Speech, voice, language and cognition in individuals with spinocerebellar ataxia (SCA)
Author: Schalling, Ellika
Date: 2007-06-15
Location: Föreläsningssalen Månen, Alfred Nobels Allé 8, Karolinska Universitetssjukhuset, Huddinge
Time: 10.00
Department: Institutionen för klinisk vetenskap / Department of Clinical Sciences
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thesis.pdf (1.689Mb)
Abstract
Spinocerebellar ataxias (SCA) constitute a group of genetically defined
hereditary, degenerative, progressive diseases affecting the cerebellum
and its connections. Few previous investigations have focused on how SCA
affects different aspects of communication. The aim of the present
investigation was to characterize speech and voice in individuals with
SCA and to investigate the progression of speech and voice symptoms,
using both perceptual and acoustic methodology. In addition, language and
cognition in individuals with SCA were studied.
Thirty-two individuals with spinocerebellar degenerative disease
participated in the studies. The majority had been diagnosed with SCA
using molecular genetic testing and the rest were clinically diagnosed by
a specialist in neurology. Matched control subjects were included in
study II and III.
Speech and voice in individuals with SCA were assessed perceptually by a
group of four speech-language pathologists with long experience of
neurogenic communication disorders. Recorded speech samples from
individuals with SCA were rated using visual analogue scales, VAS. Speech
samples were also used for computer-based acoustic analysis. Speech and
voice were characterized by the following perceptual parameters:
Equalized stress, imprecise consonants, vocal instability, monotony,
strained-strangled voice, stereotypic intonation and reduced speech rate.
Factor analysis resulted in two main factors; one associated with
temporal aspects of speech and the other with vocal quality. Acoustic
analysis confirmed the perceptual findings. Rate of speech and sequential
and alternating motion rates were reduced, and duration and variability
of syllables and pauses during rapid syllable repetition were increased
compared to matched control subjects. Inter-stress intervals (ISIs) were
also longer and more variable in subjects with SCA compared to control
subjects. Perceived vocal instability was confirmed acoustically by
increased coefficient of variation of fundamental frequency, CV of F0,
during sustained phonation. This was also found in individuals with SCA
with otherwise close to normal speech.
Speech and voice were followed in nine individuals with SCA during three
years and speech samples were analyzed both perceptually and
acoustically. Perceived imprecision of consonants and stereotypic
intonation had increased during the three years. Some acoustic measures
had also changed, e g duration of syllables in rapid syllable repetition
and duration of inter-stress intervals. In addition, there was a trend
towards change in several other perceptual and acoustic measures,
especially measures related to temporal aspects. Changes were more
substantial in individuals with early disease onset, regardless of
disease duration. An increase of mean dysarthria scores was also found.
Language and cognition were assessed in 20 individuals with SCA and
control subjects matched for age, gender, length of formal education and
estimated cognitive level. Executive functions and attention were most
severely affected, but memory and lexicosemantic knowledge were also
impaired, especially in individuals with more severely impaired estimated
global cognitive level of functioning. Cognitive impairment correlated
with low age at disease onset and also with impairment of motor speech
function, but not with disease duration.
It was concluded that dysarthria in SCA resembles previous descriptions
of ataxic dysarthria, but also includes an element of strained-strangled
voice. Equalized stress was more prominent and imprecision of vowels was
less common compared to previous studies. Vocal instability may be an
early sign of the disease. Progression of symptoms can be seen over a
three-year period, especially as increased perceived imprecision of
consonants and stereotypic intonation, but also measured with a clinical
dysarthria test. Cognition was impaired in individuals with SCA,
especially executive functions and attention. Assessment by
speech-language pathologists should include testing of cognition and
language as it may have implications for treatment. Keywords:
Spinocerebellar ataxia, ataxic dysarthria, perceptual analysis, acoustic
analysis, cerebellar degenerative disorders, cognitive impairment,
language impairment, executive dysfunction, progression of neurological
disease.
List of papers:
I. Schalling E, Hartelius L (2004). "Acoustic analysis of speech tasks performed by three individuals with spinocerebellar ataxia." Folia Phoniatr Logop 56(6): 367-80
Pubmed
II. Schalling E, Hammarberg B, Hartelius L (2007). "Perceptual and acoustic analysis of speech in individuals with spinocerebellar ataxia (SCA)." Logoped Phoniatr Vocol 32(1): 31-46
Pubmed
III. Schalling E, Tallberg IM (2007). "Executive dysfunction dominates cognitive impairment in spinocerebellar ataxia (SCA)." (Submitted)
IV. Schalling E, Hammarberg B, Hartelius L (2007). "A longitudinal study of speech and voice in spinocerebellar ataxia - acoustic and perceptual analysis." (Submitted)
I. Schalling E, Hartelius L (2004). "Acoustic analysis of speech tasks performed by three individuals with spinocerebellar ataxia." Folia Phoniatr Logop 56(6): 367-80
Pubmed
II. Schalling E, Hammarberg B, Hartelius L (2007). "Perceptual and acoustic analysis of speech in individuals with spinocerebellar ataxia (SCA)." Logoped Phoniatr Vocol 32(1): 31-46
Pubmed
III. Schalling E, Tallberg IM (2007). "Executive dysfunction dominates cognitive impairment in spinocerebellar ataxia (SCA)." (Submitted)
IV. Schalling E, Hammarberg B, Hartelius L (2007). "A longitudinal study of speech and voice in spinocerebellar ataxia - acoustic and perceptual analysis." (Submitted)
Issue date: 2007-05-25
Rights:
Publication year: 2007
ISBN: 978-91-7357-221-7
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