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Motor and Speech Disorders in Classic Galactosemia

  • Nancy L. PotterEmail author
  • Yves Nievergelt
  • Lawrence D. Shriberg
Research Report
Part of the JIMD Reports book series (JIMD, volume 11)

Abstract

Purpose To test the hypothesis that children with classic galactosemia and speech disorders are at risk for co-occurring strength and coordination disorders.

Method This is a case–control study of 32 children (66% male) with galactosemia and neurologic speech disorders and 130 controls (50% male) ages 4–16 years. Speech was assessed using the Percentage of Consonants Correct (PCC) metric from responses to the Goldman-Fristoe Test of Articulation-2 and from a 5-min recorded speech sample, hand and tongue strength using the Iowa Oral Performance Instrument, and coordination using the Movement Assessment Battery for Children. The number of days on milk during the neonatal period was obtained by parent report. Analyses of covariance, distributions, and correlations were used to evaluate relationships among speech, strength, coordination, age, gender, and days on milk.

Results Children with galactosemia had weaker hand and tongue strength and most (66%) had significant coordination disorders, primarily affecting balance and manual dexterity. Among children with galactosemia, children with more speech errors and classified as childhood apraxia of speech (n = 7) and ataxic dysarthria (n = 1), had poorer balance and manual dexterity, but not weaker hand or tongue strength, compared to the children with fewer speech errors. The number of days on milk during the neonatal period was associated with more speech errors in males but not in females.

Conclusion Children with galactosemia have a high prevalence of co-occurring speech, coordination, and strength disorders, which may be evidence of a common underlying etiology, likely associated with diffuse cerebellar damage, rather than distinct disorders.

Keywords

Manual Dexterity Speech Disorder Nondominant Hand Tongue Strength Speech Error 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Abbreviations

CAS

Childhood Apraxia of Speech

kstest

Kolmogorov-Smirnov one sample one-tail test statistic

kstest2

Kolmogorov-Smirnov two sample one-tail test statistic

MABC

Movement Assessment Battery for Children

MSD-NOS

Motor Speech Disorder-Not Otherwise Specified

PCC:AT

Percentage of Consonants Correct from an Articulation Test

PCC:CS

Percentage of Consonants Correct from a 5-minute Conversational Speech Sample

Notes

Acknowledgments

This research was supported by National Institute on Deafness and Other Communication Disorders Grant DC000496 and by a core grant to the Waisman Center from the National Institute of Child Health and Development (Grant HD03352). We thank the following colleagues for their contributions to this study: Heather Karlsson, Heather Lohmeier, Jane McSweeny, Leslie Power, Lola Rickey, Sue Siemsen, Christie Tilkens, The Galactosemia Foundation, Galactosemic Families of Minnesota, and the children and parents who participated in this study.

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Copyright information

© SSIEM and Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Nancy L. Potter
    • 1
    Email author
  • Yves Nievergelt
    • 2
  • Lawrence D. Shriberg
    • 3
  1. 1.Department of Speech and Hearing SciencesWashington State University SpokaneSpokaneUSA
  2. 2.Department of MathematicsEastern Washington UniversityCheneyUSA
  3. 3.Waisman CenterUniversity of Wisconsin-MadisonMadisonUSA

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