Summary of the Clinical Practice Guideline

Article Citation

Management of the Ataxias Towards Best Clinical Practice

Bonney, H., de Silva, R., et al. (2016).
London (United Kingdom): Ataxia UK, (3rd Edition), 1-87.
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Article Details

Description

This guideline provides recommendations for the assessment and treatment of individuals with progressive ataxia. This is an update of a previous guideline published by de Silva et al. in 2009. The target audience of this guideline is healthcare professionals involved in the management of individuals with progressive ataxia. Of particular interest to clinicians is the section for allied healthcare professionals.

Evidence Ratings for This Document

Recommendations were provided as Grade A, B, C, D, and Good Practice based on the strength and the quality of the supporting evidence using the following criteria:
  • Grade A Evidence: The overall body of evidence can be trusted to guide practice. Evidence is based on one or more level I studies or several level II studies that are directly applicable to the target population and demonstrate an overall consistency of results.
  • Grade B Evidence: The body of evidence can be trusted to guide practice in most situations. Evidence is based on one or two studies rated as level II or several level III studies that are directly applicable to the target population and demonstrate an overall consistency of results.
  • Grade C Evidence: The body of evidence provides some support for the recommendation; however care should be taken in its application. Evidence is based on level III-c studies or level I or II studies with a moderate risk of bias and some inconsistency and applicability to the target population. 
  • Grade D Evidence: The body of evidence is weak; therefore recommendations must be applied with caution. Evidence is based on level IV studies or level 1-IV studies with high risk of bias and inconsistent results that are not applicable to the target population. 
  • Good Practice Point: Recommendation is based on clinical experience or expert opinion.
Classes of evidence were defined as follows:
  • Class I Evidence: Evidence based on a systematic review of relevant randomized controlled trials.
  • Class II Evidence: Evidence from at least one randomized controlled trial.
  • Class III-a Evidence: Evidence from one or more controlled trials or pseudo-randomized trials.
  • Class III-b Evidence: Evidence from prospective or retrospective cohort studies with concurrent controls, case-control studies, or interrupted time-series studies with a control group.
  • Class III-c Evidence: Evidence from cohort studies with historical controls, two or more single-arm studies, or interrupted time-series studies without a parallel control group.
  • Class IV Evidence: Evidence from clinical experience opinions, descriptive studies, or reports by clinical bodies or committees. 

Recommendations from This Guideline

What are Recommendations?

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Assessment

Individuals with progressive ataxia and suspected hearing loss should be referred to an audiologist for a battery of hearing tests (Good Practice Point). "In specific cases (e.g., ANSD) a referral to a neuro-otologist should be considered" (Good Practice Point; p. 37).

Keywords: Comorbid Diseases and Disorders, Provider, Provider

Treatment

"A trial with an FM hearing device is recommended in cases of ataxia with [auditory neuropathy spectrum disorder] ANSD" (Grade C Evidence; p. 37).

Keywords: Hearing Assistive Technology Systems (HATS)

Service Delivery

Individuals with progressive ataxia and suspected hearing loss should be referred to an audiologist for a battery of hearing tests (Good Practice Point). "In specific cases (e.g., ANSD) a referral to a neuro-otologist should be considered" (Good Practice Point; p. 37).

Keywords: Comorbid Diseases and Disorders, Provider, Provider

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Treatment

Individuals with progressive ataxia and dysarthria should be considered for alternative and augmentative communication when speech intelligibility levels fall below 50% or when reduced intelligibility has a significant impact on functional communication (Grade Level D).

Keywords: Diagnosis/Condition, Augmentative and Alternative Communication (AAC), Progressive Ataxia, Dysarthria

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Assessment

The speech-language therapist should complete a comprehensive communication assessment for individuals with dysarthria due to progressive ataxia. Assessment should consider the impact of communication difficulties on the individual's activities of daily living and life roles (Good Practice Point). 

Keywords: Progressive Ataxia

Treatment

Individuals with progressive ataxia and dysarthria should be considered for alternative and augmentative communication when speech intelligibility levels fall below 50% or when reduced intelligibility has a significant impact on functional communication (Grade Level D).

Keywords: Diagnosis/Condition, Augmentative and Alternative Communication (AAC), Progressive Ataxia, Dysarthria

In the absence of evidence-based guidance on the most effective dysarthria treatment for individuals with progressive ataxia, the speech and language therapist should develop an individualized treatment program based on the findings of a comprehensive assessment. The clinician should be "vigilant for any signs of cognitive and/or hearing difficulties in patients with ataxia that might impact communication and the management strategy should be modified accordingly" (Good Practice Point; p. 54).

Keywords: Progressive Ataxia

Service Delivery

If individuals with progressive ataxia "experience specific difficulties with either their communication and/or swallowing [including sialorrhea] a referral to a [speech-language therapist] SLT is recommended. An open referral system should be in place where patients are able to access help from a SLT as and when required (Good Practice Point; p. 54).

Keywords: Provider (SLP/Caregiver), Provider, Progressive Ataxia, Progressive Ataxia

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Assessment

Individuals with progressive ataxia should receive an instrumental examination of swallowing "when information gained from clinical examination is not sufficient to guide management of the presenting dysphagia" (Good Practice Point; p. 54).

Keywords: Imaging Assessment (Not Specified), Progressive Ataxia

Individuals with progressive ataxia and suspected dysphagia should receive a comprehensive case history by the speech-language therapist. Case history should include the identification of the signs and symptoms of dysphagia as well as detailed information about the individual's current diet status and preferences (Good Practice Point).

Keywords: Progressive Ataxia

Treatment

Muscle strengthening exercises can be indicated for individuals with dysphagia due to progressive ataxia. Treatment should target underlying swallowing physiology (Good Practice Point).

Keywords: Oral Motor Treatments (includes Shaker Exercises), Progressive Ataxia

A multidisciplinary approach is recommended for the management of individuals with progressive ataxia and dysphagia. A multidisciplinary approach between the speech-language therapist and the dietitian is recommended to ensure optimal nutrition and hydration and between the speech-language therapist and physical and occupational therapist to ensure optimal feeding position and use of aids or adaptations as needed (Good Practice Point).

Keywords: Provider (SLP/Caregiver), Dysphagia, Adults, Progressive Ataxia

Service Delivery

A multidisciplinary approach is recommended for the management of individuals with progressive ataxia and dysphagia. A multidisciplinary approach between the speech-language therapist and the dietitian is recommended to ensure optimal nutrition and hydration and between the speech-language therapist and physical and occupational therapist to ensure optimal feeding position and use of aids or adaptations as needed (Good Practice Point).

Keywords: Provider (SLP/Caregiver), Dysphagia, Adults, Progressive Ataxia

If individuals with progressive ataxia "experience specific difficulties with either their communication and/or swallowing [including sialorrhea] a referral to a [speech-language therapist] SLT is recommended. An open referral system should be in place where patients are able to access help from a SLT as and when required (Good Practice Point; p. 54).

Keywords: Provider (SLP/Caregiver), Provider, Progressive Ataxia, Progressive Ataxia

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Assessment

Individuals with progressive ataxia and suspected hearing loss should be referred to an audiologist for a battery of hearing tests (Good Practice Point). "In specific cases (e.g., ANSD) a referral to a neuro-otologist should be considered" (Good Practice Point; p. 37).

Keywords: Comorbid Diseases and Disorders, Provider, Provider

Treatment

Individuals with progressive ataxia who do not receive benefit from hearing aids should be referred to a cochlear implant center (Grade D Evidence; p. 37). 

Keywords: Cochlear Implants, Comorbid Diseases and Disorders, Devices

"A hearing aid trial should be considered although it is often not suitable" for individuals with progressive ataxia (Good Practice Point; p. 37). 

Keywords: Hearing Aids, Comorbid Diseases and Disorders, Devices

Service Delivery

Individuals with progressive ataxia and suspected hearing loss should be referred to an audiologist for a battery of hearing tests (Good Practice Point). "In specific cases (e.g., ANSD) a referral to a neuro-otologist should be considered" (Good Practice Point; p. 37).

Keywords: Comorbid Diseases and Disorders, Provider, Provider

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