Assessment of ataxia

Last reviewed: 2 Jun 2022
Last updated: 12 May 2022

Summary

Differentials

Common

  • Alcoholic cerebellar degeneration
  • Ischaemic stroke (cerebellum)
  • Ischaemic stroke (brain stem)
  • Haemorrhage in the cerebellum
  • Multiple sclerosis (MS)
  • Wernicke-Korsakoff syndrome
Full details

Uncommon

  • Drug-induced ataxia
  • Toxic neuropathies
  • Von Hippel-Lindau syndrome
  • Sequel to hypoxic encephalopathy or heat stroke
  • Acute cerebellitis
  • HIV
  • Gerstmann-Straussler syndrome
  • Creutzfeldt-Jakob syndrome (CJD) (ataxic variant)
  • Cerebellar abscess
  • Whipple's disease
  • Neurosyphilis
  • Posterior fossa tumours
  • Craniovertebral junction anomalies
  • Paraneoplastic sensory neuropathy
  • Ataxia with anti-glutamic acid decarboxylase (GAD) antibodies
  • Coeliac disease
  • Myoclonus-opsoclonus syndrome
  • Paraneoplastic cerebellar degeneration
  • Miller-Fisher syndrome
  • Sjogren's syndrome
  • Neuropathy related to monoclonal gammopathy
  • Hypothyroidism
  • Hypoparathyroidism
  • Vitamin B1 deficiency
  • Vitamin B12 deficiency
  • Friedreich's ataxia
  • Ataxia telangiectasia
  • Ataxia with oculomotor apraxia 1 (AOA1)
  • Ataxia with oculomotor apraxia 2 (AOA2)
  • Ataxia with vitamin E deficiency
  • Abetalipoproteinaemia
  • Autosomal-recessive spastic ataxia of Charlevoix-Saguenay
  • Ataxia due to POLG1 mutation
  • Ataxia due to SCYL1 mutation
  • Ataxia associated with CoQ10 deficiency
  • Ataxia associated with metabolic errors
  • Spinocerebellar ataxia 1 (SCA1)
  • Spinocerebellar ataxia 2 (SCA2)
  • Spinocerebellar ataxia 3 (SCA3)
  • Spinocerebellar ataxia 5 (SCA5)
  • Spinocerebellar ataxia 6 (SCA6)
  • Spinocerebellar ataxia 7 (SCA7)
  • Spinocerebellar ataxia 8 (SCA8)
  • Spinocerebellar ataxia 10 (SCA10)
  • Spinocerebellar ataxia 11 (SCA11)
  • Spinocerebellar ataxia 12 (SCA12)
  • Spinocerebellar ataxia 13 (SCA13)
  • Spinocerebellar ataxia 14 (SCA14)
  • Spinocerebellar ataxia 15/16 (SCA15/16)
  • Spinocerebellar ataxia 17 (SCA17)
  • Spinocerebellar ataxia 18 (SCA18)
  • Spinocerebellar ataxia 19/22 (SCA19/22)
  • Spinocerebellar ataxia 20 (SCA20)
  • Spinocerebellar ataxia 21 (SCA21)
  • Spinocerebellar ataxia 23 (SCA23)
  • Spinocerebellar ataxia 25 (SCA25)
  • Spinocerebellar ataxia 26 (SCA26)
  • Spinocerebellar ataxia 27 (SCA27)
  • Spinocerebellar ataxia 28 (SCA28)
  • Spinocerebellar ataxia 29 (SCA29)
  • Spinocerebellar ataxia 30 (SCA30)
  • Spinocerebellar ataxia 31 (SCA31)
  • Spinocerebellar ataxia 32 (SCA32)
  • Spinocerebellar ataxia 34 (SCA34)
  • Spinocerebellar ataxia 35 (SCA35)
  • Spinocerebellar ataxia 36 (SCA36)
  • Spinocerebellar ataxia 37 (SCA37)
  • Spinocerebellar ataxia 38 (SCA38)
  • Spinocerebellar ataxia 40 (SCA40)
  • Dentatorubral-pallido-luysian atrophy (DRPLA)
  • Episodic ataxia type 1
  • Episodic ataxia type 2
  • Fragile-X tremor-ataxia syndrome (FXTAS)
  • Mitochondrial cytopathy
  • Niemann-Pick disease type C (NP-C)
Full details

Contributors

Authors

Vikram G Shakkottai, MD

Associate Professor of Neurology

University of Michigan

Ann Arbor

Michigan

Disclosures

VGS has research funding through the National Institutes of Health, and the National Ataxia Foundation. He serves on the Medical Research Advisory Board of the National Ataxia Foundation. He is listed as an inventor on a patent filed by the University of Michigan for a therapeutic combination for cerebellar ataxia. He receives royalties from UptoDate Inc. He serves on the advisory board of uniQure.

Acknowledgements

Professor Vikram G Shakkottai would like to gratefully acknowledge Dr Barbara Kelly Changizi, Dr S.H. Subramony and Dr Hartmut Uschmann, previous contributors to this topic. SHS has received honoraria for lectures given from Athena Diagnostics Company. BKC and HU declare that they have no competing interests.

Peer reviewers

Thomas Klockgether, MD

Dean of Medical Faculty

Professor and Chair of Department of Neurology

University Hospital

Bonn

Germany

Disclosures

TK declares that he has no competing interests.

Susan L. Perlman, MD

Clinical Professor of Neurology/Director

Ataxia Center and HD Center of Excellence

UCLA

Los Angeles

CA

Disclosures

SLP is the co-author of 2 systematic reviews referenced in this topic.

  • Assessment of ataxia images
  • Guidelines

    • ACR Appropriateness Criteria: ataxia - child
    • Diagnostic Criteria for Primary Autoimmune Cerebellar Ataxia—Guidelines from an International Task Force on Immune-Mediated Cerebellar Ataxias
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