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Richland Center, WI, United States

Shriberg L.D.,Waisman Center | Potter N.L.,Washington State University | Strand E.A.,Mayo Medical School
Journal of Speech, Language, and Hearing Research | Year: 2011

Purpose: In this article, the authors address the hypothesis that the severe and persistent speech disorder reported in persons with galactosemia meets contemporary diagnostic criteria for Childhood Apraxia of Speech (CAS). A positive finding for CAS in this rare metabolic disorder has the potential to impact treatment of persons with galactosemia and inform explanatory perspectives on CAS in neurological, neurodevelopmental, and idiopathic contexts. Method: Thirty-three youth with galactosemia and significant prior or persistent speech sound disorder were assessed in their homes in 17 states. Participants completed a protocol yielding information on their cognitive, structural, sensorimotor, language, speech, prosody, and voice status and function. Results: Eight of the 33 participants (24%) met contemporary diagnostic criteria for CAS. Two participants, 1 of whom was among the 8 with CAS, met criteria for ataxic or hyperkinetic dysarthria. Groupwise findings for the remaining 24 participants are consistent with a classification category termed Motor Speech Disorder-Not Otherwise Specified (Shriberg, Fourakis et al., 2010a). Conclusion: The authors estimate the prevalence of CAS in galactosemia at 18 per hundred-180 times the estimated risk for idiopathic CAS. Findings support the need to study risk factors for the high occurrence of motor speech disorders in galactosemia despite early compliant dietary management. © American Speech-Language-Hearing Association. Source

van Calcar S.C.,University of Wisconsin - Milwaukee | van Calcar S.C.,Waisman Center | Ney D.M.,University of Wisconsin - Madison
Journal of the Academy of Nutrition and Dietetics | Year: 2012

Phenylketonuria (PKU), an inborn error in phenylalanine metabolism, requires lifelong nutrition management with a low-phenylalanine diet, which includes a phenylalanine-free amino acid-based medical formula to provide the majority of an individual's protein needs. Compliance with this diet is often difficult for older children, adolescents, and adults with PKU. The whey protein glycomacropeptide (GMP) is ideally suited for the PKU diet because it is naturally low in phenylalanine. Nutritionally complete, acceptable medical foods and beverages can be made with GMP to increase the variety of protein sources for the PKU diet. As an intact protein, GMP improves protein use and increases satiety compared with amino acids. Thus, GMP provides a new, more physiologic source of low-phenylalanine dietary protein for people with PKU. © 2012 Academy of Nutrition and Dietetics. Source

Floyd F.J.,University of Hawaii at Manoa | Seltzer M.M.,Waisman Center | Greenberg J.S.,Waisman Center | Song J.,University of Wisconsin - Madison
Psychology and Aging | Year: 2013

The death of a child when parents are in mid-to-late life is a traumatic event for aging parents. In order to evaluate adjustment, the impact of unanticipated versus anticipated deaths, and the effects of internal resources for coping with bereavement, we examined pre-and postbereavement functioning, using the 1992/94 and 2004/06 waves of the Wisconsin Longitudinal Study, for parents (M age = 54 and 65 years, respectively) whose adult child died between these dates (n = 175). The results revealed a general pattern of adaptation in which most bereaved parents were functioning as well as a matched comparison group (n = 175), though more depression symptoms were present both before and after the death of the child for the mothers of children who died from long-term illnesses and the fathers of children who committed suicide, suggesting that conditions predating the death were chronic strains for these parents. Intrapersonal resources, including a sense of purpose in life and high levels of agreeableness, were associated with better functioning, particularly for bereaved parents whose children's deaths were not anticipated. The study places parental bereavement in the context of normative aging and the framework of chronic life strain. © 2013 American Psychological Association. Source

Cho W.,Waisman Center | Brenner M.,University of Alabama at Birmingham | Peters N.,University of Wisconsin - Madison | Messing A.,Waisman Center
Human Molecular Genetics | Year: 2010

Glial fibrillary acidic protein (GFAP) is the major intermediate filament protein of astrocytes in the vertebrate central nervous system. Increased levels of GFAP are the hallmark feature of gliosis, a non-specific response of astrocytes to a wide variety of injuries and disorders of the CNS, and also occur in Alexander disease where the initial insult is a mutation within the coding region of GFAP itself. In both settings, excess GFAP may cause or exacerbate astrocyte dysfunction. With the goal of finding drugs that reduce the expression of GFAP, we have devised screens to detect changes in GFAP promoter activity or protein levels in primary cultures of mouse astrocytes in a 96-well format. We have applied these screens to libraries enriched in compounds that are already approved for human use by the FDA. We report that several compounds are active at micromolar levels in suppressing the expression of GFAP. Treatment of mice for 3 weeks with one of these drugs, clomipramine, causes nearly 50% reduction in the levels of GFAP protein in brain. © The Author 2010. Published by Oxford University Press. All rights reserved. Source

Grieco-Calub T.M.,Waisman Center | Litovsky R.Y.,Waisman Center | Litovsky R.Y.,University of Wisconsin - Madison
Ear and Hearing | Year: 2010

Objectives: To measure sound source localization in children who have sequential bilateral cochlear implants (BICIs); to determine whether localization accuracy correlates with performance on a right-left discrimination task (i.e., spatial acuity); to determine whether there is a measurable bilateral benefit on a sound source identification task (i.e., localization accuracy) by comparing performance under bilateral and unilateral listening conditions; and to determine whether sound source localization continues to improve with longer durations of bilateral experience. Design: Two groups of children participated in this study: a group of 21 children who received BICIs in sequential procedures (5 to 14 years) and a group of 7 typically developing children with normal acoustic hearing (5 years). Testing was conducted in a large sound-treated booth with loudspeakers positioned on a horizontal arc with a radius of 1.2 m. Children participated in two experiments that assessed spatial hearing skills. Spatial hearing acuity was assessed with a discrimination task in which listeners determined whether a sound source was presented on the right or left side of center; the smallest angle at which performance on this task was reliably above chance is the minimum audible angle. Sound localization accuracy was assessed with a sound source identification task in which children identified the perceived position of the sound source from a multiloudspeaker array (7 or 15); errors are quantified using the root mean square (RMS) error. Results: Sound localization accuracy was highly variable among the children with BICIs, with RMS errors ranging from 19 to 56°. Performance of the normal hearing group, with RMS errors ranging from 9 to 29° was significantly better. Within the BICI group, in 11 of 21 children, RMS errors were smaller in the bilateral versus unilateral listening condition, indicating bilateral benefit. There was a significant correlation between spatial acuity and sound localization accuracy (R = 0.68, p < 0.01), suggesting that children who achieve small RMS errors tend to have the smallest minimum audible angles. Although there was large intersubject variability, testing of 11 children in the BICI group at two sequential visits revealed a subset of children who show improvement in spatial hearing skills over time. Conclusions: A subset of children who use sequential BICIs can acquire sound localization abilities, even after long intervals between activation of hearing in the first- and second-implanted ears. This suggests that children with activation of the second implant later in life may be capable of developing spatial hearing abilities. The large variability in performance among the children with BICIs suggests that maturation of sound localization abilities in children with BICIs may be dependent on various individual subject factors such as age of implantation and chronological age. Copyright © 2010 by Lippincott Williams & Wilkins. Source

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