Diagnosis and management of progressive ataxia in adults.


Journal

Practical neurology
ISSN: 1474-7766
Titre abrégé: Pract Neurol
Pays: England
ID NLM: 101130961

Informations de publication

Date de publication:
Jun 2019
Historique:
pubmed: 3 5 2019
medline: 18 12 2019
entrez: 4 5 2019
Statut: ppublish

Résumé

Progressive ataxia in adults can be difficult to diagnose, owing to its heterogeneity and the rarity of individual causes. Many patients remain undiagnosed ('idiopathic' ataxia). This paper provides suggested diagnostic pathways for the general neurologist, based on Ataxia UK's guidelines for professionals. MR brain scanning can provide diagnostic clues, as well as identify 'structural' causes such as tumours and multiple sclerosis. Advances in molecular genetics, including the wider and cheaper availability of 'next-generation sequencing', have enabled clinicians to identify many more cases with a genetic cause. Finally, autoimmunity is probably an under-recognised cause of progressive ataxia: as well as patients with antigliadin antibodies there are smaller numbers with various antibodies, including some associated with cancer. There are a few treatable ataxias, but also symptomatic treatments to help people with the spectrum of complications that might accompany progressive ataxias. Multidisciplinary team involvement and allied health professionals' input are critical to excellent patient care, including in the palliative phase. We can no longer justify a nihilistic approach to the management of ataxia.

Identifiants

pubmed: 31048364
pii: practneurol-2018-002096
doi: 10.1136/practneurol-2018-002096
pmc: PMC6585307
doi:

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

196-207

Informations de copyright

© Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY. Published by BMJ.

Déclaration de conflit d'intérêts

Competing interests: None declared.

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Auteurs

Rajith Nilantha de Silva (RN)

Department of Neurology, Essex Centre for Neurological Sciences, Queen's Hospital, Romford, UK desilva63@aol.com.

Julie Vallortigara (J)

Ataxia UK, London, UK.

Julie Greenfield (J)

Ataxia UK, London, UK.

Barry Hunt (B)

Ataxia UK, London, UK.

Paola Giunti (P)

Department of Clinical and Movement Neurosciences, Ataxia Centre, UCL Institute of Neurology, London, UK.

Marios Hadjivassiliou (M)

Academic Department of Neurosciences, Sheffield Teaching Hospitals NHS Trust and University of Sheffield, Sheffield, UK.

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