Ataxia Medical Guidelines aim to provide recommendations for healthcare professionals on the diagnosis and management of people with progressive ataxia. To download the full document, including references, click here. A summary of the Ataxia Guidelines has been published in 'Guidelines', the online resource summarising clinical guidelines for primary care and can be read here.
The progressive ataxias are rare neurological conditions, and are often poorly understood by healthcare professionals. Diagnosis has generally been a long process because of the rarity and complexity of the different ataxias1. In addition, many healthcare professionals are unsure how best to manage the conditions and there is sometimes a feeling that little can be done for these patients1,2.
Although there are no disease-modifying treatments for the majority of the progressive ataxias, there are many aspects of the conditions that are treatable and it is thus important that this is recognised by the relevant healthcare professionals. The diagnosis and management of the few treatable causes is also of paramount importance. All this highlights the importance of producing these guidelines in order to increase awareness and understanding of these conditions, and lead to their improved diagnosis and management.
With new developments in genetic technologies and the discovery of more genes, diagnosis is improving and has great scope to continue to do so. In addition, research is advancing and many human trials to test medications are taking place, making us more optimistic that disease-modifying treatments will be found for the progressive ataxias.
Conditions covered in these guidelines
Ataxia means ‘lack of coordination’ and it is a symptom of many conditions. These guidelines have been developed by the patient support organisation, Ataxia UK, through extensive consultation with numerous UK healthcare professionals with experience of ataxia. Certain aspects of these guidelines may however be relevant to these other conditions.
Table 1a: Conditions covered in these guidelines
Hereditary ataxias
|
Idiopathic progressive ataxias
|
Specific neurological disorders
|
Table 1b: Other causes of ataxia
Vascular |
Inflammatory (eg: multiple sclerosis) |
Traumatic |
Metabolic |
Developmental |
Toxic / drug-related (eg: alcohol) |
Neoplastic / paraneoplastic |
Epilepsy (in children) |
Infectious |
Epidemiology of the ataxias
Epidemiological studies of the progressive ataxias in the UK are sparse, data from the UK are thus summarised with information from studies in other countries (see Box 1). In the UK, the latest estimates based on the studies below suggest there are at least 10,000 adults and 500 children with progressive ataxia3,4.
Although the progressive ataxias are rare conditions, when taken together they are more common than other better known neurological conditions. These studies suggest that the prevalence of the progressive ataxias is higher than conditions that are generally better known such as Huntington’s disease5 and motor neurone disease6.
Box 1: Epidemiological studies |
European studies:
|
Guideline development
These guidelines have been developed through extensive consultation with numerous UK healthcare professionals with experience of ataxia, in collaboration with the patient support organisation, Ataxia UK. This is the third edition of these guidelines.
Contributors for each section were selected due to their clinical expertise in ataxia in the relevant discipline. They reviewed the medical literature for their section, provided scientific evidence for the efficacy of different interventions and graded the level of evidence following the procedure of the Guideline International Network (GIN)10.
The information on the level of evidence was then used to give a grading to each recommendation made in these guidelines. Table 2 details the level of evidence and grading system used.
A Guideline Development Group consisting of neurologists with expertise in ataxia and representatives of Ataxia UK reviewed all the sections and discussed any changes with contributors until consensus was reached.
Table 2: Evidence grading scheme for these guidelines
Level of evidence (categorisation of reference materials)11 |
|
Grading of recommendations in the guidelines based on the level of evidence12 |
(Adapted from Reference) |
This information is taken from Management of the ataxias - towards best clinical practice third edition, July 2016. This document aims to provide recommendations for healthcare professionals on the diagnosis and management of people with progressive ataxia. To view the full document, including references, click here.
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