Childhood DysarthriaChildhood Dysarthria

Kathleen Mikullitz Kathleen Mikullitz &&

Kimme Norton Kimme Norton

What is Childhood Dysarthria?What is Childhood Dysarthria?A group of motor speech disorders that affects A group of motor speech disorders that affects the neuromuscular execution of speechthe neuromuscular execution of speechCharacterized by abnormalities in:Characterized by abnormalities in:

CNS and/or PNSCNS and/or PNSNeuromuscular features, including: strength, Neuromuscular features, including: strength, steadiness, tone, accuracy, and speed and range of steadiness, tone, accuracy, and speed and range of movement movement

Can affect ALL systems necessary for speech:Can affect ALL systems necessary for speech:Respiration, phonation, articulation, resonance, and Respiration, phonation, articulation, resonance, and prosodyprosody

(van Mourik, Catsman(van Mourik, Catsman--Berrevoets, Paquier, YousefBerrevoets, Paquier, Yousef--Bak, & van Dongen, 1997; Duffy, 2005)Bak, & van Dongen, 1997; Duffy, 2005)

Types and SymptomsTypes and Symptoms

Flaccid Flaccid –– Weakness Weakness Spastic Spastic –– RigidityRigidityAtaxic Ataxic –– IncoordinationIncoordinationHypokinetic Hypokinetic –– Rigidity; Problems with initiating Rigidity; Problems with initiating movementmovementHyperkinetic Hyperkinetic –– Involuntary movements Involuntary movements Unilateral Upper Motor Neuron Unilateral Upper Motor Neuron –– Weakness; Weakness; Incoordination Incoordination Mixed Mixed –– VariableVariable

(Duffy, 2005; Andrianopoulos, 2008)

Childhood DysarthriaChildhood Dysarthria

Congenital or Developmental:Congenital or Developmental:The neurologic insult takes place at birth or The neurologic insult takes place at birth or prior to the development of speech and prior to the development of speech and languagelanguage

Acquired:Acquired:The individual may have developed some The individual may have developed some speech and language skills prior to the speech and language skills prior to the neurologic insult neurologic insult

(Caruso & Strand, 1999)

Speech CharacteristicsSpeech CharacteristicsMarked difficulties with strength, speech, and accuracy Marked difficulties with strength, speech, and accuracy of articulatory movement.of articulatory movement.Weak vocal quality (lack of respiratory support)Weak vocal quality (lack of respiratory support)HypoHypo-- or hypernasalityor hypernasalityWeak articulatory contactsWeak articulatory contactsRapid or slow speaking rateRapid or slow speaking rateDistortion of vowels that involve spreading intrinsic Distortion of vowels that involve spreading intrinsic tongue muscles: tongue muscles:

/i/, /ai/, /ei/, and //i/, /ai/, /ei/, and /ɔɔ ɪɪ/./.Imprecise or weakly targeted consonantsImprecise or weakly targeted consonants

/r/, /l/, and /s//r/, /l/, and /s/Generally weak, mushy, garbled, imprecise speechGenerally weak, mushy, garbled, imprecise speech

(McCaffrey, 2008)

NonNon--Speech CharacteristicsSpeech Characteristics

Difficulties with:Difficulties with:SuckingSuckingChewingChewingSwallowingSwallowing

May cause:May cause:DroolingDroolingGaggingGaggingChoking Choking

(McCaffrey, 2008)

Disorders/Syndromes Associated Disorders/Syndromes Associated with Childhood Dysarthriawith Childhood Dysarthria

Neurodevelopmental:Neurodevelopmental:Spina Bifida and HydrocephalusSpina Bifida and Hydrocephalus

Genetic:Genetic:Fragile X syndromeFragile X syndrome

Chromosomal:Chromosomal:DownDown’’s syndromes syndrome

Sporadic:Sporadic:PraderPrader--Willi syndromeWilli syndrome

Neurodevelopmental:Neurodevelopmental:Spina Bifida with Hydrocephalus (SBH)Spina Bifida with Hydrocephalus (SBH)

Spina Bifida: Incomplete fusion of the vertebral column Spina Bifida: Incomplete fusion of the vertebral column Hydrocephalus: Excess of cerebrospinal fluid (CSF) in Hydrocephalus: Excess of cerebrospinal fluid (CSF) in the brain the brain Enlarged skullEnlarged skullSBH: Malformation of the cerebellumSBH: Malformation of the cerebellum

Ataxic dysarthria Ataxic dysarthria

Three clusters of speech:Three clusters of speech:Articulatory inaccuracy Articulatory inaccuracy Prosodic excessProsodic excessPhonatoryPhonatory--prosodic insufficiencyprosodic insufficiency

(Bhatnagar, 2002; Huber(Bhatnagar, 2002; Huber--OkrainecOkrainec, Dennis, Brettschneider, & Spiegler, 2002), Dennis, Brettschneider, & Spiegler, 2002)

Genetic:Genetic:Fragile X SyndromeFragile X Syndrome

Prevalence: 1:4,000Prevalence: 1:4,000Cause: Mutation of FMR1 gene on the X Cause: Mutation of FMR1 gene on the X chromosome chromosome Speech is characterized by:Speech is characterized by:

Dysarthria Dysarthria Dyspraxia Dyspraxia Articulatory distortions and substitutionsArticulatory distortions and substitutions

(Shprintzen, 2000;(Shprintzen, 2000; Turner, Webb, Wake, & Robinson, 1996, as cited in Roberts, Mirreturner, Webb, Wake, & Robinson, 1996, as cited in Roberts, Mirrett, t, Anderson, Burchinal, & Neebe, 2002)Anderson, Burchinal, & Neebe, 2002)

Chromosomal:Chromosomal:DownDown’’s Syndromes Syndrome

Prevalence: 1:1,000Prevalence: 1:1,000Cause: A third 21Cause: A third 21stst chromosome chromosome Trisomy 21 Trisomy 21 Primary feature: Hypotonia Primary feature: Hypotonia Dysarthria Dysarthria

““FloppyFloppy””Speech is characterized by:Speech is characterized by:

Low pitchLow pitchHypernasality Hypernasality Breathiness Breathiness Articulatory distortions Articulatory distortions Increased rate Increased rate Reduced prosody Reduced prosody

(Jung, Gagne, Godden, Leeper, Moon, & Seewalk, 1989; Shprintzen,(Jung, Gagne, Godden, Leeper, Moon, & Seewalk, 1989; Shprintzen, 2000)2000)

Sporadic:Sporadic:PraderPrader--Willi SyndromeWilli Syndrome

Prevalence: 1: 12,000Prevalence: 1: 12,000--15,00015,000Cause: Deletion on chromosome 15q11Cause: Deletion on chromosome 15q11

Long arm of the paternally derived chromosome Long arm of the paternally derived chromosome

Delayed motor development secondary to hypotoniaDelayed motor development secondary to hypotoniaFlaccid dysarthria Flaccid dysarthria

Speech is characterized by:Speech is characterized by:Hypernasality Hypernasality Reduced intelligibility Reduced intelligibility Articulation errors Articulation errors

(Stark, 2006;(Stark, 2006; PraderPrader--Willi Syndrome Association, 2008)Willi Syndrome Association, 2008)

TreatmentTreatmentDepends on type and severity of symptomsDepends on type and severity of symptoms

Children generally receive a better prognosis than Children generally receive a better prognosis than adults due to neural plasticityadults due to neural plasticity

Intervention is not a Intervention is not a ““one size fits allone size fits all”” solutionsolutionBeneficial to the childBeneficial to the childWhat problem does it solve?What problem does it solve?

Evidence basedEvidence basedGrounded in theory?Grounded in theory?

How much treatment is necessary?How much treatment is necessary?

(Caruso & Strand, 1999; Yorkston & Beukelman, 2004)(Caruso & Strand, 1999; Yorkston & Beukelman, 2004)

SeveritySeverity--Based TreatmentBased Treatment

Level of severity and type of dysarthria Level of severity and type of dysarthria influences the course and structure of influences the course and structure of interventioninterventionRemediation of mild to moderate Remediation of mild to moderate dysarthriadysarthria

Compensatory strategiesCompensatory strategiesRemediation of severe dysarthriaRemediation of severe dysarthria

Alternative form of communication necessaryAlternative form of communication necessary

(Caruso & Strand, 1999)(Caruso & Strand, 1999)

Treatment May Target:Treatment May Target:Respiration: Respiration:

To obtain sufficient breath support for speechTo obtain sufficient breath support for speechPhonation:Phonation:

To reduce excessive Breathiness To reduce excessive Breathiness Resonance:Resonance:

To reduce hypernasality To reduce hypernasality Articulation: Articulation:

To coordinate and strengthen the articulatorsTo coordinate and strengthen the articulatorsProsody:Prosody:

To create phonetic contrasts in speechTo create phonetic contrasts in speechLoudness: Loudness:

Feedback, VisiFeedback, Visi--pitch, delayed auditory feedback (DAF)pitch, delayed auditory feedback (DAF)

(Caruso & Strand, 1999)(Caruso & Strand, 1999)

Treatment IdeasTreatment IdeasPromote the inclusion of:Promote the inclusion of:

Family members and caregivers to increase Family members and caregivers to increase generalizationgeneralizationAlternative modes of communicationAlternative modes of communicationEnhancement strategies (e.g., eye contact and facial Enhancement strategies (e.g., eye contact and facial expression)expression)Repair strategies and selfRepair strategies and self--monitoring monitoring Strategies to improve listener comprehension Strategies to improve listener comprehension

Support the development of: Support the development of: Phonological awareness and literacy Phonological awareness and literacy Receptive and expressive language Receptive and expressive language SelfSelf--esteemesteem

(Caruso & Strand, 1999)(Caruso & Strand, 1999)

Future ResearchFuture Research

Childhood DysarthriaChildhood DysarthriaPrevalence: Currently unknownPrevalence: Currently unknown

Masked by primary diagnosisMasked by primary diagnosisDiagnosticians need to include assessment Diagnosticians need to include assessment for dysarthria within the testing battery for dysarthria within the testing battery

InterventionInterventionIdentification of effective treatment methodsIdentification of effective treatment methodsEvidenceEvidence--Based PracticeBased Practice

ReferencesReferencesAndrianopoulos, M. V. (2008). Andrianopoulos, M. V. (2008). Introduction to motor speech disordersIntroduction to motor speech disorders. Retrieved . Retrieved

January 22, 2008, from University of Massachusetts Amherst, January 22, 2008, from University of Massachusetts Amherst, Communication Disorders Website: Communication Disorders Website: http://wwwhttp://www--unix.oit.umass.edu/~mva/pdf/ComDis_624_lecture_1A_08.pdfunix.oit.umass.edu/~mva/pdf/ComDis_624_lecture_1A_08.pdf

Bhatnagar, S. C. (2002). Bhatnagar, S. C. (2002). Neuroscience for the study of communicative disordersNeuroscience for the study of communicative disorders. . (2nd ed.). Philadelphia, PA: Lippincott Williams & Wilkins.(2nd ed.). Philadelphia, PA: Lippincott Williams & Wilkins.

Caruso, A. J. & Strand, E. A. (1999). Caruso, A. J. & Strand, E. A. (1999). Clinical management of motor speech Clinical management of motor speech disorders in children.disorders in children. New York: ThiemeNew York: Thieme

Duffy, J. R. (2005) Duffy, J. R. (2005) Motor speech disorders: Substrates, differential diagnosis, and Motor speech disorders: Substrates, differential diagnosis, and managementmanagement. St. Louis, MO: Elsevier Mosby.. St. Louis, MO: Elsevier Mosby.

HuberHuber--Okrainec, J., Dennis, M., Brettschneider, J. & Spiegler, B. J. (Okrainec, J., Dennis, M., Brettschneider, J. & Spiegler, B. J. (2002). 2002). Neuromotor speech deficits in children and adults with spina bifNeuromotor speech deficits in children and adults with spina bifida and ida and hydrocephalus. hydrocephalus. Brain and LanguageBrain and Language, 80(3), 592, 80(3), 592--602. 602.

Jung, J. H., Gagne, J. P., Godden, A. L., Leeper, H. A., Moon, JJung, J. H., Gagne, J. P., Godden, A. L., Leeper, H. A., Moon, J. B., & Seewald, R. . B., & Seewald, R. C. (1989). C. (1989). Genetic syndromes in communication disordersGenetic syndromes in communication disorders. Austin, TX: PRO. Austin, TX: PRO--ED.ED.

McCaffrey, P. (2008). McCaffrey, P. (2008). Neuropathologies of swallowing and speechNeuropathologies of swallowing and speech. Retrieved April . Retrieved April 29, 2008, from California State University, Chico, The Neuroscie29, 2008, from California State University, Chico, The Neuroscience on the nce on the Web Series: Web Series: http://www.csuchico.edu/~pmccaffrey/syllabi/SPPA342/342unit14.hthttp://www.csuchico.edu/~pmccaffrey/syllabi/SPPA342/342unit14.htmlml

References References –– ContinuedContinued……PraderPrader--Will Syndrome Association. (2008, February 21). Will Syndrome Association. (2008, February 21). Basic facts about PWS: A Basic facts about PWS: A

diagnosis and reference guide for physicians and other health prdiagnosis and reference guide for physicians and other health professionalsofessionals. . Retrieved April 29, 2008, from the World Wide Web: Retrieved April 29, 2008, from the World Wide Web: http://www.pwsausa.org/syndrome/basicfac.htmhttp://www.pwsausa.org/syndrome/basicfac.htm

Roberts, J. E., Mirrett, P., Anderson, K., Burchinal, M., & NeebRoberts, J. E., Mirrett, P., Anderson, K., Burchinal, M., & Neebe, E. (2002). Early e, E. (2002). Early communication, symbolic behavior, and social profiles of young mcommunication, symbolic behavior, and social profiles of young males with ales with fragile X syndrome. fragile X syndrome. American Journal of SpeechAmerican Journal of Speech--Language PathologyLanguage Pathology, 11, 295, 11, 295--304.304.

Shprintzen, R. J. (2000). Shprintzen, R. J. (2000). Syndrome identification for speechSyndrome identification for speech--language pathology: An language pathology: An illustrated pocketguideillustrated pocketguide. San Diego, CA: Singular. . San Diego, CA: Singular.

Stark, S. (2006). Stark, S. (2006). Neurodevelopmental disorders with genetic etiologies and Neurodevelopmental disorders with genetic etiologies and speech and language disorders.speech and language disorders.

van Mourik, M., Catsmanvan Mourik, M., Catsman--Berrevoets, C. E., Paquier, P. F., YousefBerrevoets, C. E., Paquier, P. F., Yousef--Bak, E., & van Bak, E., & van Dongen, H. R. (1997). Acquired childhood dysarthria: Review of iDongen, H. R. (1997). Acquired childhood dysarthria: Review of its clinical ts clinical presentation. presentation. Pediatric NeurologyPediatric Neurology, 17(4), 299, 17(4), 299--307.307.

Yorkston, K. M., & Beukelman, D. R. (2004, May 11). Dysarthria: Yorkston, K. M., & Beukelman, D. R. (2004, May 11). Dysarthria: Tools for clinicalTools for clinicaldecisiondecision--making. making. The ASHA LeaderThe ASHA Leader, pp. 4, pp. 4--5, 205, 20--21.21.