Dysarthria Profiles in Adults With Hereditary Ataxia

2019 ◽  
Vol 28 (2S) ◽  
pp. 915-924 ◽  
Author(s):  
Kristie A. Spencer ◽  
Mallory Dawson

Purpose This preliminary study examined whether speech profiles exist for adults with hereditary ataxia based on 2 competing frameworks: a pattern of instability/inflexibility or a pattern of differential subsystem involvement. Method Four dysarthria experts rated the speech samples of 8 adults with dysarthria from hereditary ataxia using visual analog scales and presence/severity rating scales of speech characteristics. Speaking tasks included diadochokinetics, sustained phonation, and a monologue. Results Speech profiles aligned with the instability/inflexibility framework, with the pattern of instability being the most common. Speech profiles did not emerge for the majority of speakers using the differential subsystem framework. Conclusions The findings extend previous research on pure ataxic dysarthria and suggest a possible framework for understanding the speech heterogeneity associated with the ataxias. The predominance of the instability profile is consistent with the notion of impaired feedforward control in speakers with cerebellar disruption.

Author(s):  
Ertan Kucuksayan ◽  
Serkan Ozben ◽  
Selma Topaloglu Tuac ◽  
Mesrure Koseoglu ◽  
Ozcan Erel ◽  
...  

Abstract Objectives Restless legs syndrome (RLS) is a common neurological condition. Oxidative stress plays an important role in its pathogenesis. Thiol-disulphide homeostasis (TDH) is a new biomarker of oxidative stress. We studied plasma TDH to determine whether TDH could be used as a new biomarker for RLS and evaluated correlations between TDH and various disease severity rating scales. Methods A total of 25 RLS patients and 25 healthy controls were included into the study. TDH status was determined using an automated spectrophotometric analysis method and correlations were analyzed between the TDH status and various disease rating scales in the RLS patients. Results Plasma total (401 ± 27 μmol/L) and native thiol (354 ± 30 μmol/L) levels were significantly lower, but disulphide level (24 ± 6 μmol/L) was significantly (<0.0001) higher in the RLS patients compared to the controls (455 ± 36, 424 ± 37, 15 ± 5 μmol/L, respectively). The disulphide/native thiol and disulphide/total thiol ratios increased, in contrast, native thiol/total thiol ratio decreased significantly in the RLS patients compared to the healthy controls (<0.0001). The disulphide levels correlated positively with age and various rating scores of the RLS patients. International Restless Legs Syndrome Study Group (IRLSSG) rating score and age correlated negatively with the total and native thiol levels. Conclusions Our findings indicate increased oxidative stress in the RLS patients reflected by decreased native and total thiol, and increased disulphide levels and positive correlations between the disulphide levels and various rating scores. We suggest dynamic TDH status to be used as a novel biomarker for the diagnosis and follow-up of the RLS patients.


2014 ◽  
Vol 108 (2) ◽  
pp. 151-156
Author(s):  
Robert Wall Emerson ◽  
Dawn Anderson

2000 ◽  
Vol 52 (5) ◽  
pp. 228-238 ◽  
Author(s):  
Lena Hartelius ◽  
Björn Runmarker ◽  
Oluf Andersen ◽  
Lennart Nord

Author(s):  
Divya Seth ◽  
Santosh Maruthy

This chapter aims to investigate and evaluate the evidence available for stuttering treatment research in children who stutter (CWS) and provide a qualitative review of the same. Studies were considered for review if (1) they were behavioral intervention of stuttering for children, (2) participants were in the age range of 2-18 years, and (3) the outcomes reported assessed stuttering. Twenty-seven studies were obtained through an electronic search of databases covering two approaches and 10 techniques. The analysis of these studies revealed that direct approaches have a stronger evidence base in comparison to the indirect approaches. The most popular approach with maximum studies is the early intervention Lidcombe program. The commonly used outcome measures included the percentage of syllables stuttered, syllables per minute, and severity rating scales. The conclusion drawn from the review suggest that evidence base to support behavioral intervention of CWS exists for a limited number of intervention techniques and among them very few exploring the long-term effects of the intervention.


2017 ◽  
Vol 27 (3) ◽  
pp. 219-224 ◽  
Author(s):  
J. W. Keeley ◽  
W. Gaebel

The subtype system for categorising presentations of schizophrenia will be removed from International Classification of Diseases 11th Revision. In its place will be a system for rating six domains of psychotic disorder pathology: positive symptoms, negative symptoms, depressive symptoms, manic symptoms, psychomotor symptoms and cognitive symptoms. This paper outlines the rationale and description of the proposed symptom rating scale, including current controversies. In particular, the scale could adopt either a 4-point severity rating or a 2-point presence/absence rating. The 4-point scale has the advantage of gathering more information, but potentially at the cost of reliability. The paper concludes by describing the field testing process for evaluating the proposed scale.


2014 ◽  
Vol 57 (4) ◽  
pp. 1284-1295 ◽  
Author(s):  
Hamid Karimi ◽  
Sue O’Brian ◽  
Mark Onslow ◽  
Mark Jones

PurposePercentage of syllables stuttered (%SS) and severity rating (SR) scales are measures in common use to quantify stuttering severity and its changes during basic and clinical research conditions. However, their reliability has not been assessed with indices measuring both relative and absolute reliability. This study was designed to provide such information. Relative reliability deals with the rank order of participants in a sample, whereas absolute reliability measures the closeness of scores to one other and to a hypothetical true score.MethodEighty-seven adult participants who stutter received a 10-min unscheduled telephone call. Three experienced judges measured %SS and also used a 9-point SR scale to measure stuttering severity from recordings of the telephone calls.ResultsRelative intrajudge and interjudge reliability were satisfactory for both scales. However, absolute intrajudge and interjudge reliability were not satisfactory. Results showed that paired-judge SR and %SS procedures improved absolute reliability compared with single-judge measures. Additionally, the paired-judge procedure improved relative reliability from high to very high levels.ConclusionMeasurement of group changes of stuttering severity can be done in research contexts using either %SS or SR. However, for detecting changes within individuals using such measures, a paired-judge procedure is a more reliable method.


1982 ◽  
Vol 13 (3) ◽  
pp. 156-162 ◽  
Author(s):  
Kenneth D. Barker ◽  
Robert A. Baldes ◽  
Phillip H. Jenkinson ◽  
Kenyon D. Wilson ◽  
J. Joseph Freilinger

PL 94-142 mandates that all pupils who are handicapped receive an appropriate program in the least restrictive environment. In implementing both federal and state laws, it was necessary to depart from the traditional methods of service delivery, and so a continuum of services concept was adopted. Attached to the continuum of services are severity rating scales that indicate milestones along the continuum. A severity rating scale was developed for articulation, language, fluency, and voice. Although providing direction for the speech-language pathologist, clinical judgment was not compromised. The severity rating scale assists the clinician in case selection and suggests the intensity of the service delivery model. Data obtained from use of the scale can be employed to appropriately assign staff. Each scale is reproduced in an appendix to this article.


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