Episodic ataxia
Last updated 18 Aug 2025
Meets Patient’s editorial guidelines
- DownloadDownload
- Share
- Language
- Discussion
- Audio Version
- Add to preferred sources on Google
Episodic ataxia is a group of related conditions that affect the nervous system and cause problems with movement and coordination. People with episodic ataxia have episodes of poor coordination and balance (ataxia). During these episodes, many people also experience dizziness (vertigo), nausea and vomiting, migraines, blurred or double vision, slurred speech, and ringing in the ears (tinnitus). Seizures, muscle weakness, and paralysis that affect one side of the body (hemiplegia) may also occur during these episodes.
Description
Episodic ataxia is a group of related conditions that affect the nervous system and cause problems with movement and coordination. People with episodic ataxia have episodes of poor coordination and balance (ataxia). During these episodes, many people also experience dizziness (vertigo), nausea and vomiting, migraines, blurred or double vision, slurred speech, and ringing in the ears (tinnitus). Seizures, muscle weakness, and paralysis that affect one side of the body (hemiplegia) may also occur during these episodes.
Additionally, a muscle abnormality called myokymia or an eye abnormality called nystagmus can occur during or between episodes. Myokymia causes muscle cramping; stiffness; or continuous, fine muscle twitching that appears as rippling under the skin. Nystagmus refers to rapid, involuntary eye movements.
Episodes of ataxia and other symptoms can begin anytime from early childhood to adulthood. They can be triggered by environmental factors such as stress, caffeine, alcohol, certain medications, physical activity, and illness. The duration of episodes may vary from seconds to days, and the frequency ranges from several episodes per day to one or two every few months. Between episodes, affected individuals may have no signs or symptoms. However, some continue to experience ataxia, which may worsen over time.
Some children with episodic ataxia have delayed development of speech or motor skills, such as standing and walking. They may also have learning difficulties.
Researchers have identified at least 11 types of episodic ataxia, distinguished by their pattern of signs and symptoms, age of onset, length of episodes, and genetic cause.
Inheritance patterns
The source lists the following possible inheritance patterns: Autosomal dominant. More than one pattern can apply in different cases.
Related genes
Other names
EA
Related patient information and medicines
Patient information leaflet
Chickenpox contact in pregnancy
There are oth... This leaflet is primarily intended for pregnant women who may have come into contact with someone who has chickenpox or shingles. Chickenpox contact in pregnancy A person with chickenpox is very infectious. About 1 in 100 pregnant women who develop chickenpox-related pneumonia die of this serious infection. Brain inflammation.
Patient information leaflet
Coeliac disease
If you have one of the following conditions, your medical professional may suggest that you have the tests done to ... Unexplained peripheral neuropathy , which could be gluten neuropathy. Persistent, unexplained abnormal liver function tests. Unexplained ataxia, that could be gluten ataxia. (Ataxia is a rare brain condition which causes problems.
Patient information leaflet
Chickenpox in adults and teenagers
Symptoms tend to be worse in adults than in children. Treatments can ease the symptoms until the illness goes. What is chickenpox. The chickenpox infection is caused by the varicella-zoster virus (VZV). The immune system makes proteins called antibodies during the infection. People who smoke are more likely to get this. Inflammation of the brain.
Patient information leaflet
Peripheral neuropathy
Peripheral neuropathy means damage to one or more of your peripheral nerves. The damage means that the messages that travel between your central ... Hereditary diseases Certain diseases that you can inherit from your parents can cause peripheral neuropathy. Two of the most common of these are Charcot-Marie-Tooth syndrome and Friedreich's ataxia..
Patient information leaflet
Chickenpox in children
It comes in various brand names. The dose for each age is given with the medicine packet. Paracetamol does not treat the cause of the fever. It merely helps to ease discomfort. For example: Reye's syndrome.... Inflammation of the heart muscle (myocarditis). Kidney inflammation (glomerulonephritis). Appendicitis. Ataxia (problems co-ordinating.
Patient information leaflet
Diabetes
Maturity-onset dia... Wolfram's syndrome : another genetic cause of diabetes. It is also called DIDMOAD (because it causes diabetes insipidus , diabetes mellitus, optic atrophy and deafness). Other genetic diseases that can cause diabetes : these include Friedreich's ataxia and haemochromatosis. What causes diabetes. Diabetes is a condition where.
Further reading and references
- Episodic ataxia; MedlinePlus, National Library of Medicine.
- Cader MZ, Steckley JL, Dyment DA, McLachlan RS, Ebers GC. A genome-wide screen and linkage mapping for a large pedigree with episodic ataxia. Neurology. 2005 Jul 12;65(1):156-8. doi: 10.1212/01.wnl.0000167186.05465.7c.
- Chivukula AS, Suslova M, Kortzak D, Kovermann P, Fahlke C. Functional consequences of SLC1A3 mutations associated with episodic ataxia 6. Hum Mutat. 2020 Nov;41(11):1892-1905. doi: 10.1002/humu.24089. Epub 2020 Sep 9.
- Conroy J, McGettigan P, Murphy R, Webb D, Murphy SM, McCoy B, Albertyn C, McCreary D, McDonagh C, Walsh O, Lynch S, Ennis S. A novel locus for episodic ataxia:UBR4 the likely candidate. Eur J Hum Genet. 2014 Apr;22(4):505-10. doi: 10.1038/ejhg.2013.173. Epub 2013 Aug 28.
- Damji KF, Allingham RR, Pollock SC, Small K, Lewis KE, Stajich JM, Yamaoka LH, Vance JM, Pericak-Vance MA. Periodic vestibulocerebellar ataxia, an autosomal dominant ataxia with defective smooth pursuit, is genetically distinct from other autosomal dominant ataxias. Arch Neurol. 1996 Apr;53(4):338-44. doi: 10.1001/archneur.1996.00550040074016.
- Escayg A, De Waard M, Lee DD, Bichet D, Wolf P, Mayer T, Johnston J, Baloh R, Sander T, Meisler MH. Coding and noncoding variation of the human calcium-channel beta4-subunit gene CACNB4 in patients with idiopathic generalized epilepsy and episodic ataxia. Am J Hum Genet. 2000 May;66(5):1531-9. doi: 10.1086/302909. Epub 2000 Apr 4.
- Jen J, Kim GW, Baloh RW. Clinical spectrum of episodic ataxia type 2. Neurology. 2004 Jan 13;62(1):17-22. doi: 10.1212/01.wnl.0000101675.61074.50.
- Jen JC, Graves TD, Hess EJ, Hanna MG, Griggs RC, Baloh RW; CINCH investigators. Primary episodic ataxias: diagnosis, pathogenesis and treatment. Brain. 2007 Oct;130(Pt 10):2484-93. doi: 10.1093/brain/awm126. Epub 2007 Jun 15.
- Jen JC, Wan J, Palos TP, Howard BD, Baloh RW. Mutation in the glutamate transporter EAAT1 causes episodic ataxia, hemiplegia, and seizures. Neurology. 2005 Aug 23;65(4):529-34. doi: 10.1212/01.wnl.0000172638.58172.5a.
- Kerber KA, Jen JC, Lee H, Nelson SF, Baloh RW. A new episodic ataxia syndrome with linkage to chromosome 19q13. Arch Neurol. 2007 May;64(5):749-52. doi: 10.1001/archneur.64.5.749.
- Piarroux J, Riant F, Humbertclaude V, Remerand G, Hadjadj J, Rejou F, Coubes C, Pinson L, Meyer P, Roubertie A. FGF14-related episodic ataxia: delineating the phenotype of Episodic Ataxia type 9. Ann Clin Transl Neurol. 2020 Apr;7(4):565-572. doi: 10.1002/acn3.51005. Epub 2020 Mar 12.
- Rajakulendran S, Schorge S, Kullmann DM, Hanna MG. Episodic ataxia type 1: a neuronal potassium channelopathy. Neurotherapeutics. 2007 Apr;4(2):258-66. doi: 10.1016/j.nurt.2007.01.010.
- Schwarz N, Hahn A, Bast T, Muller S, Loffler H, Maljevic S, Gaily E, Prehl I, Biskup S, Joensuu T, Lehesjoki AE, Neubauer BA, Lerche H, Hedrich UBS. Mutations in the sodium channel gene SCN2A cause neonatal epilepsy with late-onset episodic ataxia. J Neurol. 2016 Feb;263(2):334-343. doi: 10.1007/s00415-015-7984-0. Epub 2015 Dec 8.
- Spacey S. Episodic Ataxia Type 2 - RETIRED CHAPTER, FOR HISTORICAL REFERENCE ONLY. 2003 Feb 24 [updated 2015 Oct 15]. In: Adam MP, Bick S, Mirzaa GM, Wallace SE, Amemiya A, editors. GeneReviews(R) [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2026. Available from http://www.ncbi.nlm.nih.gov/books/NBK1501/
- Steckley JL, Ebers GC, Cader MZ, McLachlan RS. An autosomal dominant disorder with episodic ataxia, vertigo, and tinnitus. Neurology. 2001 Oct 23;57(8):1499-502. doi: 10.1212/wnl.57.8.1499.
- Strupp M, Zwergal A, Brandt T. Episodic ataxia type 2. Neurotherapeutics. 2007 Apr;4(2):267-73. doi: 10.1016/j.nurt.2007.01.014.
- Wu Q, Akhter A, Pant S, Cho E, Zhu JX, Garner A, Ohyama T, Tajkhorshid E, van Meyel DJ, Ryan RM. Ataxia-linked SLC1A3 mutations alter EAAT1 chloride channel activity and glial regulation of CNS function. J Clin Invest. 2022 Apr 1;132(7):e154891. doi: 10.1172/JCI154891.
Join the conversation
Share your thoughts or experiences with others in our patient communities.
Ask, share, connect.
Browse discussions, ask questions, and share experiences across hundreds of health topics.
Sign up to the Patient newsletter
Your weekly dose of clear, trustworthy health advice - written to help you feel informed, confident and in control.
By subscribing you accept our Privacy Policy. You can unsubscribe at any time. We never sell your data.