Ataxia
Page most recently updated 7 September 2026
This page reviews the differential diagnosis of ataxia in the context of presentations with psychiatric symptoms, focusing on conditions in which both may occur together. It begins by defining ataxia and outlining initial diagnostic considerations before examining the broader differential diagnosis.
Description
Cerebellar ataxia may manifest with abnormalities of eye movements, speech, limb coordination, posture and gait. Oculomotor findings may include nystagmus and dysmetric saccades. Dysarthric speech may occur. Limb examination may demonstrate dysmetria and kinetic tremor on finger-to-nose or heel-to-shin testing, dysdiadochokinesia on rapid alternating movements, and rebound phenomenon on the Stewart–Holmes manoeuvre. Postural abnormalities may include sway of the trunk when sitting, titubation and lateropulsion when standing, while gait may be broad-based with impaired tandem gait.
Initial considerations
Although this section is primarily concerned with subacute and chronic ataxia in adults, clinicians should remain vigilant for acute or sudden-onset presentations with ataxia, which require urgent assessment because they may represent a medical emergency, particularly cerebellar stroke, including both ischemic and haemorrhagic stroke. Other important acute causes include Wernicke encephalopathy and intoxication with alcohol or benzodiazepines.
Broader differentials
● From a cognitive lens ➚perspective
◦ Progressive supranuclear palsy ➚
◦ Spinocerebellar ataxias (SCAs), particularly SCA1, SCA2, SCA3, SCA8, SCA12, SCA17, and SCA48
◦ Dentatorubral pallidoluysian atrophy (DRPLA)
◦ Aceruloplasminemia (a type of neurodegeneration with brain iron accumulation [NBIA])
◦ Acquired hepatocerebral degeneration (AHD)
◦ Fragile X-associated tremor/ataxia syndrome (FXTAS)
● From a lesional lens ➚ perspective
Structural abnormalities affecting the cerebellum or its connections ➚ can result in cerebellar ataxia. Examples include traumatic, neoplastic and vascular lesions. Cerebellar ischemia and haemorrhage are medical emergencies requiring urgent assessment.
● Other differentials (including multisystemic conditions ➚ )
● Intrinsic
▪ Immune-mediated
◦ Autoimmune cerebellar ataxias
Anti-GAD65 autoimmune ataxia ➚
Anti-P/Q voltage-gated calcium channel autoimmune ataxia
Anti-Caspr2 autoimmune ataxia ➚
Paraneoplastic cerebellar degeneration (PCD)
(associated with a range of neuronal antibodies and malignancies)
Anti-Yo is most strongly associated with breast and gynaecological cancers
Anti-Hu ➚ and anti-Zic4 with small-cell lung cancer
Anti-Tr/DNER with Hodgkin lymphoma
Anti-Ri with breast cancer
Anti-CV2/CRMP5 with small-cell lung cancer and thymoma
◦ Connective tissue diseases
Systemic lupus erythematosus ➚
◦ Systemic vasculitides
◦ Other immune-mediated disorders
Celiac disease ➚ (and gluten ataxia)
Multiple sclerosis
Acute disseminated encephalomyelitis (ADEM)
Postinfectious cerebellitis (after recent infection with several types of viruses, e.g., VZV, measles, EBV)
▪ Endocrine
▪ Genetic
(in addition to genetic conditions mentioned above with the cognitive lens)
◦ Episodic ataxias
◦ Friedreich ataxia
◦ Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS)
◦ Autosomal recessive cerebellar ataxia type 1 (ARCA1)
◦ Autosomal recessive cerebellar ataxia type 3 (ARCA3)
◦ Ataxia-telangiectasia
◦ Ataxia-telangiectasia-like disorder
◦ Xeroderma pigmentosum
◦ Cockayne syndrome
◦ Ataxia with oculomotor apraxia (AOA)
◦ Neurometabolic disorders
Mitochondrial disorders (ataxia frequent in MERRF and KSS; less frequent in MELAS) ➚
Cerebrotendinous xanthomatosis ➚
Hereditary vitamin E deficiency (AVED)
▪ Other
(not cerebellar ataxia but may resemble it)
◦ Functional movement disorder
◦ Normal pressure hydrocephalus (gait impairment [often broad-based]; important to recognize because it may be treatable
◦ Migraine with brainstem aura
◦ Bilateral vestibulopathy (e.g., ototoxicity due to aminoglycosideantibiotics, chemotherapy, loop diuretics or other agents; meningitis)
● Extrinsic
▪ Toxic and deficiency states
◦ Drug-induced
Benzodiazepines
Antiseizure medications and several mood stabilizers, including (but not limited to):
Carbamazepine, lamotrigine (particularly with toxicity or polypharmacy), phenytoin
Valproic acid in the setting of hyperammonemia
Chemotherapeutic agents, such as cytarabine
◦ Toxic exposures/poisoning
Excessive alcohol ingestion, carbon tetrachloride, heavy metals, phencyclidine, and toluene poisoning
◦ Deficiency states
Thiamine (vitamin B1) deficiency ➚ (Wernicke encephalopathy)
Vitamin B12 deficiency ➚ (typically sensory ataxia)
▪ Infectious
◦ Bacterial
Neurosyphilis ➚ (sensory ataxia with tabes dorsalis; not a cerebellar ataxia but may resemble it)
◦ Viral
Progressive multifocal leukoencephalopathy (PML)
◦ Other
Prion diseases, particularly sporadic and variant Creutzfeldt-Jakob disease (sCJD and vCJD) (ataxia is common in both sCJD and vCJD, with a particularly prominent cerebellar presentation in vCJD)
Selected references and further reading — Movement lens
Jankovic J, Hallett M, Okun MS, Comella CL, Fahn S. Principles and practice of movement disorders. 3rd ed. Philadelphia: Elsevier; 2021.
Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, editors. Bradley and Daroff's neurology in clinical practice. 8th ed. Philadelphia: Elsevier; 2022.
Campbell WW. DeJong's the neurologic examination. 8th ed. Philadelphia: Wolters Kluwer; 2020.
Arciniegas DB, Yudofsky SC, Hales RE, editors. The American Psychiatric Association Publishing textbook of neuropsychiatry and clinical neurosciences. 6th ed. Washington (DC): American Psychiatric Association Publishing; 2018.
Agrawal N, Faruqui R, Bodani M, editors. Oxford textbook of neuropsychiatry. Oxford: Oxford University Press; 2020.
Dening T, Thomas A, editors. Oxford textbook of old age psychiatry. 3rd ed. Oxford: Oxford University Press; 2021.
Blazer DG, Steffens DC, Busse EW, editors. The American Psychiatric Publishing textbook of geriatric psychiatry. 5th ed. Washington (DC): American Psychiatric Publishing; 2015.