Inherited Ataxias

Ataxia means ‘lack of coordination,’ and it is a symptom of many conditions. These are placed into the broad groups below.

Canvas – see the Canvas factsheet here: Canvas 2020.

Hereditary ataxias – including Friedreich’s ataxia, spinocerebellar ataxias, and episodic ataxias (but excluding ataxia-telangiectasia*).

Idiopathic progressive ataxias – forms of cerebellar ataxia associated with neurodegeneration of unknown aetiology.

Specific neurological disorders in which progressive ataxia is the dominant symptom, e.g., cerebellar variant of MSA.

Other causes of ataxia

Vascular, Inflammatory (e.g., multiple sclerosis), Traumatic, Metabolic, Developmental, Toxic/drug-related (e.g., alcohol), Neoplastic/paraneoplastic, Epilepsy (in children), Infectious.

The MDA offers support for Hereditary Ataxias and Idiopathic Progressive Ataxias only. Specific neurological disorders will have their own support networks, as will other causes.

Medical Guidelines

These Medical Guidelines were produced to help healthcare professionals in the diagnosis and management of people with ataxia. They were developed by the patient support organisation, Ataxia UK, through extensive consultation with numerous UK healthcare professionals with experience in ataxia. 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. 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. Although these were developed in the UK and cannot be enforced in New Zealand, they are based on clinical expertise, scientific evidence, and extensive consultation with ataxia specialist neurologists and other healthcare professionals. They may be of interest to health professionals supporting people with Ataxia in New Zealand.

The guidelines include comprehensive sections on diagnosis, medical interventions for symptom relief, and therapies. In November 2018, they were endorsed by the European Reference Network for rare neurological diseases (ERN-RND). On 20 February 2019, the Medical Guidelines were published in the Orphanet Journal of Rare Diseases.

Download the latest edition of Ataxia Medical Guidelines 2016 or A Summary of the Ataxia Guidelines.

Useful Websites

• The National Ataxia Foundation – USA – www.ataxia.org

• http://www.livingwithataxia.org/