Acute cerebellar ataxia in children
- Donald L Gilbert, MD, MS
Donald L Gilbert, MD, MS
- Professor of Pediatrics and Neurology; Director, Movement Disorders and Tourette Syndrome Clinics
- Cincinnati Children's Hospital Medical Center
- Section Editors
- Marc C Patterson, MD, FRACP
Marc C Patterson, MD, FRACP
- Section Editor — Pediatric Neurology
- Professor of Neurology, Pediatrics, and Medical Genetics
- Chair, Division of Child and Adolescent Neurology
- Mayo Clinic College of Medicine
- Stephen J Teach, MD, MPH
Stephen J Teach, MD, MPH
- Section Editor — Pediatric Signs and Symptoms
- Professor of Pediatrics and Emergency Medicine
- George Washington University School of Medicine and Health Sciences
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 .
- Connolly AM, Dodson WE, Prensky AL, Rust RS. Course and outcome of acute cerebellar ataxia. Ann Neurol 1994; 35:673.
- Nussinovitch M, Prais D, Volovitz B, et al. Post-infectious acute cerebellar ataxia in children. Clin Pediatr (Phila) 2003; 42:581.
- Desai J, Mitchell WG. Acute cerebellar ataxia, acute cerebellitis, and opsoclonus-myoclonus syndrome. J Child Neurol 2012; 27:1482.
- Poretti A, Benson JE, Huisman TA, Boltshauser E. Acute ataxia in children: approach to clinical presentation and role of additional investigations. Neuropediatrics 2013; 44:127.
- Weiss, S, Carter, S. Course and prognosis of acute cerebellar ataxia in childhood. Neurology 1959; 9:711.
- van der Maas NA, Bondt PE, de Melker H, Kemmeren JM. Acute cerebellar ataxia in the Netherlands: a study on the association with vaccinations and varicella zoster infection. Vaccine 2009; 27:1970.
- Davis DP, Marino A. Acute cerebellar ataxia in a toddler: case report and literature review. J Emerg Med 2003; 24:281.
- Rudloe T, Prabhu SP, Gorman MP, et al. The Yield of Neuroimaging in Children Presenting to the Emergency Department With Acute Ataxia in the Post-Varicella Vaccine Era. J Child Neurol 2015; 30:1333.
- Guess HA, Broughton DD, Melton LJ 3rd, Kurland LT. Population-based studies of varicella complications. Pediatrics 1986; 78:723.
- Hata A, Fujita M, Morishima T, et al. Acute cerebellar ataxia associated with primary human herpesvirus-6 infection: a report of two cases. J Paediatr Child Health 2008; 44:607.
- Stumpf DA. Acute ataxia. Pediatr Rev 1987; 8:303.
- Ryan MM, Engle EC. Acute ataxia in childhood. J Child Neurol 2003; 18:309.
- Lopez MD, Wise C. Acute ataxia in a 4-year-old boy: a case of Lyme disease neuroborreliosis. Am J Emerg Med 2008; 26:1069.e5.
- Cohen HA, Ashkenazi A, Nussinovitch M, et al. Mumps-associated acute cerebellar ataxia. Am J Dis Child 1992; 146:930.
- Shimizu Y, Ueno T, Komatsu H, et al. Acute cerebellar ataxia with human parvovirus B19 infection. Arch Dis Child 1999; 80:72.
- Ito H, Sayama S, Irie S, et al. Antineuronal antibodies in acute cerebellar ataxia following Epstein-Barr virus infection. Neurology 1994; 44:1506.
- Cho TA, Schmahmann JD, Cunnane ME. Case records of the Massachusetts General Hospital. Case 30-2013. A 19-year-old man with otalgia, slurred speech, and ataxia. N Engl J Med 2013; 369:1253.
- Deisenhammer F, Pohl P, Bösch S, Schmidauer C. Acute cerebellar ataxia after immunisation with recombinant hepatitis B vaccine. Acta Neurol Scand 1994; 89:462.
- Sunaga Y, Hikima A, Ostuka T, Morikawa A. Acute cerebellar ataxia with abnormal MRI lesions after varicella vaccination. Pediatr Neurol 1995; 13:340.
- Cutroneo PM, Italiano D, Trifirò G, et al. Acute cerebellar ataxia following meningococcal group C conjugate vaccination. J Child Neurol 2014; 29:128.
- Yonee C, Toyoshima M, Maegaki Y, et al. Association of acute cerebellar ataxia and human papilloma virus vaccination: a case report. Neuropediatrics 2013; 44:265.
- Adams C, Diadori P, Schoenroth L, Fritzler M. Autoantibodies in childhood post-varicella acute cerebellar ataxia. Can J Neurol Sci 2000; 27:316.
- Fritzler MJ, Zhang M, Stinton LM, Rattner JB. Spectrum of centrosome autoantibodies in childhood varicella and post-varicella acute cerebellar ataxia. BMC Pediatr 2003; 3:11.
- Shiihara T, Kato M, Konno A, et al. Acute cerebellar ataxia and consecutive cerebellitis produced by glutamate receptor delta2 autoantibody. Brain Dev 2007; 29:254.
- Uchibori A, Sakuta M, Kusunoki S, Chiba A. Autoantibodies in postinfectious acute cerebellar ataxia. Neurology 2005; 65:1114.
- Papp J, Dorsey ST. A preschool-age child with first-time seizure and ataxia. J Emerg Med 2009; 36:30.
- Whelan HT, Verma S, Guo Y, et al. Evaluation of the child with acute ataxia: a systematic review. Pediatr Neurol 2013; 49:15.
- Sugiyama N, Hamano S, Tanaka M, et al. A prognostic value of neuron-specific enolase in cerebrospinal fluid of acute cerebellar ataxia. Tokai J Exp Clin Med 2010; 35:25.
- Gieron-Korthals MA, Westberry KR, Emmanuel PJ. Acute childhood ataxia: 10-year experience. J Child Neurol 1994; 9:381.
- De Bruecker Y, Claus F, Demaerel P, et al. MRI findings in acute cerebellitis. Eur Radiol 2004; 14:1478.
- Yeung WL, Li CK, Nelson EA, et al. Unusual neurological presentation of neuroblastoma. Hong Kong Med J 2003; 9:142.
- Visudtibhan A, Visudhiphan P, Chiemchanya S. Recurrent acute cerebellar ataxia of childhood following nonspecific respiratory tract infection. J Med Assoc Thai 1998; 81:1015.
- Yazawa S, Ohi T, Sugimoto S, Matsukura S. [A case of acute cerebellar ataxia with abnormal single photon emission computed tomography]. Rinsho Shinkeigaku 1995; 35:424.
- Göhlich-Ratmann G, Wallot M, Baethmann M, et al. Acute cerebellitis with near-fatal cerebellar swelling and benign outcome under conservative treatment with high dose steroids. Eur J Paediatr Neurol 1998; 2:157.
- Daaboul Y, Vern BA, Blend MJ. Brain SPECT imaging and treatment with IVIg in acute post-infectious cerebellar ataxia: case report. Neurol Res 1998; 20:85.
- Go T. Intravenous immunoglobulin therapy for acute cerebellar ataxia. Acta Paediatr 2003; 92:504.
- Yamashita S, Murakami C, Izumi Y, et al. Severe chronic active Epstein-Barr virus infection accompanied by virus-associated hemophagocytic syndrome, cerebellar ataxia and encephalitis. Psychiatry Clin Neurosci 1998; 52:449.