receptive language are “Little Einsteins” (He/She will outgrow it)

Listen to parental concerns because they are accurate indicators of true problems
Dworkin et al (1997) Contemporary Pediatrics

Glascoe (1995) Pediatrics

Early Diagnosis (2)

Referral to Early Intervention

Improves outcome

At no cost for families (in most states)

N-D specialists (neurologists developmental pediatricians) should work collaboratively with SLPs (speech language pathologists) in determining correct diagnosis and treatment plan
Role of Essential Fatty Acids

Supplementation appears to cause dramatic leaps in development in children receiving combination of fish oils (omega-3s) and borage or evening primrose oil (omega-6 oils)

The effect is greater than one can expect from speech therapy alone

Can this effect be clinically validated and how do we account for it?