Speech_and_Language_Therapy_Interventions_in_Ataxia8dc5.pptx

Ppt

1. Speech and Language Therapy Interventions in Ataxia Prof Anja Lowit Strathclyde University Glasgow

2. Structure What problems do people with ataxia experience? What is the evidence base for effective treatment? Where do we go from here?

3. Auditory-Perceptual Difficulties Rance et al. (2010 ): presence and consequences of auditory neuropathy (AN) in FA individuals Basic auditory processing was affected in each FA individual showing poorer temporal processing and discrimination than their matched control. Speech perception in the presence of background noise was also impaired, with F A listeners typically able to access only around 50% of the information available to their normal peers. Could potentially affect self-monitoring of speakers as well as their language comprehension in some situations

4. Cognitive Deficits Timmann et al. (2002 ): Cerebellar patients showed a learning deficit, compared to the normal controls Deficit was independent of the motor difficulty of the task => The cerebellum seems to contribute to motor-independent processes, which are generally involved in associative learning

5. Language/Cognitive Difficulties: Wollmann , et al. (2002 ): A ssessed major cognitive areas in patients with FA and matched normal controls. Neuropsychological assessment showed decreased motor and mental reaction times , reduced verbal span, deficits in letter fluency , impaired acquisition and consolidation of verbal information ,

6. Swallowing Difficulties Vogel et al. ( 2014): Aim : characterise dysphagia in FA and determine the impact of swallowing dysfunction on activities, participation, and sense of well-being. 92% of participants exhibited clinical signs of dysphagia impairment related to incoordination & weakness relationship between severity of impairment and distress/well-being, suggesting that swallowing function decreases in line with overall quality of life. No relationship between dysphagia severity, age of onset or disease severity. Early detection of swallowing impairment and consideration of the potential impact on quality of life should be key aspects in disease management.

7. Speech Problems in Ataxia Articulatory inaccuracy Phonatory-prosodic insufficiency Prosodic excess Imprecise consonants Distorted vowels Irregular articulatory breakdowns Harsh Voice Monopitch Monoloudness Disturbances of pitch (flat F0) Inability to sustain vowels Reduced stress contrasts (excess and equal stress) Sudden burst-like increases of loudness Prolonged phonemes Prolonged intervals Slow rate Based on Darley FL, Aronson AE, and Brown JR: Differential diagnostic patterns of dysarthria, J Speech Hear Res 12:246, 1969

8. A closer look at speech problems

9. Evidence that symptom profiles differ across different severities and types of ataxic dysarthria (in SCA and FA) ( Blaney & Hewlett (2007a ), Folker et al. (2010, 2012), Sidtis et al. (2011 )), for example: Folker et al. ( 2010) found 3 subgroups of FA speakers, the first presenting with mild dysarthric symptoms, the second with increased velopharyngeal involvement and the third characterized by increased laryngeal dysfunction. Dysarthria severity showed a significant correlation to disease duration but to no other clinical measure . Sidtis et al. ( 2011) found that SCA1, 5 & 6 groups shared articulatory problems but differed with respect to abnormal voice features

10. Some more in-depth investigations:

11. Articulation Blaney & Hewlett ( 2007a & b): Mild to severe speakers with FA Highest source of error were consonants at the end of words Problems with vowel length accounted for about 60% of the reduction in intelligibility scores

12. Intonation: Lowit & Kuschmann ( 2012) / Lowit et al. (2014 ): AT speakers had the shortest phrase length Produced a higher number of stresses per phrase Had problems de-emphasising unimportant words in a sentence Used more rising tones than CON speakers

13. Stress: Poor contrastive stress production

14. Rhythm Perception: Equalised stress / Scanning speech Acoustics: Different patterns

15. Treatment of Speech Problems

16. Fonteyn, E. M. R., et al. (2014). "The effectiveness of allied health care in patients with ataxia: a systematic review." Journal of Neurology 261 (2): 251-258. The combined data indicate that physical therapy may lead to an improvement of ataxia symptoms and daily life functions in patients with degenerative cerebellar ataxia (level 2), and in other diseases causing cerebellar ataxia (level 3). When added to physical therapy, occupational therapy might improve global functional status, and occupational therapy alone may diminish symptoms of depression (level 3). There are insufficient data for speech and language therapy .

17. Cochrane Review on Treatment for speech disorder in Friedreich ataxia and other hereditary ataxia syndromes (Vogel et al. 2014) 14 clinical trials reviewed, 13 involved pharmacological treatment, 1 physical and OT treatment, none SLT “There is insufficient and low or very low quality evidence from either RCTs or observational studies to determine the effectiveness of any treatment for speech disorder in any of the hereditary ataxia syndromes.” (p.1)

18. Cochrane Review on Treatment for swallowing difficulties in hereditary ataxia(Vogel et al. 2015) We found no RCTs or quasi-RCTs of treatment for swallowing difficulties in hereditary ataxia.

19. So what do we do?

20. Detailed assessment is important: Find out the exact presentation of symptoms of the speaker Then use techniques from general dysarthria treatment regimes to address these Multi-disciplinary management together with PT and OT Suggestion that severity/type of gross-motor impairments are related to speech performance ( Chiu et al ., 1996 ) , so joint management might be beneficial for speech

21. Compensation: Ataxic speakers were able to compensate for intonation/stress production deficits, e.g. although some speakers were unable to de-accent, they could mark stress appropriately through use of other phonetic parameters (Lowit et al. 2012) Ability to compensate furthermore demonstrated by Sidtis et al. (2010) in a PET study (intelligibility was maintained while rhythm degraded)

22. Ongoing feasibility study looking into the use of LSVT with people with FA Sapir, S., et al. (2003). "Effects of intensive voice treatment (the Lee Silverman Voice Treatment [LSVT]) on ataxic dysarthria: a case study." American Journal of Speech-Language Pathology 12 (4): 387-399. Study examined the effects of intensive voice treatment (the Lee Silverman Voice Treatment [LSVT]) on ataxic dysarthria in a woman with cerebellar dysfunction secondary to thiamine deficiency. Results indicate short- and long-term improvement in voice and articulation, speech intelligibility, and overall communication and job-related activity following LSVT.

23. Our study 14 people recruited so far 6 have completed treatment Study involves: 2 assessments BEFORE therapy 8 weeks of treatment (2 x45 min sessions per week plus home practice) 2 assessments AFTER therapy Looks at speech performance as well as fatigue and psycho-social measures Treatment and assessment done over Skype in participant’s own home

24. Outcomes so far: Participants can hold an “ah” for longer = better breath support for speech Voice has become stronger and more stable Articulation is clearer Speak more slowly Can speak for longer Need to repeat less often Feel more confident about their communication and participate more in activities Results maintained after 6 weeks of no therapy