Last reviewed:October 2019
Last updated:January  2019

Summary

Differentials

Common

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

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
  • 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 (AOA 1)
  • Ataxia with oculomotor apraxia 2 (AOA 2)
  • Ataxia with vitamin E deficiency
  • Abetalipoproteinaemia
  • Autosomal-recessive spastic ataxia of Charlevoix-Saguenay
  • Ataxia due to POLG 1 mutation
  • Ataxia due to SCYL1 mutation
  • Ataxia associated with CoQ10 deficiency
  • Ataxia associated with metabolic errors
  • Spinocerebellar ataxia 1 (SCA 1)
  • Spinocerebellar ataxia 2 (SCA 2)
  • Spinocerebellar ataxia 3 (SCA 3)
  • Spinocerebellar ataxia 5 (SCA 5)
  • Spinocerebellar ataxia 6 (SCA 6)
  • Spinocerebellar ataxia 7 (SCA 7)
  • Spinocerebellar ataxia 8 (SCA 8)
  • Spinocerebellar ataxia 10 (SCA 10)
  • Spinocerebellar ataxia 11 (SCA 11)
  • Spinocerebellar ataxia 12 (SCA 12)
  • Spinocerebellar ataxia 13 (SCA 13)
  • Spinocerebellar ataxia 14 (SCA 14)
  • Spinocerebellar ataxia 15/16 (SCA 15/16)
  • Spinocerebellar ataxia 17 (SCA 17)
  • Spinocerebellar ataxia 18 (SCA 18)
  • Spinocerebellar ataxia 19/22 (SCA 19/22)
  • Spinocerebellar ataxia 20 (SCA 20)
  • Spinocerebellar ataxia 21 (SCA 21)
  • Spinocerebellar ataxia 23 (SCA 23)
  • Spinocerebellar ataxia 25 (SCA 25)
  • Spinocerebellar ataxia 26 (SCA 26)
  • Spinocerebellar ataxia 27 (SCA 27)
  • Spinocerebellar ataxia 28 (SCA 28)
  • Spinocerebellar ataxia 29 (SCA 29)
  • Spinocerebellar ataxia 30 (SCA 30)
  • Spinocerebellar ataxia 31 (SCA 31)
  • Spinocerebellar ataxia 32 (SCA 32)
  • Spinocerebellar ataxia 34 (SCA 34)
  • Spinocerebellar ataxia 35 (SCA 35)
  • Spinocerebellar ataxia 36 (SCA 36)
  • Spinocerebellar ataxia 37 (SCA 37)
  • Spinocerebellar ataxia 38 (SCA 38)
  • Spinocerebellar ataxia 40 (SCA 40)
  • 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)

Contributors

Assistant Professor of Neurology

The Ohio State University

Columbus

OH

Disclosures

BKC declares that she has no competing interests.

Dr Barbara Kelly Changizi would like to gratefully acknowledge Dr S.H. Subramony and Dr Hartmut Uschmann, previous contributors to this topic. SHS has received honoraria for lectures given from Athena Diagnostics Company. HU declares that he has no competing interests.

Peer reviewersVIEW ALL

Dean of Medical Faculty

Professor and Chair of Department of Neurology

University Hospital

Bonn

Germany

Disclosures

TK declares that he has no competing interests.

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.

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