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Acute cerebellar ataxia in children

Donald L Gilbert, MD, MS
Section Editors
Marc C Patterson, MD, FRACP
Stephen J Teach, MD, MPH
Deputy Editor
Carrie Armsby, MD, MPH


Acute cerebellar ataxia is a syndrome that occurs in previously well children, often presenting as a postinfectious disorder [1-4]. The pathogenesis, clinical presentation, evaluation, and prognosis of acute cerebellar ataxia will be reviewed here. The differential diagnosis and evaluation of the child presenting with acute ataxia is discussed separately. (See "Approach to the child with acute ataxia".)

In adults, a wide variety of processes can cause cerebellar ataxia, and these can present with acute or chronic disease courses. The diagnostic considerations in an adult with ataxia are discussed separately. (See "Overview of cerebellar ataxia in adults".)


Acute cerebellar ataxia accounts for 30 to 50 percent of all cases of pediatric ataxia and usually occurs in children under six years of age, although older children and adolescents can also be affected [1,2,5]. The incidence of this disorder in the pediatric age group is at least 1 in 100,000 to 500,000, making it the most common cause of ataxia in children [3,6,7].


Acute cerebellar ataxia may occur unheralded or, more commonly, after an acute febrile illness [1-3]. Varicella infection-induced cases, once the most common single post-infectious cause, has diminished due to vaccination [6,8]. (See "Clinical features of varicella-zoster virus infection: Chickenpox", section on 'Neurologic complications'.)

Numerous other infectious agents have been implicated in the pathogenesis of acute cerebellar ataxia, including coxsackievirus, echovirus, enteroviruses, Epstein-Barr virus (EBV), hepatitis A, herpes simplex virus (HSV) I, human herpesvirus 6 (HHV-6), measles, mumps, parvovirus B19, Borrelia burgdorferi (Lyme disease), malaria, Mycoplasma pneumoniae, and typhoid fever [2,9-17]. The syndrome has been anecdotally reported following vaccination for varicella, hepatitis B, rabies, meningococcal group C, and human papilloma virus (HPV), all without evidence of systemic infection [1,2,18-21]. However, rates after vaccination are much lower than in association with infection: For example, the association of acute cerebellar ataxia with varicella infection is at least 35 times higher than after vaccination [6].


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Literature review current through: Sep 2016. | This topic last updated: Sep 29, 2016.
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