Evaluation of ataxia

Last reviewed: 7 Apr 2025
Last updated: 25 Jan 2024

Summary

Differentials

Common

  • Alcoholic cerebellar degeneration
  • Ischemic stroke (cerebellum)
  • Ischemic stroke (brain stem)
  • Hemorrhage 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 disease
  • Neurosyphilis
  • Posterior fossa tumors
  • Craniovertebral junction anomalies
  • Paraneoplastic sensory neuropathy
  • Ataxia with anti-glutamic acid decarboxylase (GAD) antibodies
  • Celiac disease
  • Myoclonus-opsoclonus syndrome
  • Paraneoplastic cerebellar degeneration
  • Miller-Fisher syndrome
  • Sjogren syndrome
  • Neuropathy related to monoclonal gammopathy
  • Hypothyroidism
  • Hypoparathyroidism
  • Vitamin B1 deficiency
  • Vitamin B12 deficiency
  • Friedreich ataxia
  • Ataxia telangiectasia
  • Ataxia with oculomotor apraxia 1 (AOA1)
  • Ataxia with oculomotor apraxia 2 (AOA2)
  • Ataxia with vitamin E deficiency
  • Abetalipoproteinemia
  • 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.

Disclosures

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.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Friedreich’s Ataxia Research Alliance. Clinical management guidelines for Friedreich ataxia: best practice in rare diseases. Nov 2022 [internet publication].Full text  Abstract

Ataxia UK. Management of the ataxias - towards best clinical practice (third edition). Jul 2016 [internet publication].Full text

American College of Radiology. ACR appropriateness criteria: dizziness and ataxia. 2023 [internet publication].Full text

American College of Radiology. ACR appropriateness criteria: ataxia - child. 2022 [internet publication].Full text

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

Use of this content is subject to our disclaimer