Ann Child Neurol.  2021 Jul;29(3):109-114. 10.26815/acn.2021.00010.

Clinical Characteristics and Effects of Steroid Therapy in Children with Acute Cerebellar Ataxia

Affiliations
  • 1Department of Pediatrics, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 2Department of Pediatrics, Yeouido St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 3Department of Pediatrics, Daejeon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea

Abstract

Purpose
Acute cerebellar ataxia (ACA) is characterized by unsteady gait and instability of the trunk, and is caused by secondary autoimmune responses to infection or vaccination in healthy children. Although its prognosis is usually very good, full symptom recovery generally takes 2 to 3 months. This study aimed to investigate clinical symptoms, neuroimaging findings, and laboratory findings in children with ACA, and to evaluate the effects of steroid therapy on ACA according to the method of administration (intravenous methylprednisolone vs. oral prednisolone).
Methods
We retrospectively analyzed nine patients diagnosed with ACA or acute cerebellitis (AC) who received steroid therapy.
Results
Nine children were included in this study (mean age, 3.71±2.89 years). The mean duration between prodromal febrile illness and cerebellar symptoms was 9.63±4.66 days. Ataxia (limb and/or truncal) was the most common cerebellar sign. Steroids were administered in two ways: methylprednisolone (20 to 30 mg/kg/day) was changed to an oral steroid (prednisolone, 1 mg/kg/day) after 2 to 3 days of administration; an oral steroid was used from the beginning of treatment. The cerebellar symptoms began to improve within 2 to 4 days of steroid therapy. All patients fully recovered without sequelae. The mean interval until full recovery of the cerebellar symptoms was 28.0±19.3 days, and was not significantly different between patients who received an oral steroid after methylprednisolone pulse therapy and patients who only received an oral steroid (P>0.05).
Conclusion
Regardless of the method of drug administration, steroid therapy helps to improve cerebellar symptoms in children with ACA/AC.

Keyword

Cerebellar ataxia; Cerebellar diseases; Steroids
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